Sarcoma of vulva, vagina and ovary

Less than 5% of vulvar, vaginal and ovarian malignant diseases are sarcomas. Adequate knowledge of these particular malignant diseases is essential for accurate diagnosis and for choice of surgical treatment, adjuvant therapy and efficient medical treatment in relapse. A crucial aspect in the management of women with these diseases is a multidisciplinary approach. Globally, presenting signs and symptoms of these sarcomas are non-specific of histological type but linked to initial location. In view of this, management should be undertaken by clinicians experienced in these particular malignancies. Long-term side-effects, particularly in children with sarcoma, adversely affect quality of life. New treatment strategies require special attention.

Introduction

Sarcomas are rare tumours (1–2% of all cancers) of mesenchymal origin that may develop in soft tissues and viscera. More precisely, primary soft-tissue sarcomas account for less than 2–3% of all malignant tumours occurring in the female genital tract. The most common gynaecological sarcoma is uterine sarcoma (90% of cases). Other gynaecological sarcomas include vulva, vagina, ovary, fallopian tubes and uterine ligaments sarcomas (10%). As the International Classification of Disease attributes visceral sarcomas to the organ of origin, the incidence of sarcoma is grossly underestimated.

Adequate knowledge of these particular malignant diseases is essential for accurate diagnosis and for choice of surgical treatment, adjuvant therapy and efficient medical treatment in relapse. These malignancies should be treated as sarcomas and not as carcinomas. A multidisciplinary approach is crucial in the management of these women; this includes pathologists, radiologists, surgeons, gynaecologists, radiotherapists and medical oncologists.

In this chapter, we review all aspects of vulva, vagina and ovary sarcomas, including epidemiology and treatment.

Sarcoma of vulva

Invasive vulvar carcinoma is a rare disease that accounts for about 5% of gynaecologic cancers. Sarcomas of the vulva are extremely rare, and account for only 1–3% of all vulvar malignancies. The most common vulvar sarcomas are leiomyosarcomas, rhabdomyosarcomas, angiosarcomas, neurofibrosarcomas, malignant fibrohistiocytomas, and aggressive angiomyxoma. Few cases of gastrointestinal stromal tumours can also be found, and have been described in case reports of the female genital tract.

Median age at diagnosis is 50 years. Lesions are frequently misdiagnosed as Bartholin’s cysts or abscesses, and the most common presenting symptom is local discomfort with a vulvar mass, chronic vulvar pruritus, or both, and pain with intercourse.

Prognosis seems to depend on three main characteristics: lesion size, tumour involvement with adjacent tissue, and mitotic activity. Disease in women with lesions greater than 5 cm in diameter, with infiltrating margins, extensive necrosis, and with more than five mitotic figures per 10 high-power fields, is most likely to recur after surgical resection.

Surgery remains the mainstay of treatment for vulvar sarcomas. Radical vulvectomy carries a high risk of short- and long-term morbidity. A limited wide local excision, however, may result in positive or close margins requiring the use of adjuvant radiation, particularly for high-grade lesions. Issues related to multimodality treatment of soft-tissue sarcoma in gynaecologic malignancies are, therefore, discussed. Investigators such as Holloway et al. have described a comprehensive multimodality treatment plan that includes preoperative intensity modulated radiotherapy, conservative surgery with flap reconstruction, interstitial brachytherapy boost, and postoperative systemic chemotherapy in one case of synovial cell sarcoma. This strategy resulted in good cosmetic and functional outcome in a sexually active female.

Sarcoma of vulva

Invasive vulvar carcinoma is a rare disease that accounts for about 5% of gynaecologic cancers. Sarcomas of the vulva are extremely rare, and account for only 1–3% of all vulvar malignancies. The most common vulvar sarcomas are leiomyosarcomas, rhabdomyosarcomas, angiosarcomas, neurofibrosarcomas, malignant fibrohistiocytomas, and aggressive angiomyxoma. Few cases of gastrointestinal stromal tumours can also be found, and have been described in case reports of the female genital tract.

Median age at diagnosis is 50 years. Lesions are frequently misdiagnosed as Bartholin’s cysts or abscesses, and the most common presenting symptom is local discomfort with a vulvar mass, chronic vulvar pruritus, or both, and pain with intercourse.

Prognosis seems to depend on three main characteristics: lesion size, tumour involvement with adjacent tissue, and mitotic activity. Disease in women with lesions greater than 5 cm in diameter, with infiltrating margins, extensive necrosis, and with more than five mitotic figures per 10 high-power fields, is most likely to recur after surgical resection.

Surgery remains the mainstay of treatment for vulvar sarcomas. Radical vulvectomy carries a high risk of short- and long-term morbidity. A limited wide local excision, however, may result in positive or close margins requiring the use of adjuvant radiation, particularly for high-grade lesions. Issues related to multimodality treatment of soft-tissue sarcoma in gynaecologic malignancies are, therefore, discussed. Investigators such as Holloway et al. have described a comprehensive multimodality treatment plan that includes preoperative intensity modulated radiotherapy, conservative surgery with flap reconstruction, interstitial brachytherapy boost, and postoperative systemic chemotherapy in one case of synovial cell sarcoma. This strategy resulted in good cosmetic and functional outcome in a sexually active female.

Sarcoma of vagina

Carcinomas of the vagina are rare, accounting for only about 2–3% of gynaecologic malignancies. More commonly, the vagina is a site of metastasis or direct extension from tumours originating in other genital sites such as the cervix or endometrium, or from extragenital sites, including the rectum and bladder. About 3% of vaginal cancers are sarcomas; about two-thirds of these are leiomyosarcomas, but endometrial stromal sarcomas, malignant mixed Müllerian tumours, and others have been also reported. A particular histological sub-group is represented by rhabdomyosarcoma (sarcoma botryoides). This is a highly malignant mesenchymal tumour accounting for 15–20% of malignant disease in childhood, and is the most common soft-tissue tumour affecting children. Incidence is about 250 new cases every year in the USA. Rhabdomyosarcoma arising in the female genital tract (vulva, vagina, cervix and uterus) is rare, and accounts for less than 4% of all paediatric rhabdomyosarcomas. This tumour usually forms soft nodules that fill and protrude from the vagina.

About 50–60% of women with invasive cancer present with abnormal vaginal bleeding, frequently after coitus or vaginal douching. Women may also present with complaints of vaginal discharge, a palpable mass, dyspareunia, or pain in the perineum or pelvis.

With rhabdomyosarcomas, the prognosis for children with this tumour has improved with the use of multimodality treatment, including surgery, chemotherapy and radiation. Rhabdomyosarcoma of the genital tract seems to be a highly curable disease. Nevertheless, an important issue in this population is the incidence of complications. Major long-term complications can potentially impair genitourinary function and adversely affect fertility. More recently, some investigators have reported on the psychological effects of women who have undergone treatment for childhood cancer, and on the effect of such treatments on their quality of life. That is why careful long-term follow up is essential to record prospectively all long-term treatment-related adverse effects. Because the cure rates for childhood cancers continue to improve, the incidence and management of long-term consequences are a constant challenge.

Brachytherapy has the advantage of affecting a smaller tissue volume than external-beam radiotherapy, thereby restricting the amount of healthy tissue affected and potentially reducing later growth and functional impairment. Brachytherapy can also be regarded as an alternative to surgery when hysterectomy or colpectomy has to be carried out. In the Institut Gustave Roussy, the ballistic advantage of brachytherapy has been clearly shown in this situation, with a high-dose gradient sparing normal tissue and high tumour dose. The low local relapse rates highlight the role of brachytherapy in controlling residual disease after initial chemotherapy. By decreasing the treated volume and restricting brachytherapy to tumour remnants after initial chemotherapy, the long-term sequelae were significantly reduced without compromising local control. That is why a multidisciplinary approach with more efficacious multidrug chemotherapy, conservative surgery and limited radiotherapy, such as brachytherapy, is necessary.

Globally, surgery for sarcomas of vulva and adult sarcomas of vagina is crucial in the treatment of high-risk, short- and long-term morbidity. A limited wide local excision, however, may result in positive or close margins requiring multimodality treatment of soft-tissue sarcoma in gynaecologic malignancies.

Sarcomas of ovary

Epithelial ovarian cancer is the leading cause of death from gynaecologic cancers in Western countries. Most women present with advanced disease (i.e. stage III and IV), and are managed with surgical resection followed by platinum-based chemotherapy. During the past decade, advances in chemotherapy have resulted in improved survival and in more effective treatment of relapsed disease. Five-year overall survival, however, remains relatively low at around 30%. Non-epithelial ovarian cancers are rare; their natural history is poorly understood and prognostic factors remain unclear. In fact, from March 2002 and June 2009, the French GINECO group ran an “Observatory for rare malignant tumours of the ovaries” on their website and included their first 100 patients.

Ovarian sarcoma is clearly a distinct entity compared with ovarian adenocarcinoma: older mean age of onset, an inferior response to platinum-based chemotherapy, and worse progression-free and cause-specific survival. Global management remains unclear. In the ‘Observatory for rare malignant tumours of the ovaries’ from the GINECO group, two cases of primary fibrosarcomas of ovary were clearly identified, and four women were registered in the clear cell, sarcomas or small cell carcinomas group.

Pure rhabdomyosarcomas of the ovary are extremely rare, and are lethal primary neoplasms. Few cases of primary rhabdomyosarcomas of the ovary have been reported. Most reported cases were of embryonal rhabdomyosarcomas and in children. The age of individuals at diagnosis ranged between 13 months and 86 years, with 60% of women aged 40 years and older. About 50% of the individuals were diagnosed with disease beyond the ovary, and survival ranged between 18 days and 15 months after diagnosis.

Primary ovarian angiosarcomas represent an extremely rare entity. The tumour is usually unilateral, with uncertain histogenesis. It may originate from the vascular tissue present in the ovary or from a teratoma, in which the vascular component has overgrown other parts of the tumour. Women usually have symptoms related to the presence of a lower abdominal mass, which may be associated with torsion of the tumour and haemorrhage. Twenty-eight cases have been reported; 11 women in the early stages of disease; six stage III and seven stage IV disease. The remaining four women had unknown stage of disease. Even though overall survival for all women ranged from 18 days to 9 years, 12 out of 13 women with stage III and stage IV disease died within 1 year of diagnosis. Most common treatment approaches for women with advanced-stage disease include full staging surgery (if possible) and a variety of chemotherapy regimens based on doxorubicin plus ifosfamide or paclitaxel. Nevertheless, the median overall survival achieved in women with advanced-stage disease is 6–7 months. Taking into account the reported results, a doxorubicin-based regimen still remains the gold standard for those angiosarcomas that arise below the clavicle. Additional data concerning antiangiogenic treatments will provide new insights into the treatment of this neoplasm.

A special relatively well-studied entity is the carcinosarcoma (also termed malignant mixed mesodermal tumours, sarcomatoid carcinomas or malignant mixed Müllerian tumours) of the ovary. Histologically, carcinosarcomas are epithelial tumours that are composed of carcinomatous and sarcomatous elements. The sarcomatous part contains elements that resemble endometrial stromal sarcoma, fibrosarcoma or leiomyosarcoma. Clearly, surgery remains the standard approach, and the extent of the benefit from adjuvant and initial chemotherapy is unclear to date.

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Nov 9, 2017 | Posted by in OBSTETRICS | Comments Off on Sarcoma of vulva, vagina and ovary

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