Benign gynecologic lesions: Vulva, Vagina, Cervix, Uterus, Oviduct, Ovary, Ultrasound Imaging of Pelvic Structures





Key points





  • The most common large cyst of the vulva is a cystic dilation of an obstructed Bartholin duct, with a lifetime risk estimated to be 2%. These cysts occur most often during the third decade. Inflamed cysts may be treated with oral antibiotics or incision and drainage.



  • The vulva contains 1% of the skin surface of the body, but 5% to 10% of all malignant melanomas in women arise from this region. Melanoma is the second most common malignancy arising in the vulva and accounts for 2% to 3% of all of the melanomas occurring in women.



  • Ideally all vulvar nevi should be excised and examined histologically. Special emphasis should be directed toward the flat junctional nevus and the dysplastic nevus because they have the greatest potential for malignant transformation. The dysplastic nevus is characterized by being more than 5 mm in diameter, with irregular borders and patches of variegated pigment.



  • The management of nonobstetric vulvar hematomas is usually conservative unless the hematoma is greater than 10 cm in diameter or rapidly increasing.



  • In adult women, 50% of cases of chronic vulvovaginal pruritus are due to allergic and irritant contact dermatitis. The most common causes of vulvar contact dermatitis are cosmetic and local therapeutic agents. Initial treatment of severe lesions is removal of all irritants or potential allergens and application of topical steroids until the skin returns to normal.



  • Women usually develop psoriasis during their teenage years, with approximately 3% of adult women being affected. Approximately 20% of these have involvement of the vulvar skin. The margins of psoriasis are better defined than the common skin conditions in the differential diagnosis, including candidiasis, seborrheic dermatitis, and eczema.



  • Psoriasis does not involve the vagina, only the vulva.



  • Lichen sclerosus does not involve the vagina, whereas lichen planus may involve the vagina.



  • Vulvar pain (vulvodynia) is one of the most common gynecologic problems, reported by up to 16% of women in the general population; 30% of women will have spontaneous relief of their symptoms without any treatment.



  • Classically the symptoms associated with the urethral diverticulum are extremely chronic in nature and do not resolve with multiple courses of oral antibiotic therapy.



  • Cervical stenosis may occur after loop electrocautery excision procedures (LEEPs). The volume of tissue removed and repeated excisional procedures have been reported to increase the risk for cervical stenosis.



  • Endocervical polyps are smooth, soft, red, fragile masses found most commonly in multiparous women in their 40s and 50s. After the endocervical polyp is removed, endometrial sampling should be performed to diagnose a coexisting endometrial hyperplasia or carcinoma.



  • Endometrial polyps are noted in approximately 10% of women when the uterus is examined at autopsy. Approximately one in four women with abnormal bleeding will have an endometrial polyp.



  • Leiomyomas are the most common benign neoplasms of the uterus. The lifetime prevalence of leiomyomas is greater than 80% among African American women and approaches 70% among white women.



  • Cytogenetically, leiomyomas are chromosomally normal and arise from a single cell (they are clonal). All the cells are derived from one progenitor myocyte.



  • Abnormal bleeding is experienced by a third of women with myomas, the most common pattern being intermenstrual spotting. Women with myomas and abnormal bleeding should be thoroughly evaluated for concurrent causes of bleeding.



  • Adenomyosis is often asymptomatic. If multiple serial sections of the uterus are obtained, the incidence may exceed 60% in women aged 40 to 50 years.



  • Adenomyosis rarely causes uterine enlargement greater than a size that corresponds to 14 weeks’ gestation unless there is concomitant uterine pathologic change.



  • The initial management of a suspected follicular cyst is conservative observation. The majority of follicular cysts disappear spontaneously by either reabsorption of the cyst fluid or silent rupture within 4 to 8 weeks of the initial diagnosis.



  • The practice of aspirating cysts laparoscopically should be limited to cysts that are completely simple and associated with normal CA-125 levels. The intraoperative spillage of malignant cystic tumors should be avoided if possible, although the true risk that spillage poses is unknown.



  • The differential diagnosis of a woman with acute pain and a suspected ruptured corpus luteum cyst includes ectopic pregnancy, a ruptured endometrioma, and adnexal torsion.



  • The treatment of unruptured corpus luteum cysts is conservative. However, if the cyst persists or intraperitoneal bleeding occurs, necessitating operation, the treatment is cystectomy.



  • Drainage or fenestration is effective for follicular cysts and poorly effective for cystadenomas. They will tend to recur. When cysts are drained, it is essential to remember that the cytologic examination of cyst fluid has poor predictive value and poor sensitivity in differentiating benign from malignant cysts.



  • Theca lutein cysts arise from either prolonged or excessive stimulation of the ovaries by endogenous or exogenous gonadotropins or increased ovarian sensitivity to gonadotropins. The condition of ovarian enlargement secondary to the development of multiple luteinized follicular cysts is termed hyperreactio luteinalis. Approximately 50% of molar pregnancies and 10% of choriocarcinomas have associated bilateral theca lutein cysts.



  • Benign ovarian teratomas vary from a few millimeters to 25 cm, may be single or multiple, and are bilateral 10% to 15% of the time. Dermoids are believed to arise during fetal life from a single germ cell. They are 46,XX in karyotype.



  • Operative treatment of benign cystic teratomas is via cystectomy with preservation of as much normal ovarian tissue as possible.



  • Fifty percent of patients with an ovarian fibroma will have ascites if the tumor is greater than 6 cm. The incidence of associated ascites is directly proportional to the size of the tumor.



  • Transitional cell tumors (Brenner tumors) are small, smooth, solid, fibroepithelial tumors of the ovary. They usually occur in women between the ages of 40 and 60 and are predominantly unilateral.



  • Adnexal torsion occurs most commonly in the reproductive years, with the average age of patients being in the mid-20s. Pregnancy predisposes to adnexal torsion.



  • Ovarian tumors are discovered in 50% to 60% of women with adnexal torsion.



  • Abnormal color Doppler flow is highly predictive of torsion of the ovary. However, approximately 50% of women with surgically confirmed adnexal torsion will have a normal Doppler flow study.



  • Conservative surgery, either through the laparoscope or via laparotomy, entails gentle untwisting of the pedicle, possibly cystectomy, and stabilization of the ovary with sutures. Detorsion and fixation of the ovary is a safe procedure that reduces the risk of recurrence.



  • The risk of pulmonary embolus with adnexal torsion is approximately 0.2%. The risk is similar regardless of whether the condition is managed by conservative surgery with untwisting or adnexal removal without untwisting.



Introduction


This chapter reviews benign gynecologic lesions; however, the symptoms and differential diagnoses of these lesions have definite similarity with those of malignant disease. As in many areas of medicine, gynecologic problems do not fall into definitive categories, and those that include malignant disease often overlap with those that include benign disease. When the diagnosis from the history, physical examination, and laboratory tests is clear, management is usually self-evident. When a specific diagnosis is unclear, tissue biopsy may be appropriate. Thus the clinical approach to patient complaints or findings must be broad and not so focused as to prematurely exclude dangerous pathologic conditions within the differential diagnosis.


The discussions in this chapter are arranged anatomically, beginning with the vulva and subsequently covering the vagina, cervix, uterus, oviducts, and ovaries. This chapter does not attempt to be encyclopedic; rather, lesions have been selected based on their clinical importance and prevalence. Therefore extremely rare lesions have been omitted. Because several nonneoplastic abnormalities and lesions present in ways similar to those of benign tumors, this chapter also discusses entities that are not specifically abnormal growths. Clinical problems such as torsion of the ovary, lacerations of the vagina, and hematomas of the vulva are examples of common conditions included in this chapter. Gynecologic infections and associated changes are discussed in Chapter 23 .


The successful clinician uses both deductive and inductive reasoning in making a diagnosis. To master both these techniques, one must be adept at history taking and physical examination and be able to form a complete list of possible causes that may be related to the patient’s complaint. An understanding of the problems discussed in this chapter will be helpful in that endeavor.


Vulva


Urethral caruncle and urethral prolapse


Urethral caruncle and urethral prolapse are conditions that primarily affect postmenopausal women and premenarchal girls. They are thought to occur as a result of decreased estrogen. A urethral caruncle is a small, fleshy mass that occurs at the posterior portion of the urethral meatus. The tissue of the caruncle is soft, smooth, friable, and bright red and initially appears as an eversion of the urethra ( Fig. 18.1 ). Urethral caruncles are generally small, single, and sessile, but they may be pedunculated and grow to be 1 to 2 cm in diameter. Urethral caruncles are believed to arise from an ectropion of the posterior urethral wall associated with retraction and atrophy of the postmenopausal vagina. The growth of the caruncle is secondary to chronic irritation or infection.




Fig. 18.1


Photo of urethral caruncle at the base of the meatus.

(From Cundiff GW, Bent AE. Endoscopic Diagnosis of the Female Lower Urinary Tract. London: WB Saunders; 1999.)


Histologically the caruncle is composed of transitional and stratified squamous epithelium with a loose connective tissue, and often the submucosal layer contains relatively large dilated veins. Caruncles are often subdivided by their histologic appearance into papillomatous, granulomatous, and angiomatous varieties.


They are often secondarily infected, producing ulceration and bleeding. The symptoms associated with urethral caruncles are variable. Many women are asymptomatic, whereas others experience dysuria, frequency, and urgency. Sometimes the caruncle produces point tenderness after contact with undergarments or during intercourse. Ulcerative lesions usually produce spotting on contact more commonly than hematuria. The diagnosis of a urethral caruncle is established by biopsy under local anesthesia because it can appear similar to a neoplasm.


Initial therapy is oral or topical estrogen and avoidance of irritation. If the caruncle does not regress or is symptomatic, it may be destroyed by cryosurgery, laser therapy, fulguration, or operative excision. After operative destruction, a Foley catheter is usually left in place for 48 to 72 hours to prevent urinary retention. Follow-up is necessary to ensure that the patient does not develop urethral stenosis. It is not uncommon for the caruncle to recur. Small, asymptomatic urethral caruncles do not need treatment.


Urethral prolapse is predominantly a disease of the premenarcheal girl, although it can occur in postmenopausal women ( Fig. 18.2 ). Patients may have dysuria; however, most are asymptomatic. The annular rosette of friable, edematous, prolapsed mucosa does not have the bright red color of a caruncle and is easily distinguished from a caruncle because it is circumferential ( ). It may be ulcerated with necrosis or grossly edematous. Therapy for a prolapsed urethra is hot sitz baths and antibiotics to reduce inflammation and infection. Topical estrogen cream is sometimes an effective treatment. In rare cases it may be necessary to excise the redundant mucosa.




Fig. 18.2


Urethral prolapse found incidentally in a 5-year-old girl on a colposcopic examination for suspected abuse with an edematous red collar of tissue surrounding the urethral meatus.

(From Hudson MJ, Swenson AD, Kaplan R, et al. Medical conditions with genital/anal findings that can be confused with sexual abuse. In: Jenny C, ed. Child Abuse and Neglect: Diagnosis, Treatment and Evidence. St. Louis: Elsevier; 2011.)


The differential diagnosis of urethral caruncles includes primary carcinoma of the urethra and prolapse of the urethral mucosa. Malignant lesions are usually hard and irregular in shape and typically are within the urethra itself ( ). Urethral carcinoma is primarily a disease in elderly women. The symptoms of a urethral carcinoma include bleeding, urinary frequency, and dysuria. The majority of urethral carcinomas are of squamous cell origin. Most of these rare carcinomas arise from the distal urethra.


The differential diagnosis of a periurethral mass also includes urethral diverticulum, leiomyoma, vaginal wall inclusion cyst, Skene gland cyst or abscess, and, less commonly, Gartner duct cyst and ectopic ureterocele ( ). These are discussed later in this chapter in the Vagina section.


Cysts


The most common large cyst of the vulva is a cystic dilation of an obstructed Bartholin duct. Bartholin glands open into the vulvar vestibule at about the 5 and 7 o’clock position, distal to the hymenal ring. Bartholin duct cysts and abscesses are fairly common, with a lifetime risk estimated to be 2% ( ). They occur most often during the third decade. Noninflamed cysts contain sterile, clear, mucinous fluid. They do not require treatment unless large enough to cause discomfort. Inflamed cysts may be treated with oral antibiotics or incision and drainage. Lesions in the Bartholin gland can occur as carcinomas, a rare tumor that accounts for 2% to 7% of vulvar carcinomas.


Occasionally the ducts of mucous glands of the vestibule become occluded. The resulting small cysts (usually 0.5 to 2 cm) may be clear, yellow, or blue. Similar small mucous cysts occur in the periurethral region. Wolffian duct cysts or mesonephric cysts are rare, but when they do occur, they are found near the clitoris and lateral to the hymeneal ring. These cysts have thin walls and contain clear serous fluid.


Skene duct cysts are rare, usually small, located on the anterior wall of the vagina along the distal urethra, and may present with symptoms of discomfort or be found on routine examination. These cysts arise secondary to infection and scarring of the small ducts ( Fig. 18.3 ). The differential diagnosis includes urethral diverticula; however, clinically, physical compression of the cyst, unlike compression of a urethral diverticula, should not produce fluid from the urethral meatus. Imaging studies such as magnetic resonance imaging (MRI) and ultrasound may also assist in establishing the diagnosis. Asymptomatic cysts in premenopausal women may be managed conservatively. Treatment is excision with careful dissection to avoid urethral injury.




Fig. 18.3


Skene gland cyst.

(From Shah SR, Nitti VW. Benign vaginal wall masses and paraurethral lesions. In: Nitti VW, ed. Vaginal Surgery for the Urologist. Philadelphia: Elsevier; 2012.)


The most common small vulvar cysts are epidermal (or epidermoid) cysts, which are firm, smooth-surfaced, white, yellow, slightly pink, or skin-colored papules or nodules averaging 0.5 to 2 cm in size ( ). They are most commonly located on the hair-bearing areas. One or several lesions may be present, usually nontender and slow growing. They are firm to shotty in consistency, and their contents are usually under pressure. When noninflamed, they are asymptomatic and no treatment is necessary. If confirmation is needed, incision reveals white, caseous material, like thick cheese. Vulvar epidermal cysts do not have sebaceous cells or sebaceous material identified on microscopic examination but have keratin produced by keratinocytes in the lining of the cyst wall ( ). With rupture or leakage of a cyst, inflammation can occur, necessitating treatment with heat applied locally and possibly incision and drainage. Cysts that become recurrently infected or produce pain should be excised when the acute inflammation has subsided. The typical epidermoid cyst develops from embryonic remnants of an anatomically malformed pilosebaceous unit.


An “inclusion cyst” may arise when bits of epithelium are implanted in the skin during surgery or trauma sufficient to break the skin surface. These may be seen at the site of an episiotomy or obstetric laceration. Large epidermal cysts may be confused with fibromas, lipomas, and hidradenomas.


Nevus


A nevus, commonly referred to as a mole, is a localized nest or cluster of melanocytes. These undifferentiated cells arise from the embryonic neural crest and are present from birth. Many nevi are not recognized until they become pigmented at the time of puberty. Vulvar nevi are one of the most common benign neoplasms in women. As with nevi in other parts of the body, they exhibit a wide range in depth of color, from blue to dark brown to black, and some may be amelanotic. The diameter of most common nevi ranges from a 3 to 10 mm. Grossly a benign nevus may be flat, elevated, or pedunculated. The borders are sharp, the color even, and the shape is symmetric. Dysplastic nevi are commonly 6 to 20 mm with one or more atypical features such as speckling of color, diffuse margination, additional red, white, or blue hues, and asymmetry. Other pigmented lesions in the differential diagnosis include hemangiomas, endometriosis, malignant melanoma, vulvar intraepithelial neoplasia, and seborrheic keratosis.


Vulvar nevi are generally asymptomatic. Most women do not closely inspect their vulvar skin; however, during examination, the use of a mirror held by the patient may facilitate teaching self-vulvar exam. Histologically the lesions are subdivided into three major groups: junctional (a symmetric macule), compound, and intradermal nevi (both papules) ( Fig. 18.4 ).




Fig. 18.4


Vulvar nevi. A, Dome-shaped intradermal nevus. B, Compound nevus with irregular pigmentation.

(From Fisher BK, Margesson LJ. Genital Skin Disorders: Diagnosis and Treatment. St. Louis: Mosby; 1998.)


Melanoma is the second most common malignancy arising in the vulva and accounts for 2% to 3% of all of the melanomas occurring in women, even though the vulva contains approximately 1% of the skin surface area of the body. The incidence of vulvar melanoma is stable or slightly decreasing. It is more common in older, white women, with a mean age at diagnosis of 68 years ( ). It is estimated that 50% of malignant melanomas arise from a preexisting nevus. Family history of melanoma is one of the strongest risk factors for the disease.


Ideally, all vulvar nevi should be excised and examined histologically. Special emphasis should be directed toward the flat junctional nevus and the dysplastic nevus because they have the greatest potential for malignant transformation ( Fig. 18.5 ). The lifetime risk of a woman developing melanoma from a congenital junctional nevus that measures greater than 2 cm in diameter is estimated to be approximately 10%. The lifetime risk of a melanoma forming in women with dysplastic nevi is 15 times that of the general population.




Fig. 18.5


Suprapubic dysplastic nevus with an irregular shape, reddish hue to the edges, and indistinct margins.

(From Fisher BK, Margesson LJ. Genital Skin Disorders: Diagnosis and Treatment. St. Louis: Mosby; 1998.)


Removal may be accomplished with local anesthesia or coincidentally with obstetric delivery or gynecologic surgery. Proper excisional biopsy should be three-dimensional and adequate in width and depth. Approximately 5 to 10 mm of normal skin surrounding the nevus should be included, and the biopsy specimen should include the underlying dermis as well.


Some patients are reluctant to have a “normal” appearing nevus removed. Nevi that are raised or contain hair rarely undergo malignant change. However, if they are often irritated or bleed spontaneously, they should be removed. Recent changes in growth or color, ulceration, bleeding, pain, or the development of satellite lesions mandate biopsy. The characteristic clinical features of an early malignant melanoma may be remembered by thinking ABCD: asymmetry, border irregularity, color variegation, and a diameter usually greater than 6 mm.


Hemangioma


Hemangiomas are rare malformations of blood vessels rather than true neoplasms. Vulvar hemangiomas often are discovered initially during childhood. They are usually single, 1 to 2 cm in diameter, flat, and soft, and they range in color from brown to red or purple. Histologically the multiple channels of hemangiomas are predominantly thin-walled capillaries arranged randomly and separated by thin connective tissue septa. These tumors change in size with compression and are not encapsulated. Most hemangiomas are asymptomatic; occasionally they may become ulcerated and bleed.


There are at least five different types of vulvar hemangiomas. The strawberry and cavernous hemangiomas are congenital defects discovered in young children. The strawberry hemangioma is usually bright red to dark red, is elevated, and rarely increases in size after age 2. Approximately 60% of vulvar hemangiomas discovered during the first years of life spontaneously regress in size by the time the child goes to school. Cavernous hemangiomas are usually purple and vary in size, with the larger lesions extending deeply into the subcutaneous tissue. These hemangiomas initially appear during the first few months of life and may increase in size until age 2. Similar to strawberry hemangiomas, spontaneous resolution generally occurs before age 6. Senile or cherry angiomas are common small lesions that arise on the labia majora, usually in postmenopausal women. They are most often less than 3 mm in diameter, multiple, and red-brown to dark blue. Angiokeratomas are approximately twice the size of cherry angiomas, are purple or dark red, and occur in women between the ages of 30 and 50. They are noted for their rapid growth and tendency to bleed during strenuous exercise. In the differential diagnosis of an angiokeratoma is Kaposi sarcoma and angiosarcoma. Pyogenic granulomas are an overgrowth of inflamed granulation tissue that grow under the hormonal influence of pregnancy, with similarities to lesions in the oral cavity. Pyogenic granulomas are usually small, slightly pedunculated nodules approximately 1 cm in diameter, appearing “pinched in” at the base. They may be mistaken clinically for malignant melanomas, basal cell carcinomas, vulvar condylomas, or nevi. Treatment of pyogenic granulomas involves wide and deep excision to prevent recurrence.


The diagnosis is usually established by gross inspection of the vascular lesion. Asymptomatic hemangiomas and hemangiomas in children rarely require therapy. In adults, initial treatment of large symptomatic hemangiomas that are bleeding or infected may require subtotal resection. When the differential diagnosis is questionable, excisional biopsy should be performed. A hemangioma that is associated with troublesome bleeding may be destroyed by cryosurgery, sclerotherapy, or with the use of lasers. Cryosurgical treatment usually involves a single freeze/thaw cycle repeated three times at monthly intervals. Obviously, if the histologic diagnosis is questionable, any bleeding vulvar mass should be treated by excisional biopsy so that the definitive pathologic diagnosis can be established. Surgical removal of a large, cavernous hemangioma may be technically difficult. Lymphangiomas of the vulva do exist but are extremely rare.


Another rare malformation is the vulvar venous malformation. These lesions may become symptomatic at any age and are relatively prone to thrombosis. Venous malformations are different from vulvar varicosities, which are exacerbated with pregnancy and tend to regress postpartum. There are reports of the successful use of sclerotherapy for the treatment of the malformations.


Fibroma


Fibromas are the most common benign solid tumors of the vulva. They are more common than lipomas, the other common benign tumors of mesenchymal origin. Fibromas occur in all age groups and most commonly are found in the labia majora ( Fig. 18.6 ). However, they actually arise from deeper connective tissue. Thus they should be considered as dermatofibromas. They grow slowly and vary from a few centimeters to one gigantic vulvar fibroma reported to weigh more than 250 pounds. Most are between 1 and 10 cm in diameter. The smaller fibromas are discovered as subcutaneous nodules. As they increase in size and weight, they become pedunculated. Smaller fibromas are firm; however, larger tumors often become cystic after undergoing myxomatous degeneration. Sometimes the vulvar skin over a fibroma is compromised by pressure and ulcerates.




Fig. 18.6


Clinical photograph of a patient showing a pedunculated fibroma from the labia majora.

(From Najam R, Chowdhury HH, Awasthi S. A large fibroma polyp of labia majora—a case report. J Clin Case Rep. 2013;3:297.)


Fibromas have a smooth surface and a distinct contour. On cut surface the tissue is gray-white. Fat or muscle cells microscopically may be associated with the interlacing fibroblasts. Fibromas have a low-grade potential for becoming malignant. Smaller fibromas are asymptomatic; larger ones may produce chronic pressure symptoms or acute pain when they degenerate. Treatment is operative removal if the fibromas are symptomatic or continue to grow. Occasionally they are removed for cosmetic reasons.


Lipoma


Lipomas are the second most common type of benign vulvar mesenchymal tumor. A common hamartoma of fat, lipomas of the vulva are similar to lipomas of other parts of the body. In the vulva, they are most commonly located periclitorally or within the labia majora ( ). When discovered they are softer and usually larger than fibromas ( Fig. 18.7 ). The majority of lipomas in the vulvar region are smaller than 3 cm. The largest vulvar lipoma reported in the literature weighed 44 pounds. They are slow growing, and their malignant potential is extremely low.




Fig. 18.7


Vulvar lipoma arising from the left labia majora.

(From Hasija S, Khoiwal S, Bilwal B. Vulvar lipoma—a rare case report. Am J Med Case Rep. 2015;3(12):413-414.)


When a lipoma is cut, the substance is soft, yellow, and lobulated. Histologically, lipomas are usually more homogeneous than fibromas. Prominent areas of connective tissue occasionally are associated with the mature adipose cells of a true lipoma. Unless extremely large, lipomas do not produce symptoms. Computed tomography and MRI may be used to evaluate tumor extensions and anatomic connections with surrounding structures. MRI has been reported to facilitate the differentiation of vulvar lipomas from vulvar liposarcomas ( ). Excision is usually performed to establish the diagnosis, although smaller tumors may be followed conservatively.


Hidradenoma (mammary-like gland adenoma)


The hidradenoma is a rare, small, benign vulvar tumor thought to be derived from mammary-like glands located in the anogenital area of women ( Fig. 18.8 ). In a review of 46 cases, the tumors occurred only in postpubertal women aged 30 to 90 ( ). Clinically, hidradenoma are small, smooth-surfaced, medium soft to firm nodules found most commonly on the labia majora or labia minora. They appear cystic and are usually asymptomatic; however, some patients report itching, bleeding, and mild pain. Hidradenomas may be cystic or solid, and approximately 50% are less than 1 cm in diameter. These tumors have well-defined capsules and arise deep in the dermis. Histologically, because of its hyperplastic, adenomatous pattern, a hidradenoma may be mistaken at first glance for an adenocarcinoma. On close inspection, however, although there is glandular hyperplasia with numerous tubular ducts, there is a paucity of mitotic figures and a lack of significant cellular and nuclear pleomorphism ( Fig. 18.9 ). Excisional biopsy is the treatment of choice.




Fig. 18.8


Hidradenoma.

(From Shea CH, Stevens A, Dalziel KL, et al. The vulva: cysts, neoplasms, and related lesions. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)



Fig. 18.9


Histologic views: low- and high-power micrographs of hidradenoma.

(From Clement PB, Young RH. Atlas of Gynecologic Surgical Pathology. Philadelphia: WB Saunders; 2000.)


Syringoma


The syringoma is a benign skin adnexal neoplasm thought to be of eccrine origin. It is common on the face and eyelids but unusual on the vulva. In the vulvar area these small, asymptomatic papules (usually less than 5 mm in diameter) are located on the labia majora. The papules are skin colored or yellow and may coalesce to form cords of firm tissue. They may be hormonally active because pregnancy may aggravate associated pruritus and progesterone receptors have been detected in this neoplasm ( ). This tumor is usually treated by excisional biopsy, cryosurgery, or laser therapy. The most common differential diagnosis is Fox-Fordyce disease, a condition of multiple retention cysts of apocrine glands accompanied by inflammation of the skin. The latter disease often produces intense pruritus, whereas syringoma is generally asymptomatic. Fox-Fordyce disease improves with pregnancy and oral contraceptive use and remits after menopause. It is treated with topical steroids, topical tretinoin cream, and oral isotretinoin.


Endometriosis


Endometriosis of the vulva is uncommon. Only 1 in 500 women with endometriosis present with vulvar lesions. The firm, small nodule or nodules may be cystic or solid and vary from a few millimeters to several centimeters in diameter. The subcutaneous lesions are blue, red, or purple, depending on their size, activity, and closeness to the surface of the skin. The gross and microscopic pathologic picture of vulvar endometriosis is similar to endometriosis of the pelvis (see Chapter 19 ). Vulvar adenosis may appear similar to endometriosis. The former condition occurs after laser therapy of condylomata acuminata.


Endometriosis of the vulva is usually found at the site of an old, healed obstetric laceration, an episiotomy site, an area of operative removal of a Bartholin duct cyst, or along the canal of Nuck. The pathophysiology of vulvar endometriosis development may be secondary to metaplasia, retrograde lymphatic spread, or potential implantation of endometrial tissue during operation. In one series, 15 cases of vulvar endometriosis were believed to be associated with prophylactic postpartum curettage performed in 2028 deliveries, since there was not a single case in 13,800 deliveries without curettage. In general, symptoms do not appear for many months after implantation.


The most common symptoms of endometriosis of the vulva are pain and introital dyspareunia. The classic history is cyclic discomfort and an enlargement of the mass associated with menstrual periods. Treatment of vulvar endometriosis is by wide excision or laser vaporization depending on the size of the mass. Recurrences are common after inadequate operative removal of the entire involved area, and as a result most would also recommend medical therapy with continuous oral contraceptives, progestins, or gonadotropin-releasing hormone (GnRH) agonists.


Granular cell myoblastoma


Granular cell myoblastoma is a rare, slow-growing, solid vulvar tumor originating from neural sheath (Schwann) cells and is sometimes called a schwannoma. These tumors are found in connective tissues throughout the body, most commonly in the tongue, and occur in any age group. Approximately 7% of solitary granular cell myoblastomas are found in the subcutaneous tissue of the vulva. Twenty percent of multiple granular cell myoblastomas are located in the vulva. The tumors are usually located in the labia majora but occasionally involve the clitoris.


These tumors are subcutaneous nodules, usually 1 to 5 cm in diameter. They are benign but characteristically infiltrate the surrounding local tissue. The tumors are slow growing, but as they grow, they may cause ulcerations in the skin. The overlying skin often has hyperplastic changes that may look similar to invasive squamous cell carcinoma. Grossly these tumors are not encapsulated, and the cut surface of the tumor is yellow. Histologically, there are irregularly arranged bundles of large, round cells with indistinct borders and pink-staining cytoplasm. Initially the cell of origin was believed to be striated muscle; however, electron microscopic studies have demonstrated that this tumor is from cells of the neural sheath.


The tumor nodules are painless. Treatment involves wide excision to remove the filamentous projections into the surrounding tissue. If the initial excisional biopsy is not sufficiently aggressive, these benign tumors tend to recur. Recurrence occurs in approximately one in five of these vulvar tumors. The appropriate therapy is a second operation with wider margins, as these tumors are not radiosensitive.


Von Recklinghausen disease


The vulva is sometimes involved with the benign neural sheath tumors of von Recklinghausen disease (generalized neurofibromatosis and café-au-lait spots). The vulvar lesions of this disease are fleshy, brownish red, polypoid tumors. Approximately 18% of women with von Recklinghausen disease have vulvar involvement. Excision is the treatment of choice for symptomatic tumors.


Other abnormal tissues presenting as vulvar masses


The differential diagnosis of vulvar masses includes a large array of rare lesions and aberrant tissues, including leiomyomas, squamous papillomas, sebaceous adenomas, dermoids, accessory breast tissue and müllerian or wolffian duct remnants, epidermal inclusion cysts, sebaceous cysts, mucous cysts, and skin diseases such as seborrheic keratosis, condylomata acuminata, and molluscum contagiosum. Some of these diseases are discussed in this chapter, others in Chapter 23 .


Hematomas


Hematomas of the vulva are usually secondary to blunt trauma such as a straddle injury from a fall, an automobile accident, or a physical assault. Traumatic injuries producing vulvar hematomas have been reported secondary to a wide range of recreational activities, including bicycle, motorcycle, and go-cart riding; sledding; water skiing; cross-country skiing; and amusement park rides ( Fig. 18.10 ). Spontaneous hematomas are rare and usually occur from rupture of a varicose vein during pregnancy or the postpartum period.




Fig. 18.10


Traumatic hematoma of the left vulva.

(From Taingson MC, Adze JA, Bature SB, Durosinlorun AM, Caleb M, Amina A. Haematoma of the labia minora following consensual sexual intercourse. Sahel Med J. 2018;21:52-54.)


The management of nonobstetric vulvar hematomas is usually conservative unless the hematoma is greater than 10 cm in diameter or is rapidly expanding. The bleeding that produces a vulvar hematoma is usually venous in origin. Therefore it may be controlled by direct pressure. Compression and application of an ice pack to the area are appropriate therapy. If the hematoma continues to expand, operative therapy is indicated in an attempt to identify and ligate the damaged vessel. Often identification of the “key responsible vein” is a futile operative procedure. However, obvious bleeding vessels are ligated, and a pack is placed to promote hemostasis. During the operation, careful inspection and, if needed, endoscopy are performed to rule out injury to the urinary bladder and rectosigmoid.


The majority of small hematomas regress with time; however, a “chronic expanding hematoma” may become particularly problematic. The most familiar clinical example of this type of problem is the chronic subdural hematoma, but a similar situation may accompany vulvar hematomas. The underlying pathophysiology is repetitive episodes of bleeding from capillaries in the granulation tissue of the hematoma, which result in a chronic, slowly expanding vulvar mass. Treatment of a chronic expanding hematoma is drainage and debridement.


Dermatologic diseases


The skin of the vulva is similar to the skin over any surface of the body and is therefore susceptible to any generalized skin disease or involvement by systemic disease. The most common skin diseases involving the vulva include contact dermatitis, neurodermatitis, psoriasis, seborrheic dermatitis, cutaneous candidiasis, and lichen planus. The majority of vulvar skin problems are red, scalelike rashes, and the woman’s primary complaint is of pruritus. The diagnosis and treatment of these lesions are often obscured or modified by the environment of the vulva. The combination of moisture and heat of the intertriginous areas may produce irritation, maceration, and a wet, weeping surface. Patients will commonly apply ointments and lotions, which may produce secondary irritation. Therefore it is important that the gynecologist examine the skin of the entire body because the patient may have more classic lesions of the dermatologic disease in another location. The skin of the vulva is susceptible to acute infections produced by Streptococcus or Staphylococcus, such as folliculitis, furunculitis, impetigo, and a chronic infection, hidradenitis suppurativa.


The nonspecific symptom complex of vulvar pruritus and burning is presented next as an introduction to the discussion of dermatologic diseases of the vulva.


Pruritus


Pruritus is the single most common gynecologic problem; it is a symptom of intense itching with an associated desire to scratch and rub the affected area. Not uncommonly, secondary vulvar pain develops in association or subsequent to pruritus. In some women pruritus becomes an almost unrelenting symptom, with the development of repetitive “itch-scratch” cycles. The itch-scratch cycle is a complex of itching leading to scratching, producing excoriation and then healing. The healing skin itches, leading to further scratching. Pruritus is a nonspecific symptom. The differential diagnosis includes a wide range of vulvar diseases, including skin infections, sexually transmitted diseases, specific dermatosis, vulvar dystrophies, lichen sclerosus, premalignant and malignant disease; contact dermatitis; neurodermatitis; atrophy; diabetes; drug allergies; vitamin deficiencies; pediculosis, scabies; psychological causes; and systemic diseases such as leukemia and uremia.


The management of pruritus involves establishing a diagnosis, treating the offending cause, and improving local hygiene. For successful treatment the itch-scratch cycle must be interrupted before the condition becomes chronic, resulting in lichenification of the skin (lichen simplex chronicus). Lichenification clinically is recognized by palpably thickened skin, exaggerated skin markings, and lichen-type scale. The resulting dry, scaly skin often cracks, forms fissures, and becomes secondarily infected, thus complicating the treatment (see Chapter 30 ).


Contact dermatitis


The vulvar skin, especially the intertriginous areas, is a common site of contact dermatitis. The vulvar skin is more reactive to exposure by irritants than other skin areas such as the extremities. Contact dermatitis is usually caused by one of two basic pathophysiologic processes: a primary irritant (nonimmunologic) or a definite allergic (immunologic) origin. A large proportion of adult patients with chronic vulvovaginal pruritis are symptomatic because of contact dermatitis. Substances that are irritants produce immediate symptoms such as a stinging and burning sensation when applied to the vulvar skin. The symptoms and signs secondary to an irritant disappear within 12 hours of discontinuing the offending substance. In contrast, allergic contact dermatitis requires 36 to 48 hours to manifest its symptoms and signs.


Allergic contact dermatitis is a cell-mediated delayed-type (type IV) hypersensitivity reaction. There is development of antigen-specific T cells that may return to the skin at the next contact with the allergen. Often the signs of allergic contact dermatitis persist for several days despite removal of the allergen. Rarely, some women will be allergic to latex or semen. These elicit type 1, immunoglobulin E (IgE)–mediated, immediate reactions. Angioedema and urticarial plaques and papules arise within minutes of contact, and anaphylaxis may result.


Excessive cleansing of the vulvar skin and urinary or fecal incontinence may precipitate an irritant dermatitis. The majority of chemicals that produce hypersensitivity of the vulvar skin are cosmetic or therapeutic agents, including vaginal contraceptives, lubricants, sprays, perfumes, douches, fabric dyes, fabric softeners, synthetic fibers, bleaches, soaps, chlorine, dyes in toilet tissues, and local anesthetic creams ( Fig. 18.11 ). External chemicals that trigger the disease process must be avoided. Some of the most severe cases of contact dermatitis involve lesions of the vulvar skin secondary to poison ivy or poison oak. Women with a history of atopy or eczema are more at risk for contact dermatitis and tend to be more sensitive to skin irritations.




Fig. 18.11


Acute contact dermatitis to chlorhexidine. Edema and erythema are present in areas where the antiseptic chlorhexidine solution was applied.

(From Stevens A, Dalziel KL. Vulvar dermatoses. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


Acute contact dermatitis results in a red, edematous, inflamed skin. The skin may become weeping and eczematoid. The most severe skin reactions form vesicles and at any stage may become secondarily infected. The common symptoms of contact dermatitis include superficial vulvar tenderness, burning, and pruritus. Chronic untreated contact dermatitis can evolve into a syndrome of lichenification, with the skin developing a leathery appearance and texture , known as lichen simplex chronicus ( Fig. 18.12 ).




Fig. 18.12


A, Lichen simplex chronicus manifesting of the right labium majus. There is thickening and accentuation of skin markings, with surface excoriation caused by recent scratching. B, Lichen simplex chronicus. The epidermis shows thickening of rete ridges, thickening of the granular layer, and overlying hyperkeratosis.

(From Stevens A, Dalziel KL. Vulvar dermatoses. In Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


The foundation of treatment of contact dermatitis is withdrawal of the offending substance. Sometimes the distribution of the vulvar erythema helps to delineate the irritant. For example, localized erythema of the introitus often results from vaginal medication, whereas generalized erythema of the vulva is secondary to an allergen in clothing. It is possible to use a vulvar chemical innocuously for many months or years before the topical vulvar “allergy” develops.


Once the offending substances and all potential allergens have been eliminated, topical steroids can be applied to the vulva until the skin returns to normal. The vulvar skin should be kept clean and dry. Use of a barrier product such as zinc oxide ointment or vitamin A and D ointment may be needed to keep urine and feces away from the skin in patients with incontinence. The pain and burning can be treated with tepid water bath soaks several times a day for the first few days. Use of a lubricating agent such as petroleum jelly or Eucerin cream will reduce the pruritus by rehydrating the skin and should be applied after the soaks. Cotton undergarments that allow the vulvar skin to aerate should be worn, and constrictive, occlusive, or tight-fitting clothing such as pantyhose should be avoided. Use of a nonmedicated cornstarch baby powder may facilitate vulvar dryness. Hydrocortisone (0.5% to 1%) and fluorinated corticosteroids (Valisone, 0.1%, or Synalar, 0.01%) as lotions or creams may be rubbed into the skin two to three times a day for a few days to control symptoms. Synthetic systemic corticosteroids (prednisone, starting with 50 mg/day for 7 to 10 days in a decreasing dose) are sometimes necessary for treatment of poison ivy and poison oak or severe reactions. Antipruritic medications, such as antihistamines, are not of great therapeutic benefit except as soporific agents. Women often experience pruritus after steroid therapy for vulvar dermatitis. This is not necessarily a recurrence but rather represents a type of withdrawal reaction. This rebound pruritus is seen most commonly with prolonged and higher doses of steroids. After examination, the optimal treatment is a step-down to a short course of a low-potency topical steroid such as 1% hydrocortisone. Topical steroids should be continued for a month or more after clinical improvement because microscopic evidence of inflammation remains for a considerable period ( ).


Psoriasis


Psoriasis is a common, generalized skin disease of unknown origin. Generally, women develop psoriasis during their teenage years, with approximately 3% of adult women affected. Approximately 20% of these cases involve vulvar skin. Similar to candidiasis, psoriasis may be the first clinical manifestation of human immunodeficiency virus (HIV) infection. Psoriasis is chronic and relapsing, with an extremely variable and unpredictable course marked by spontaneous remissions and exacerbations. Twenty-five percent of women have a family history of the disease. Genetic susceptibility to develop psoriasis is believed to be multifactorial. Common areas of involvement are the scalp and fingernails. When psoriasis involves the vulvar skin, it produces both anxiety and embarrassment.


Vulvar psoriasis usually affects intertriginous areas and is manifested by red to red-yellow papules. These papules tend to enlarge, becoming well-circumscribed, dull-red plaques ( Fig. 18.13 ). Though the presence of classic silver scales and bleeding on gentle scraping of the plaques may help establish the diagnosis, the scales are less common in the vulva than on other areas of the body.




Fig. 18.13


A, Psoriasis of perineum and vulva. Flexural psoriasis often lacks the typical parakeratotic scale of psoriasis on other body sites. Painful erosion of the natal cleft is common. B, Psoriasis. There is psoriasiform hyperplasia of rete ridges with papillary dermal edema and telangiectasia. The parakeratotic scale on the skin surface is not prominent in vulvar psoriasis.

(From Stevens A, Dalziel KL. Vulvar dermatoses. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


With psoriasis on the vulvar region, the number of scales is extremely variable and often they are absent. Under the influence of the moisture and heat of the vulva, vulvar psoriasis may resemble candidiasis. Importantly for the diagnosis, psoriasis does not involve the vagina. Sometimes dermatologists treat refractory cases of psoriasis with oral retinoids. The margins of psoriasis are more well defined than the common skin conditions in the differential diagnosis, including candidiasis, seborrheic dermatitis, and eczema. Initial treatment for mild disease is 1% hydrocortisone cream. If the patient has pain secondary to chronic fissures or more moderate disease, a 4-week course of a fluorinated corticosteroid cream should be given. If this treatment is not successful, a dermatologist should be consulted. Several newer antipsoriatic treatments may benefit this condition, especially when it becomes moderate to severe, including vitamin D analogs, topical retinoids, calcineurin inhibitors, salicylic acid, coal tar cyclosporine, and drugs that alter the immune system (biologics). Systemic steroids often produce a rebound flare-up of the disease.


Seborrheic dermatitis


Seborrheic dermatitis is a common chronic skin disease of unknown origin that classically affects the face, scalp, sternum, and the area behind the ears. Rarely, the mons pubis and vulvar areas may be involved. Vulvar lesions are pale to yellow-red, erythematous, and edematous, and they are covered by a fine, nonadherent scale that is usually oily. Excessive sweating and emotional tension precipitate attacks. Although the cause is unknown, an abnormal reaction in the skin to a commensal yeast, Pityrosporum ovale, has been implicated in the pathogenesis. Treatment with topical and oral antifungal agents causes improvement; however, they are not as effective as topical steroids ( ). Approximately 2% to 4% of women have some form of the disease. The pruritus associated with seborrheic dermatitis varies from mild to severe. Treatment is similar to that for contact dermatitis, with hydrocortisone cream being the most effective medication. The differential diagnosis of seborrheic dermatitis includes psoriasis, cutaneous candidiasis, and contact dermatitis. Often it is difficult to differentiate between the cutaneous manifestations of psoriasis and seborrheic dermatitis. Clinically and pragmatically the exact diagnosis is only of academic interest because the treatment is similar.


Lichen planus


Lichen planus is an uncommon vulvovaginal dermatosis. Women complain of soreness, burning, itching, and dyspareunia. The disease presents most commonly as a hypertrophic, coalesced plaque similar to lichen sclerosis. Lichen sclerosis, though, does not involve the vagina, whereas lichen planus can. Three types of vulvar lichen planus have been described: erosive, classical, and hypertrophic. Erosive lichen planus is the most common and is characterized by erosions around the introitus, clitoris, and labia majora and minora ( Fig. 18.14 ). A lacy white edge is commonly seen. Vaginal involvement is common, and patients may also present with contact bleeding, erythema, and scarring with synechiae. Many patients may also report mouth pain and have gingival lesions that appear erosive and desquamative. The classical type presents with small purple, polygonal papules, with sometimes a reticulate lace pattern. Hyperkeratotic lichen planus presents as single or multiple white, hyperkeratotic papules and plaques. Lichen planus is an inflammatory condition with unknown cause; however, evidence suggests it to be an autoimmune disease of cellular immunity ( ). The autoimmune phenomenon can be triggered by certain drugs, including beta-blockers, angiotensin-converting enzyme (ACE) inhibitors, and other medications. It may also arise spontaneously. The correct diagnosis is confirmed by a small punch biopsy of the vagina or vulva. Histologic findings ( Fig. 18.15 ) include degeneration of the basal layers, a lymphocytic infiltrate of the dermis, and epidermal acanthosis.




Fig. 18.14


Lichen planus. A, Eroded ulcers in the vulva. B, Lacy reticulated pattern of lichen planus with periclitoral scarring in a 71-year-old woman who has had oral lichen planus for 10 to 15 years, cutaneous lichen planus of arms and legs for 18 months, and bouts of erosive vaginal lichen planus with scarring and partial vaginal stenosis.

(From Fisher BK, Margesson LJ. Genital Skin Disorders: Diagnosis and Treatment. St. Louis: Mosby; 1998.)



Fig. 18.15


Lichen planus, histologic view. Note hyperkeratosis with extensive basal layer destruction and a dense lichenoid infiltrate at the dermoepidermal junction.

(From Stevens A, Dalziel KL. Vulvar dermatoses. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


This chronic disease tends to have spontaneous remissions and exacerbations that last for weeks to months. Treatment of local lesions is by use of a potent topical steroid ointment such as clobetasol applied twice daily. Steroid suppositories may be inserted intravaginally at night. If the patient is intensely symptomatic, oral steroids may be necessary. In postmenopausal women, topical or systemic estrogen replacement can also be crucial to avoid additional mucosal thinning. Other treatments for resistant cases include methotrexate, oral retinoids, oral griseofulvin, dapsone, azathioprine, cyclophosphamide, and topical cyclosporine. Surgery may be necessary to separate vaginal adhesions or uncover a buried clitoris. Postoperatively the use of vaginal dilators can prevent scar re-formation. Women with this condition should be monitored at periodic intervals because of an associated increased risk of developing vulvar squamous cell carcinoma.


Behçet disease


Behçet disease is a rare disorder initially described as a triad of oral aphthous ulcers, genital aphthous ulcers, and uveitis. It is now known to be a multisystem disease with potential development of problems in many organ systems: skin, joints, cardiovascular, central nervous system, and gastrointestinal tract. The prevalence is high in the Mediterranean region, Middle East, and Japan. Turkey has the highest prevalence, with a rate of 100 to 400 in 100,000 individuals ( ). The diagnosis is made after exclusion of herpetic lesions and other ulcerative diseases. The symptoms respond to topical anesthetics. Severe disease may require antineoplastic therapy including methotrexate, steroids, or other medications.


Hidradenitis suppurativa


Hidradenitis suppurativa is a chronic, unrelenting, refractory infection of skin and subcutaneous tissue that contains apocrine glands. The apocrine glands are found mainly in the axilla and the anogenital region. The disease is rare before puberty; 98% of cases are found in reproductive-age women, and most all disease regresses after menopause. As the infection progresses over time, deep scars and pits are formed ( Fig. 18.16 ). The patient undergoes great emotional distress as this condition is both painful and is associated with a foul-smelling discharge. Theories of the cause of this condition favor an inflammation beginning in the hair follicles ( Fig. 18.17 ). Thus the term sometimes used synonymously is acne inversa. The lesions involve the mons pubis, the genitocrural folds, and the buttocks. The differential diagnosis of hidradenitis suppurativa includes simple folliculitis, Crohn disease of the vulva, pilonidal cysts, and granulomatous sexually transmitted diseases. The differentiation from Crohn disease is usually made by history with an absence of gastrointestinal (GI) involvement. The early phase of the disease involves infection of the follicular epithelium, at first appearing as a boil. This is followed by erythema, involvement of multiple follicles, and chronic infections that burrow and form cysts that break open and track through subcutaneous tissue, creating odiferous and painful sinuses and fistula in the vulva. The chronic scarring, fibrosis, and hyperpigmentation with foul-smelling discharge and soiling of underclothes lead to a socially debilitating condition. The diagnosis should be confirmed by biopsy.




Fig. 18.16


Hidradenitis suppurativa: multiple vulvar abscesses with edema of the mons pubis and labia majora. Notice the “pitting” and “scars” from chronic infection.

(From Amankwah Y, Haefner H. Vulvar edema. Dermatol Clin. 2010;28(4):765-777.)



Fig. 18.17


Hydradenitis suppurativa. Biopsy with follicular plugging and connection to dilated apocrine duct.

(From Kelly P. Folliculitis and the follicular occlusion tetrad. In Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. Edinburgh: Mosby; 2003.)


Early on in the disease process there are small furuncles and folliculitis, for which topical and oral clindamycin is usually effective in the short term, usually requiring a 3-month course of antibiotics. Unfortunately, relapse is common; if treatment with long-term antibiotic therapy and topical steroids is unsuccessful, other medical therapies have included antiandrogens, isotretinoin, and cyclosporine. The treatment of refractory cases is aggressive, wide operative excision of the infected skin.


Edema


Edema of the vulva may be a symptom of either local or generalized disease. Two of the most common causes of edema of the vulva are secondary reactions to inflammation or to lymphatic blockage. Vulvar edema is often recognized before edema in other areas of the female body is noted. The loose connective tissue of the vulva and its dependent position predispose to early development of pitting edema. Systemic causes of vulvar edema include circulatory and renal failure, ascites, and cirrhosis. Vulvar edema also may occur after intraperitoneal fluid is instilled to prevent adhesions or for dialysis. Local causes of vulvar edema include allergy, neurodermatitis, inflammation, trauma, and lymphatic obstruction caused by carcinoma or infection. Infectious diseases that are associated with vulvar edema include necrotizing fasciitis, tuberculosis, syphilis, filariasis, and lymphogranuloma venereum.


Vulvar pain syndromes: Vulvar vestibulitis, vestibulodynia, and dysesthetic vulvodynia


Vulvar pain (vulvodynia) is one of the most common gynecologic problems, reported by up to 16% of women in the general population ( ). Vulvodynia is a pain disorder that occurs without visible findings, infection, inflammation, neoplasia, or a neurologic disorder. The disease has a wide spectrum of symptomology and response to treatment; therefore causation is most likely multifactorial. The diagnosis is made after excluding other treatable causes. A complete history identifying the onset of pain, other associated symptoms, duration of pain, medical and sexual history, treatments tried, allergies, and triggers for pain should be taken. A physical examination with a cotton swab to identify specific areas of pain should be documented. Large population-based studies have noted that symptoms wax and wane, with many women having spontaneous remission (up to 10% of the time).


The terms vulvar pain syndrome, vulvodynia, and vulvar vestibulitis are often used interchangeably. Vulvar vestibulitis is somewhat of a misnomer because it is not inflammation. Vulvar pain syndrome is further subdivided into two categories: vestibulodynia and dysesthetic vulvodynia. The two conditions have a significant amount of overlap, although with different causes and clinical courses. In general, vestibulodynia is found in younger women, most commonly white, with onset shortly after puberty through the mid-20s. Dysesthetic vulvodynia is most common in peri- and postmenopausal women who have rarely if ever had previous vulvar pain.


The differential diagnosis of vulvar pain includes neurologic diseases, herpes simplex infection, chronic infections, abuse, pain syndromes, neoplasia, contact dermatitis, and psychogenic causes. Chronic pain is considered to be part of the vulvodynia spectrum once the diagnoses of infection, invasive disease, and inflammation have been excluded. Severe chronic pain can be socially debilitating, and these patients have a wide spectrum of associated affective symptomology as well. Women with vulvodynia have greater psychological distress than women who have other vulvar problems. Importantly, these psychological concerns must be addressed as part of the therapeutic management.


Vestibulodynia involves the symptom of allodynia, which is hyperesthesia, a pain that is related to nonpainful stimuli. The pain is not present without stimulation. The diagnostic maneuver to establish the presence of allodynia is to lightly touch the vulvar vestibule with a cotton-tipped applicator. The vulvar areas most likely to be affected are from the 4 to 8 o’clock positions along the vulvar-vaginal borders. Erythema is not always present, but when present, it is confined to the vulvar vestibule ( Fig. 18.18 ). Additionally, patients with vestibulodynia experience intolerance to pressure in the vulvar region. This pain is neurogenic in origin. The intolerance to pressure may be caused by tampon use, sexual activity, or tight clothing, and the pain is described as raw and burning. It is not a spontaneous pain; it is invoked. However, it is severe in nature. Some authors have suggested that symptoms be present for at least 6 months before establishing the diagnosis. The symptoms may appear around the time of first intercourse, or within the next 5 to 15 years.




Fig. 18.18


Vulvar vestibulitis. A, Redness localized to the right Bartholin duct opening and, below it, vulvar vestibulitis. B, Discrete localized periglandular erythema in vulvar vestibulitis in a 60-year-old woman.

(From Fisher BK, Margesson LJ. Genital Skin Disorders: Diagnosis and Treatment. St. Louis: Mosby; 1998.)


Studies of women with vulvar vestibulodynia have found no increased incidence of sexual abuse compared with controls. However, many women are found to have erotophobia. Some even noted an increased nerve density and normal estrogen receptors compared with controls. In contrast, other investigators have noted an increase in alpha-estrogen receptors. Theories regarding the cause cite potential immunologic and infectious factors, though no theory has been proved to date. Oral contraceptive use in younger women and hormone replacement in older women have no association with vestibulodynia.


Vulvar dysesthesia, or vulvodynia, is a nonlocalized pain that is constant (not provoked by touch), mimicking a neuralgia. Allodynia is rarely noted, and erythema is also much less common than in vulvar vestibulodynia. Women with vulvodynia are more often perimenopausal or postmenopausal. Dyspareunia is currently present but has usually not been present before the development of dysesthesia. Similar to women with vulvar vestibulodynia, there is not an increased history of sexual abuse compared with controls. Women with dysesthesia also have an increased incidence of chronic interstitial cystitis. In general, both groups of women have an increased incidence of atopy. In some, a history of inflammation from topical agents may be elicited. These agents have usually either been self-prescribed or prescribed by a professional to treat initially what seems to be infection. Patients are often depressed and anxious, but this is thought to be a secondary reaction to the chronic pain. An outline for evaluating these patients is presented in Box 18.1 . Before the diagnosis, one should exclude infection by atypical Candida (which may not be obvious on inspection and should be diagnosed by culture) and by group B Streptococcus. Some would recommend that before extensive treatment a punch biopsy should be obtained to rule out dermatitis presenting atypically, including lichen sclerosis.



BOX 18.1

Evaluation of Patients With Vulvar Pain





  • Examination of vulva for abnormal redness, erosions, crusting, ulceration, hypopigmentation



  • Cotton swab test to identify areas of pain on pressure (e.g., vestibule)



  • Sensory neurologic examination for allodynia and symmetric sensation



  • Examination for vaginal redness, erosions, pallor, dryness



  • Biopsy of specific skin findings for evaluation by dermatopathologist



  • Microscopic evaluation of vaginal secretions for yeast, pH, increased white blood cells



  • Culture for Candida (exclusive of C. albicans ) and bacteria (especially group B Streptococcus )



  • Evaluation for depression and impact on quality of life



  • Classification of vulvar vestibulitis syndrome or dysesthetic vulvodynia




The therapeutic approach for these two conditions emphasizes a sensitivity to the debilitating social aspects of the problem. Similar to other chronic pain syndromes, tricyclic antidepressants or gabapentin have been found to be successful in several series. Doses of gabapentin range from 300 to 3600 mg, usually given with increasing doses every week. Most authors start at 300 mg daily, increase to 300 mg twice daily, then three times a day, then 600 mg three times per day to 900 three times per day, and so on; the average effective dose is approximately 1800 mg daily. Approximately 66% to 75% of women have a response to treatment with gabapentin. When the medication is discontinued, it should be tapered.


Biofeedback and behavior modification therapy have also produced relief. Topical 5% lidocaine ointment has been described as a local treatment option with limited success.


In the past, women with refractory vulvar vestibulitis have been treated with surgical removal of the vulvar vestibule and reapproximation of tissue. The surgery is difficult, with a significant complication rate, but results are generally good. In one series of 126 women with vulvar vestibulitis, the complication rate was 39%; 89% of women felt that the surgery improved their condition enough to recommend it to other women ( ). Importantly, 30% of women have spontaneous relief of their symptoms without any treatment. Reports have indicated that multilevel nerve block given simultaneously for refractory cases has produced some response. Botulinum neurotoxin is also effective in some women, particularly those with concurrent vaginismus and levator ani spasm. Series of treatments and combinations of treatments are often used.


For women with vestibulodynia unresponsive to other therapies, surgery is usually recommended. Vestibulectomy and modified vestibulectomy (partial or limited from 3 to 9 o’clock) have resulted in resolution in 60% to 90% of patients compared with 40% to 80% for nonsurgical interventions ( ). Surgery has been noted to be most effective in younger women. Some advocate for partial vestibulectomy because most pain and painful skin occur in the lower half of the vestibule. Complications from vestibulectomy include occlusion of the Bartholin gland, leading to development of cysts. This problem requires surgical “unroofing” of the duct.


Vagina


Urethral diverticulum


A urethral diverticulum is a permanent, epithelialized, saclike projection that arises from the posterior urethra. Most are thought to be acquired and occur in women between ages 30 and 60 years ( ). It often presents as a mass on the anterior vaginal wall, and urethral diverticula represent approximately 84% of periurethral masses ( Table 18.1 ). It is a common problem, being discovered in approximately 1% to 3% of women. Most urogynecologists have noted a decline in the prevalence of this condition since the early 1990s. The majority of cases are initially diagnosed in reproductive-age women, with the peak incidence in the fourth decade of life. The symptoms of a urethral diverticulum are nonspecific and are identical to the symptoms of a lower urinary tract infection. To diagnose this elusive condition, one should suspect urethral diverticulum in any woman with chronic or recurrent lower urinary tract symptoms. The urologic aspects of this condition are discussed in Chapter 21 . Histologically the diverticulum is lined by epithelium; however, there is a lack of muscle in the saclike pocket.



TABLE 18.1

Final Diagnosis of Periurethral Mass and Frequency

From Blaivas JG, Flisser AJ, Bleustein CB, Panagopoulos G. Periurethral masses: diagnosis in a large series of women. Obstet Gynecol. 2004; 103(5 Pt 1):842-847.




































Diagnosis N (%) 95% Confidence Interval (%)
Urethral diverticulum 66 (84) 73,91
Diverticulum with malignancy 4 (6) 2,14.8
Vaginal cyst 6 (7) 3,15
Leiomyoma 4 (5) 1,12
Vaginal squamous cell carcinoma 2 (2.5) 0.03,8.8
Ectopic ureter 2 (2.5) 0.03,8.8
Granuloma 1 (1) 0.03,6.8


Urethral diverticula may be congenital or acquired. Few urethral diverticula are present in children; therefore it is assumed that most diverticula are not congenital. The anatomy of the urethra has been described as a tree with many stunted branches that represent the periurethral ducts and glands. It is assumed that the majority of urethral diverticula result from repetitive or chronic infections of the periurethral glands. The suburethral infection may cause obstruction of the ducts and glands, with subsequent production of cystic enlargement and retention cysts. These cysts may rupture into the urethral lumen and produce a suburethral diverticulum. Persistent inflammation and stasis can lead to stone formation (10%). Malignancy has been reported in 6% to 9% of cases, mostly adenocarcinoma ( ). Urethral diverticula are small, from 3 mm to 3 cm in diameter. The majority of urethral diverticula open into the midportion of the urethra ( Table 18.2 ). Occasionally, multiple suburethral diverticula occur in the same woman.



TABLE 18.2

Location of the Ostium in 108 Female Patients With Diverticulum of the Urethra

From Lee RA. Diverticulum of the urethra: clinical presentation, diagnosis, and management. Clin Obstet Gynecol. 1984;27:490-498.






















Site Number of Patients
Distal (external) third of the urethra 11
Middle third of the urethra 55
Proximal (inner) third of the urethra (including vesical neck) 18
Multiple sites 18
Unknown 6


Classically the symptoms associated with the urethral diverticulum are extremely chronic in nature and have not resolved with multiple courses of oral antibiotic therapy. The most common symptoms associated with urethral diverticula are urinary urgency and frequency and dysuria, which is the presenting symptom in about 90% of cases. Approximately 15% of women with urethral diverticula experience hematuria. Other authors have stressed the three D s associated with a diverticulum: dysuria, dyspareunia, and dribbling of the urine. Although for years, postvoiding dribbling has been termed a classic symptom of urethral diverticulum, it is reported by fewer than 10% of women with this condition. In Lee’s series a palpable, tender mass was discovered in 56 of 108 patients ( ). It is interesting that in most large series, approximately 20% of the women are asymptomatic. A classic sign of a suburethral diverticulum is the expression of purulent material from the urethra after compressing the suburethral area during a pelvic examination. Although the sign of producing a discharge by manual expression is specific, its sensitivity is poor.


The foundation of diagnosing urethral diverticulum is the physician’s awareness of the possibility of this defect occurring in women with chronic symptoms of lower urinary tract infection. Historically the two most common methods of diagnosing urethral diverticulum have been the voiding cystourethrography and cystourethroscopy. Approximately 70% of urethral diverticula will be filled by contrast material on a postvoiding radiograph with a lateral view. Cystourethroscopy will demonstrate the urethral opening of the urethral diverticulum in approximately 6 of 10 cases. Other diagnostic tests used to identify urethral diverticula include urethral pressure profile recordings, transvaginal ultrasound, computed tomography (CT) scans, MRI, and positive-pressure urethrography. For diagnosis of urethral diverticulum, MRI sensitivity is 100% because the resolution is excellent ( ). Ultrasonography, done translabially (or introitally), may assist in the assessment of the mass as cystic or solid. Positive-pressure urethrography is done with a special double-balloon urethral catheter (Davis catheter) ( Fig. 18.19 ). Classically the recordings of the pressure profile of the urethra demonstrate a biphasic curve in a woman with a urethral diverticulum. If a woman has a urethral diverticulum and urinary incontinence, performing a stress urethral pressure profile will help differentiate the cause. The differential diagnosis includes Gartner duct cyst, an ectopic ureter that empties into the urethra, and Skene glands cysts.




Fig. 18.19


Double-balloon catheter in use for positive-pressure urethrography.


Several different operations can correct urethral diverticula. Excisional surgery should be scheduled when the diverticulum is not acutely infected. Operative techniques can be divided into transurethral and transvaginal approaches, with most gynecologists preferring the transvaginal approach as described by Lee ( ). The majority of diverticula enter into the posterior aspect of the urethra. Diverticula of the distal one-third may be treated by simple marsupialization. After operations, approximately 80% of patients obtain complete relief from symptoms. Some diverticula have multiple openings into the urethra. Complete excision of this network of fistulous connections is important. The recurrence rate varies between 10% and 20%, and many failures are due to incomplete surgical resection. The most serious consequences of surgical repair of urethral diverticula are urinary incontinence and urethrovaginal fistula. Postoperative incontinence usually follows operative repairs of large diverticula that are near the bladder neck. This incontinence may be secondary to damage to the urethral sphincter. The incidence of each of these complications is approximately 1% to 2%.


Inclusion cysts


Inclusion cysts are the most common cystic structures of the vagina. In a series of 64 women with cystic masses of the vagina, 34 had inclusion cysts ( ). The cysts are usually discovered in the posterior or lateral walls of the lower third of the vagina. Inclusion cysts vary from 1 mm to 3 cm in diameter. Similar to inclusion cysts of the vulva, inclusion cysts of the vagina are more common in parous women. Inclusion cysts usually result from birth trauma or gynecologic surgery. Often they are discovered in the site of a previous episiotomy or at the apex of the vagina after hysterectomy.


Histologically, inclusion cysts are lined by stratified squamous epithelium. These cysts contain a thick, pale yellow substance that is oily and formed by degenerating epithelial cells. Often these cysts are erroneously called sebaceous cysts in the misbelief that the central material is sebaceous. Similar to vulvar inclusion cysts, the cause is either a small tag of vaginal epithelium buried beneath the surface after a gynecologic or obstetric procedure or a misplaced island of embryonic remnant that was destined to form epithelium.


The majority of inclusion cysts are asymptomatic. If the cyst produces dyspareunia or pain, the treatment is excisional biopsy.


Dysontogenetic cysts


Dysontogenetic cysts of the vagina are thin-walled, soft cysts of embryonic origin. Whether the cysts arise from the mesonephros (Gartner duct cyst), the paramesonephricum (müllerian cyst), or the urogenital sinus (vestibular cyst) is predominantly of academic rather than clinical importance. The cysts may be differentiated histologically by the epithelial lining ( Fig. 18.20 ). Most mesonephric cysts have cuboidal, nonciliated epithelium. Most perimesonephric cysts have columnar, endocervical-like epithelium. Occasionally pressure produced by the cystic fluid produces flattening of the epithelium, which makes histologic diagnosis less reliable. Although most commonly single, dysontogenetic cysts may be multiple. Usually the cysts are 1 to 5 cm in diameter and are discovered in the upper half of the vagina ( Fig. 18.21 ). Sometimes multiple small cysts may present like a string of large, soft beads. A large cyst presenting at the introitus may be mistaken for a cystocele, anterior enterocele, or obstructed aberrant ureter. Approximately 1 in 200 women develop these cysts.




Fig. 18.20


Histologic examination of a Gartner duct cyst from the lateral vaginal wall. The cyst is lined by nonciliated cells.

(From Clement PB, Young RH. Atlas of Gynecologic Surgical Pathology. Philadelphia: WB Saunders; 2000.)



Fig. 18.21


A, Normal mesonephric duct. On cross section it is a single duct in the submucosa surrounded by clusters of smooth muscle bands. B, Mesonephric duct. The mother duct, located deep in the wall of the vagina, is surrounded by smaller arborized offshoots. C, Elongated mesonephric duct.

(From Robboy SJ, Anderson MC, Russell P, et al. The vagina. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


Embryonic cysts of the vagina, especially those discovered on the anterior lateral wall, are usually Gartner duct cysts. In the embryo the distal portion of the mesonephric duct runs parallel with the vagina. It is assumed that a segment of this embryonic structure fails to regress, and the obstructed vestigial remnant becomes cystic. These cysts are most commonly found in the lower one-third of the vagina.


Most of these benign cysts are asymptomatic, sausage-shaped tumors that are discovered only incidentally during pelvic examination. Small asymptomatic Gartner duct cysts may be followed conservatively. In a series of 25 women undergoing operations for symptomatic dysontogenetic cysts, a wide range of symptoms were reported, including dyspareunia, vaginal pain, urinary symptoms, and a palpable mass. Sometimes large cysts interfere with the use of tampons. MRI can be useful in delineating the course and anatomic arrangement of vaginal cysts ( ).


Operative excision is indicated for chronic symptoms. Rarely, one of these cysts becomes infected, and if operated on during the acute phase, marsupialization of the cyst is preferred. Excision of the vaginal cyst may be a much more formidable operation than anticipated. The cystic structure may extend up into the broad ligament and anatomically be in proximity to the distal course of the ureter.


Rare tumors of the vagina include fibromas, angiomyxomas, and hemangiomas. All are usually found by the patient and require surgical excision.


Tampon problems


The vaginal tampon has achieved immense popularity and ubiquitous use. It is not surprising that there are rare associated risks with tampon usage: vaginal ulcers, the “forgotten” tampon, and toxic shock syndrome. The latter, related to toxins elaborated by Staphylococcus aureus, is discussed in Chapter 23 .


Wearing tampons for a few days has been associated with microscopic epithelial changes. The majority of women develop epithelial dehydration and epithelial layering, and some will develop microscopic ulcers. These minor changes take between 48 hours and 7 days to heal. Using colposcopy to evaluate the vaginal epithelium after tampon use, Friedrich found serial changes of epithelial drying, peeling, layering, and ultimately microulceration in 15% of women wearing tampons only during the time of normal menstruation. No clinical symptoms were associated with these microscopic changes. Theoretically these microulcerations are a potential portal of entry for HIV.


Large macroscopic ulcers of the vaginal fornix have been described in women using vaginal tampons for prolonged times for persistent vaginal discharge or spotting. The ulcers have a base of clean granulation tissue with smooth, rolled edges. One can even find tampon fibers in the biopsy specimens of these ulcers. The pathophysiology of the ulcer is believed to be secondary to drying and pressure necrosis induced by the tampon. Obviously, many of these young women use tampons for the identical symptoms that are associated with a vaginal ulcer—that is, spotting and vaginal discharge. Often the intermenstrual spotting is believed to be breakthrough bleeding from oral contraceptives, and the possibility of a vaginal ulcer from chronic tampon usage is overlooked.


Vaginal ulcers are not uncommon secondary to several types of foreign objects, including diaphragms, pessaries, and medicated silicon rings. Management is conservative because the ulcers heal spontaneously when the foreign object is removed. Any persistent ulcer should be biopsied to establish the cause.


A woman with a “lost” or “forgotten” tampon presents with a classic foul vaginal discharge and occasionally spotting. The tampon is usually found high in the vagina. The odor from a forgotten tampon is overwhelming. The tampon is removed using a “double glove technique” where two gloves are donned on the removal hand and, on grasping the tampon, the outer glove is pulled over the tampon and tied as the tampon is removed. The woman should be treated with an antibiotic vaginal cream or gel (such as metronidazole or clindamycin) for the next 5 to 7 days.


Local trauma


The most common cause of trauma to the lower genital tract of adult women is coitus. Approximately 80% of vaginal lacerations occur secondary to sexual intercourse. Other causes of vaginal trauma are straddle injuries, penetration injuries by foreign objects, sexual assault, vaginismus, and water-skiing accidents. The management of vulvar and vaginal trauma in children is discussed in Chapter 12 .


The predisposing factors believed to be related to coital injury include virginity, the state of the postpartum and postmenopausal vaginal epithelium, pregnancy, intercourse after a prolonged period of abstinence, hysterectomy, and inebriation. In one series of 19 injuries from normal coitus, 12 of the women were between the ages of 16 and 25 and 5 were older than 45 ( ). The most common injury is a transverse tear of the posterior fornix. Similar linear lacerations often occur in the right or left vaginal fornices. The location of the coital injury is believed to be related to the poor support of the upper vagina, which is supported only by a thin layer of connective tissue. The most prominent symptom of a coital vaginal laceration is profuse or prolonged vaginal bleeding. Many women experienced sharp pain during intercourse, and 25% noted persistent abdominal pain. The most troublesome but extremely rare complication of vaginal laceration is vaginal evisceration. Coital injury to the vagina should be considered in any woman with profuse or prolonged abnormal vaginal bleeding. Sensitive but thorough history regarding abuse is always appropriate.


Management of coital lacerations involves prompt suturing under adequate anesthesia. Secondary injury to the urinary and gastrointestinal tracts should be ruled out.


Cervix


Endocervical and cervical polyps


Endocervical and cervical polyps are the most common benign neoplastic growths of the cervix, reported in 4% of gynecologic patients. Endocervical polyps are most common in multiparous women in their 40s and 50s. Cervical polyps usually present as a single polyp, but multiple polyps do occur occasionally. The majority are smooth, soft, reddish purple to cherry red, and fragile. They readily bleed when touched. Endocervical polyps may be single or multiple and are a few millimeters to 4 cm in diameter. The stalk of the polyp is of variable length and width ( Fig. 18.22 ). Polyps may arise from either the endocervical canal (endocervical polyp) or ectocervix (cervical polyp). Endocervical polyps are more common than are cervical polyps. Often the terms endocervical and cervical polyps are used to describe the same abnormality. Polyps whose base is in the endocervix usually have a narrow, long pedicle and occur during the reproductive years, whereas polyps that arise from the ectocervix have a short, broad base and usually occur in postmenopausal women.




Fig. 18.22


Cervical polyp. A large polyp protrudes from the external cervical os. The surface is red and rough, covered by endocervical epithelium.

(From Anderson MC, Robboy SJ, Russell P, et al. The cervix—benign and non-neoplastic conditions. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


The hypothesis of the origin of endocervical polyps is that they are usually secondary to inflammation or abnormal focal responsiveness to hormonal stimulation. Focal hyperplasia and localized proliferation are the response of the cervix to local inflammation. The color of the polyp depends in part on its origin, with most endocervical polyps being cherry red and most cervical polyps grayish white.


The classic symptom of an endocervical polyp is intermenstrual bleeding, especially after contact such as coitus or a pelvic examination. Sometimes an associated leukorrhea emanates from the infected cervix. Many endocervical polyps are asymptomatic and recognized for the first time during a routine speculum examination. Often the polyp seen on inspection is difficult to palpate because of its soft consistency.


Histologically the surface epithelium of the polyp is columnar or squamous epithelium, depending on the site of origin and the degree of squamous metaplasia ( Fig. 18.23 ). The stalk is composed of an edematous, inflamed, loose, and richly vascular connective tissue. Six different histologic subtypes have been described: adenomatous, cystic, fibrous, vascular, inflammatory, and fibromyomatous. Greater than 80% are of the adenomatous type. During pregnancy, focal areas of decidual changes may develop in the stroma. Often there is ulceration of the stalk’s most dependent portion, which explains the symptom of contact bleeding. Malignant degeneration of an endocervical polyp is extremely rare; the reported incidence is less than 1 in 200. Considerations in the differential diagnosis include endometrial polyps, small prolapsed myomas, retained products of conception, squamous papilloma, sarcoma, and cervical malignancy. Microglandular endocervical hyperplasia sometimes presents as a 1- to 2-cm polyp. This is an exaggerated histologic response, usually to oral contraceptives.




Fig. 18.23


Cervical polyp. The stroma is fibromuscular and the base contains thick-walled blood vessels. Endocervical crypts, some dilated, are present within the polyp.

(From Anderson MC, Robboy SJ, Russell P, et al. The cervix—benign and non-neoplastic conditions. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


Most endocervical polyps may be managed in the office by grasping the base of the polyp with an appropriately sized clamp. The polyp is avulsed with a twisting motion and sent to the pathology laboratory for microscopic evaluation. The polyp is usually friable. If the base is broad or bleeding ensues, the base may be treated with chemical cautery, electrocautery, or cryocautery. After the polyp is removed, the endometrium should be evaluated in women older than 40 who have presented with abnormal bleeding, to rule out coexisting pathologic changes because a significant endometrial pathologic condition is found in approximately 5% of asymptomatic women with endocervical polyps.


Nabothian cysts


Nabothian cysts are retention cysts of endocervical columnar cells occurring where a tunnel or cleft has been covered by squamous metaplasia. These cysts are so common that they are considered a normal feature of the adult cervix. Many women have multiple cysts. Grossly these cysts may be translucent or opaque whitish or yellow in color, and they vary from microscopic to macroscopic size, with the majority between 3 mm and 3 cm in diameter. Rarely, a woman with several large nabothian cysts may develop gross enlargement of the cervix. These mucous retention cysts are produced by the spontaneous healing process of the cervix. The area of the transformation zone of the cervix is in an almost constant process of repair, and squamous metaplasia and inflammation may block the cleft of a gland orifice. The endocervical columnar cells continue to secrete, and thus a mucous retention cyst is formed. Nabothian cysts are asymptomatic, and no treatment is necessary.


Lacerations


Cervical lacerations may occur during obstetric deliveries. Obstetric lacerations vary from minor superficial tears to extensive full-thickness lacerations at 3 and 9 o’clock, respectively, which may extend into the broad ligament. Lacerations may occur in nonpregnant women with mechanical dilation of the cervix. The atrophic cervix of the postmenopausal woman increases the risk of cervical laceration when the cervix is mechanically dilated for dilation and curettage (D&C) or hysteroscopy.


Acute cervical lacerations bleed and should be sutured. Cervical lacerations that are not repaired may give the external os of the cervix a fish-mouthed appearance; however, they are usually asymptomatic. The use of laminaria tents to slowly soften and dilate the cervix before mechanical instrumentation of the endometrial cavity has reduced the magnitude of iatrogenic cervical lacerations. Furthermore, the practice of routine inspection of the cervix after every second- or third-trimester delivery has enabled physicians to discover and repair extensive cervical lacerations. Extensive cervical lacerations, especially those involving the endocervical stroma, may lead to incompetence of the cervix during a subsequent pregnancy.


Cervical myomas


Cervical myomas are smooth, firm masses that are similar to myomas of the fundus ( Figs. 18.24 and 18.25 ). A cervical myoma is usually a solitary growth in contrast to uterine myomas, which, in general, are multiple. Depending on the series, 3% to 8% of myomas are categorized as cervical myomas. Because of the relative paucity of smooth muscle fibers in the cervical stroma, the majority of myomas that appear to be cervical actually arise from the isthmus of the uterus.




Fig. 18.24


Large fibroid originating from the lateral wall of the cervix and growing into the broad ligament.

(Courtesy Fidel A. Valea, MD.)


Most cervical myomas are small and asymptomatic. When symptoms do occur, they are dependent on the direction in which the enlarging myoma expands. The expanding myoma produces symptoms secondary to mechanical pressure on adjacent organs. Cervical myomas may produce dysuria, urgency, urethral or ureteral obstruction, dyspareunia, or obstruction of the cervix. Occasionally a cervical myoma may become pedunculated and protrude through the external os of the cervix. These prolapsed myomas are often ulcerated and infected. A very large cervical myoma may produce distortion of the cervical canal and upper vagina. Rarely a cervical myoma causes dystocia during childbirth.


The diagnosis of a cervical myoma is by inspection and palpation. Grossly and histologically, cervical myomas are identical to and indistinguishable from myomas of the corpus of the uterus. Occasionally the histologic picture of cervical myomas will demonstrate many hyalinized, thick-walled blood vessels that are postulated to be the source of the neoplastic smooth muscle tumor. This latter subtype of cervical myoma is termed a vascular leiomyoma. Management is similar to that of uterine myomas in that asymptomatic, small myomas may be observed for rate of growth. The occurrence and persistence of symptoms from a cervical myoma are an indication for medical therapy with GnRH agonists or myomectomy or hysterectomy, depending on the patient’s age and future reproductive plans. Because of both a complex blood supply and involvement with the distal course of the ureter, treatment of cervical myomas that grow laterally may become a challenge if myomectomy is the operation of choice. Cervical myomas may be treated by radiologic catheter embolization. Prolapsed uterine myomas are discussed later in this chapter.


Cervical stenosis


Cervical stenosis most often occurs in the region of the internal os and may be divided into congenital or acquired types. The causes of acquired cervical stenosis are operative, radiation induced, infectious, neoplastic, or atrophic changes. Loop electrocautery excision procedure (LEEP), cone biopsy, and cautery of the cervix (either electrocautery or cryocoagulation) are the operations most commonly associated with cervical stenosis, which often depends on the volume of tissue removed. The symptoms of cervical stenosis depend on whether the patient is premenopausal or postmenopausal and whether the obstruction is complete or partial. Common symptoms in premenopausal women include dysmenorrhea, pelvic pain, abnormal bleeding, amenorrhea, and infertility. The infertility is usually associated with endometriosis, which is commonly found in reproductive-age women with cervical stenosis. Postmenopausal women are usually asymptomatic for a long time. Slowly they develop a hematometra (blood), hydrometra (clear fluid), or pyometra (exudate).


The diagnosis is established by inability to introduce a 1- to 2-mm dilator into the uterine cavity. If the obstruction is complete, a soft, slightly tender, enlarged uterus is appreciated as a midline mass, and ultrasound examination demonstrating fluid within the uterine cavity. Management of cervical stenosis is dilation of the cervix with dilators under ultrasound guidance. If stenosis recurs, monthly laminaria tents may be used. Similarly, office follow-up and sounding of the cervix of women who have had a cone biopsy or cautery of the cervix is important to establish patency of the endocervical canal. Postmenopausal women with pyometra usually do not need antibiotics. After the acute infection has subsided, endometrial carcinoma and endocervical carcinoma should be ruled out by appropriate diagnostic biopsies. After cervical dilation, it is often useful to leave a T tube or latex nasopharyngeal airway as a stent in the cervical canal for a few days to maintain patency.


Uterus


Ultrasound


Ultrasound, primarily endovaginal, is the most common and most efficient imaging technique for pelvic structures. For endovaginal ultrasound, transducers are configured on vaginal probes and placed in a sterile sheath, usually a glove or condom, before an examination. During the examination the woman is in a dorsal lithotomy position and has an empty bladder. Because the transducer is closer to the pelvic organs than when a transabdominal approach is employed, endovaginal resolution is usually superior. However, if the pelvic structures to be studied have expanded and extend into the patient’s abdomen, the organs are difficult to visualize with an endovaginal probe. Most ultrasound machines are equipped with both types of transducers.


For transabdominal gynecologic examinations, a sector scanner is preferable. It provides greater resolution of the pelvis and an easier examination than the linear array. During abdominal pelvic ultrasound examination, it is helpful for the patient to have a full bladder. This serves as an acoustic window for the high-frequency sound waves. Ultrasound is more than 90% accurate in recognizing the presence of a pelvic mass, but it does not establish a tissue diagnosis.


Ultrasonography employs an acoustic pulse echo technique. The transducer of the ultrasound machine is made up of piezoelectric crystals that vibrate and emit acoustic pulses. Acoustic echoes return from the tissues being scanned and cause the crystals to vibrate again and release an electric charge. A computer within the ultrasound machine then integrates the electric charges to form the image. Present equipment provides resolution of less than 0.2 mm.


Doppler ultrasound techniques assess the frequency of returning echoes to determine the velocity of moving structures. Measurement of diastolic and systolic velocities provides indirect indices of vascular resistance. Muscular arteries have high resistance. Newly developed vessels, such as those arising in malignancies, have little vascular wall musculature and thus have low resistance. Three-dimensional ultrasound is a computer technique in which multiple two-dimensional images are compiled to render either a surface- or volume-based image that appears to occupy space, as opposed to being flat. Three-dimensional ultrasound has of yet not been shown to have a specific diagnostic advantage in gynecology compared with other modalities.


A disadvantage of ultrasound is its poor penetration of bone and air; thus the pubic symphysis and air-filled intestines and rectum often inhibit visualization. Advantages of ultrasound include the real-time nature of the image, the absence of radiation, the ability to perform the procedure in the office before, during, or immediately after a pelvic examination, and the ability to describe the findings to the patient while she is watching. One of the most reassuring aspects of sonography is the absence of adverse clinical effects from the energy levels used in diagnostic studies.


Sonographic evaluation of endometrial pathologic changes involves measurement of the endometrial thickness or stripe. The normal endometrial thickness is 4 mm or less in a postmenopausal woman not taking hormones. The thickness varies in premenopausal women at different times of the menstrual cycle and in women taking hormone replacement ( Fig. 18.26 ), making endometrial thickness measurements less reliable in that setting. The endometrial thickness is measured in the longitudinal plane, from outer margin to outer margin, at the widest part of the endometrium. Ultrasound is not a screening tool in asymptomatic women. However, several studies of postmenopausal women with vaginal bleeding have documented that malignancy is extremely rare in women with an endometrial thickness of 4 mm or less. Systematic reviews have noted that ultrasound may be reliably used to predict 96% to 99% of endometrial cancers in women with postmenopausal bleeding. The flip side of the coin is that 1% to 4% of malignancies will be missed using a cutoff of less than 4 mm ( ). In addition, papillary-serous adenocarcinomas of the endometrium do not always develop the same endometrial stripe thickness as endometrioid cancer. Two caveats for using ultrasound in screening of postmenopausal bleeding are (1) ultrasound does not provide a diagnosis—a tissue specimen is necessary for a diagnosis; and (2) all women with bleeding, no matter the endometrial thickness, should have a tissue biopsy. If an endometrial biopsy obtains inadequate tissue and the endometrial thickness is 5 mm or greater, a repeat biopsy, hysteroscopically directed biopsy, or curettage should be performed.




Fig. 18.26


Variation in endometrium during menstrual cycle. A, Early proliferative phase. B, Late proliferative phase. C, Periovulatory phase. D, Late secretory phase. Note increase in endometrial thickness throughout the menstrual cycle. Also note multilayered appearance in the late proliferative phase.

(From Fleischer AC, Kepple DM. Benign conditions of the uterus, cervix, and endometrium. In: Nyberg DA, Hill LM, Bohm-Velez M, et al, eds. Transvaginal Ultrasound. St. Louis: Mosby–Year Book; 1992.)


Sonohysterography is an easily accomplished and validated technique for evaluating the endometrial cavity. The technique involves instilling saline into the uterine cavity. Sonohysterography is an alternative to office hysteroscopy. In this procedure a thin balloon-tipped catheter or intrauterine insemination catheter is inserted through the cervical os, and 5 to 30 mL of warmed saline is slowly injected into the uterine cavity. Meta-analyses of sonohysterography have found the procedure to be successful in obtaining information in 95% of women, with minimal complications. Contraindications are active cervical or uterine infection. Some clinicians will have patients take a dose of ibuprofen before the procedure. Preferably, sonohysterography is performed in the proliferative phase of the cycle when the endometrial lining is at its lowest level. Sonohysterography has also been helpful in the evaluation of polyps, filling defects, submucous myomas, and uterine septae ( Fig. 18.27 ). Importantly, sonohysterography, as with all types of ultrasound, does not make a tissue diagnosis.




Fig. 18.27


Sonohysterograms. A, Well-defined, round echogenic polyp. B, Carpet of small polyps. C, Polyp on a stalk. D, Polyp with cystic areas. E, Small polyp. F, Small polyp. G, Hypoechoic submucosal fibroid. H, Hypoechoic attenuating submucosal fibroid. I, Endometrial adhesions. Note bridging bands of tissue within fluid-filled endometrial canal.

(From Salem S. The uterus and adnexa. In Rumack CM, Wilson SR, Charboneau JW, eds. Diagnostic Ultrasound. 2nd ed. St. Louis: Mosby; 1998:538.)


Sonography is the method of choice to locate a “missing” intrauterine device (IUD). It will help in diagnosing perforation of the uterus or unrecognized expulsion of the device. Endovaginal ultrasound transducers equipped with needle guides are often used for oocyte aspiration as part of in vitro fertilization.


In summary, ultrasound has become an extremely valuable adjunct to the bimanual examination. In many patients, particularly those with obesity, it is superior to perform bimanual examination alone. An endovaginal ultrasound of an early pregnancy has become a mainstay in the evaluation of the pregnant woman with first-trimester vaginal bleeding.


Endometrial polyps


Endometrial polyps are localized overgrowths of endometrial glands and stroma that project beyond the surface of the endometrium. They are soft, pliable, and may be single or multiple. Most polyps arise from the fundus of the uterus. Polypoid hyperplasia is a benign condition in which numerous small polyps are discovered throughout the endometrial cavity. Endometrial polyps vary from a few millimeters to several centimeters in diameter, and it is possible for a single large polyp to fill the endometrial cavity. Endometrial polyps may have a broad base (sessile) or be attached by a slender pedicle (pedunculated). They occur in all age groups but have a peak incidence between the ages of 40 and 49. The prevalence of endometrial polyps in reproductive-age women is 20% to 25%, and they are noted in approximately 10% of women when the uterus is examined at autopsy. The cause of endometrial polyps is unknown. Because polyps are often associated with endometrial hyperplasia, unopposed estrogen has been implicated as a possible cause.


The majority of endometrial polyps are asymptomatic. Those that are symptomatic are associated with a wide range of abnormal bleeding patterns. No single abnormal bleeding pattern is diagnostic for polyps; however, menorrhagia, premenstrual and postmenstrual staining, and scanty postmenstrual spotting are the most common. Occasionally a pedunculated endometrial polyp with a long pedicle may protrude from the external cervical os. Sometimes large endometrial polyps may contribute to infertility.


Polyps are succulent and velvety, with a large central vascular core. The color is usually gray or tan but may occasionally be red or brown. Histologically, an endometrial polyp has three components: endometrial glands, endometrial stroma, and central vascular channels ( Fig. 18.28 ; see Fig. 18.27 ). Epithelium must be identified on three sides, like a peninsula. Approximately two out of three polyps consist of an immature endometrium that does not respond to cyclic changes in circulating progesterone. This immature endometrium differs from surrounding endometrium and often appears as a “Swiss cheese” cystic hyperplasia during all phases of the menstrual cycle ( Fig. 18.29 ). The other one-third of endometrial polyps consist of functional endometria that will undergo cyclic histologic changes. The tip of a prolapsed polyp often undergoes squamous metaplasia, infection, or ulceration. The clinician cannot distinguish whether the abnormal bleeding originates from the polyp or is secondary to the commonly coexisting endometrial hyperplasia. Approximately one in four reproductive-age women with abnormal bleeding will have endometrial polyps discovered in her uterine cavity.




Fig. 18.28


Endometrial polyp. A, Note cystic glands in the polyp. B, The fibrous stroma of the polyp contrasts with the cellular stroma of the adjacent endometrium.

(From Anderson MC, Robboy SJ, Russell P, et al. Endometritis, metaplasias, polyps, and miscellaneous changes. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)



Fig. 18.29


Endometrial polyp showing multiple cystic glands with flattened epithelial lining.

(From Anderson MC, Robboy SJ, Russell P, et al. Endometritis, metaplasias, polyps and miscellaneous changes. In: Robboy SJ, Anderson MC, Russell P, eds. Pathology of the Female Reproductive Tract. Edinburgh: Churchill Livingstone; 2002.)


Malignancy in an endometrial polyp is related to patient’s age and is most often of a low stage and grade. In one series of 67 women from the United Kingdom with endometrial polyps, 86% were benign, 13% hyperplastic, and 3% malignant. Another series of 61 women with polyps found 88% were benign and 5% were malignant. In a review and meta-analysis of the oncogenic potential of reported endometrial polyps, the prevalence of premalignant or malignant polyps was 5.42% in postmenopausal women compared with 1.7% in reproductive-age women. Furthermore, the prevalence of endometrial neoplasia within polyps in women with symptomatic bleeding was 4.15% compared with 2.16% for those without bleeding. Among symptomatic postmenopausal women with endometrial polyps, 4.47% had a malignant polyp compared with 1.51% in asymptomatic postmenopausal women ( ). The question of an association of endometrial polyps with endometrial carcinoma is still debated. A population-based, case-control study from Sweden estimated that the increased risk of subsequent endometrial carcinoma in women with endometrial polyps is only twofold. It is interesting that benign polyps have been found in approximately 20% of uteri removed for endometrial carcinoma.


Unusual polyps have been described in association with chronic administration of the nonsteroidal antiestrogen tamoxifen. The endometrial abnormalities associated with chronic tamoxifen therapy include polyps, 20% to 35%; endometrial hyperplasia, 2% to 4%; and endometrial carcinoma, 1% to 2%; and often with multiple irregular sonolucencies suggesting the presence of cysts.


Most endometrial polyps are asymptomatic, and the diagnosis is not usually established until the uterus is opened after hysterectomy for other reasons. Endometrial polyps may be discovered by vaginal ultrasound, with or without hydrosonography, hysteroscopy, or hysterosalpingography, during the diagnostic workup of a woman with a refractory case of abnormal uterine bleeding or pelvic mass. Endometrial polyps are often confused with endocervical polyps ( Fig. 18.30 ). A well-defined, uniformly hyperechoic mass that is less than 2 cm in diameter, identified by vaginal ultrasound within the endometrial cavity, is usually a benign endometrial polyp (see Fig. 18.27 , AC ). Most endometrial polyps usually resolve after a few years, although new polyps can form.


Aug 8, 2021 | Posted by in GYNECOLOGY | Comments Off on Benign gynecologic lesions: Vulva, Vagina, Cervix, Uterus, Oviduct, Ovary, Ultrasound Imaging of Pelvic Structures
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