Radical hysterectomy with en bloc total vaginectomy is rarely performed in gynecologic oncology; however, both radical hysterectomy and vaginectomy are separately considered classic procedures. The first hysterectomy with resection of lateral parametria was described in 1895 by Clark.¹ However, lymphadenectomy was not part of this procedure. Three years later, Wertheim performed the first radical hysterectomy in combination with pelvic lymphadenectomy in Austria.² Wertheim’s early mortality rate was about 30%, but this decreased quickly in time, with a cumulative experience of 10% in his report of 500 operations published in 1911.³ In parallel to the abdominal approach to radical hysterectomy, Schauta developed a vaginal technique, which was first published in 1908.⁴ Both approaches, abdominal and vaginal, form the current basis for the radical surgical treatment of cervical cancer. The surgical principles were modified during the twentieth century by many other surgeons; Amreich made meaningful contributions to the vaginal approach, while Wertheim’s abdominal procedure has been expanded upon by Latzko, Okabayashi, and Meigs.⁵

Indications

The most frequent contemporary indication for radical hysterectomy with en bloc vaginectomy is early-stage (FIGO I–II) vaginal cancer, provided the disease is localized to the proximal part of the vagina and is invading into the paracolpium and parametrium.^6,7 This procedure is also indicated for the clinical scenario with combined pathology consisting of early-stage cervical cancer (FIGO I-IIA) and vaginal intraepithelial neoplasia (VaIN). VaIN is often multifocal, and an underlying invasive cancer is reported in nearly one-third of cases.⁸ Although more conservative treatment modalities are usually preferred, especially in patients who are younger, partial or total vaginectomy is a treatment of choice in women who are not sexually active, in recurrent lesions, or in multifocal dispersive lesions involving the entire vagina. Less common indications described in the literature include cervical embryonal rhabdomyosarcoma,⁹ recurrent endometrial cancer in the upper vagina,¹⁰ or clear cell carcinoma of the cervix or vagina.^11,12,13

Considering radical hysterectomy as an individual procedure, the indications are not entirely uniform. One commonly accepted indication for radical hysterectomy is the treatment of cervical cancer stage IB1, in which radical hysterectomy has been associated with excellent oncologic outcomes. Primary surgical treatment is also feasible and has a satisfactory oncologic outcome, according to available data, for locally advanced cervical cancer tumors of stages IB2, IIA, and selected patients with stage IIB disease.^14,15 However, the major limitation of the surgical approach in locally advanced stages is the high proportion of patients with lymph node involvement, which may be as high as 40% for patients with stage IIB disease, and is a requirement for adjuvant radiotherapy.¹⁶ The requirement for 2 treatment modalities—extensive surgical procedure and adjuvant radiotherapy—is associated with a substantial increase in the risk of postoperative morbidity. An alternative treatment modality for bulky cervical tumors or tumors with parametrial invasion is primary chemoradiation. There are no data available comparing directly the morbidity and oncologic outcome of surgery and radiotherapy.

Radical hysterectomy with or without vaginectomy may be indicated for other disease processes, depending on the extent of local disease. The role of parametrial resection with hysterectomy is not well established in locally advanced endometrial cancer. Radical hysterectomy is fully advocated only in tumors invading into the parametria, while, in stage II endometrial cancer with the invasion limited to cervical stroma, a survival benefit of radical hysterectomy over simple hysterectomy has not been established.¹⁷ In clinical practice, most patients with endometrial cancer and stage II disease are referred for adjuvant radiotherapy, which likely abolishes any benefit from surgical removal of parametria. Radical hysterectomy may also be a necessary part of the debulking procedure in ovarian cancer surgery if there is an invasion of carcinomatosis into the broad ligament and lateral parametria. Radical hysterectomy is occasionally required to ensure satisfactory excision of non-oncologic disease process such as severe endometriosis or extensive tubo-ovarian abscess.

Classification

Even though radical hysterectomy with en bloc vaginectomy is performed in 1 step and results in 1 specimen removed en bloc, in terms of procedure performance it essentially consists of 2 different surgeries: radical hysterectomy and vaginectomy. Therefore, both procedures must be separately classified.

The key parameter for differentiating among the types of radical hysterectomy is the extent of parametrial resection. Due to the location of autonomic nerves in the caudal parts of the parametria, it is largely the vertical (deep parametrial) resection that determines late morbidity, particularly bladder and rectal dysfunctions.^18,19,20,21 The extent of resection should be precisely defined for all 3 parts of the parametria (ventral, lateral, dorsal) in 3 planes (sagittal, frontal, and transverse). The classification system described in this chapter is based on the proposal published by Querleu and Morrow²² and later extended into three dimensions.²³ Five types of the procedure (A, B, C1, C2, D) coincide with other common historical types of radical hysterectomy (Table 3-1). Because type A corresponds with the extrafascial hysterectomy and type D, aiming at the resection of internal iliac vessels, is rarely used; differentiating between types B, C1, and C2 is clinically the most relevant.

The type B procedure corresponds with modified radical hysterectomy. The main resection margin is located ventrally and laterally at the ureter. Identification of autonomic nerves is not required, because the nerves remain unexposed in the parametria and the hypogastric plexus is fully preserved. The ureter is identified in the parametrium, unroofed, dissected from the cervix and laterally displaced. The objective of this type of radical hysterectomy is the resection of just a small initial part of the medial leaf of the ventral parametria and 1 to 1.5 cm of the lateral and dorsal parametria.

The major intention of the type C1 procedure is to remove adequate parts of the ventral, lateral and dorsal parametria, while concurrently preserving the major autonomic nerves comprised of the inferior hypogastric plexus and splanchnic nerves. The inferior hypogastric plexus runs in the lateral part of the dorsal parametrium, laterally around the cervix below the ureter at the level of the vaginal fornix and ventrally in the infra-ureteral part of the ventral parametrium towards the urinary bladder.^18,19,20 It receives branches of the splanchnic nerves localized at the bottom of the lateral pararectal space and in the caudal part of the lateral parametrium below the parametrial veins. The surgical margins of this nerve sparing procedure must therefore be kept above the course of nerve structures in the ventral, lateral and dorsal parametrium. The radicality of the C2 procedure is substantially different, with the aim being to remove the majority of all three parametrial components. The resection margins are extended caudally to the sacral bone and ventrally to the urinary bladder wall. As a consequence, major branches of the autonomic nerves are sacrificed during the C2 type procedure.

The classification of the vaginectomy procedure is simpler than radical hysterectomy. Considering the anatomic relationship of the vagina to other organs such as the urinary bladder, the rectum and the urethra, no option exists for increasing the radicality of resection in the sagittal plane. The length of the resected vagina can be adjusted, either described by the length of the proximal vagina that should be excised (eg, 3-cm excision of proximal vagina), as a proximal vaginectomy (proximal one-half of the vagina), or as a total vaginectomy (complete excision of the vagina to the introitus). In cases in which other associated organs must be removed together with the vagina (urinary bladder, rectum, urethra) the procedure is more accurately classified as pelvic exenteration.

Clinical Staging

Radical hysterectomy alone or in combination with vaginectomy is a demanding procedure, which may have serious long-term consequences for the patient. Therefore, prior to undertaking surgery, all efforts should be made to exclude distant metastases, which, if present, will dictate an alteration in treatment strategy. Positron emission tomography scanning is currently the most accurate method in detecting extrapelvic tumor spread from cervical cancer. Preoperative imaging is helpful for local staging of disease in the pelvis to exclude invasion into other surrounding organs, including the urinary bladder, ureters, rectum, and large vessels. In case of their involvement, exenterative surgery is required to achieve complete tumor removal; however, primary chemoradiation is the typical treatment of choice. Magnetic resonance imaging (MRI) is considered to be the gold standard in local staging due to high soft-tissue contras.²⁴ Recently, it has been shown that ultrasonography may have comparable accuracy with MRI, particularly in local staging of cervical cancer.^25,26,27 The accuracy of ultrasonography is improved with the use of power Doppler, which may help to detect abundant tumor vascularization and dynamic examination, enabling the differentiation of tumor or organ borders due to its movement aroused by the pressure of the probe inserted transvaginally, transrectally, or on the abdominal wall. Although cystoscopy and proctoscopy are commonly recommended for exclusion of adjacent organ invasion from the cervix, these procedures cannot identify the tumor involvement into the organ walls until the whole thickness of the wall has been permeated and in most cases they do not bring any additional information beyond MRI or ultrasonography. If a lesion is present in the vagina, then vaginoscopy is important to allow exact description of the lesion location.

Performance Status, Body Mass Index, and Nutritional Status Assessment

There are no strict requirements for an upper age limit or performance status for radical hysterectomy with vaginectomy. At the same time, one must be cognizant that this surgery is always challenging for the surgeon and can be associated with major complications for the patient. Therefore, a preoperative Gynecologic Oncology Group performance status 0 or 1 is required in our department. Technical difficulty can be further increased by various factors, including the size of the tumor, its localization (ie, its attachment to pelvic diaphragm or to large vessels), and obesity. A high body mass index (BMI) does not mean that surgery is contraindicated, but rather that the risks and feasibility should be carefully considered in combination with other factors. In patients with significant visceral obesity, it is usually more favorable to perform a larger part of the operation (complete vaginectomy) from a perineal approach.

In patients with vaginal cancer or locally advanced cervical cancer, significant nutritional deficiency is only rarely manifested. However, severe malnutrition is associated with significantly increased morbidity; therefore, a comprehensive assessment of nutritional status is an essential part of preoperative preparation. Different laboratory tests and scoring systems are used for the assessment; the authors use serum albumin (< 3 g/dL) and Malnutrition Universal Screening Tool score in their department. Preoperative total parenteral nutrition for 5 to 7 days may be considered for patients who are severely malnourished.²⁸

Antibiotic Prophylaxis, Bowel Preparation, Thromboembolic Prophylaxis

Antibiotic prophylaxis is justified in all patients with cancer who are candidates for radical pelvic surgery and should be administered no longer than 30 minutes prior to incision. The choice of the regimen should be directed according to the epidemiological situation in each particular institution. In general, ampicillin or a second-generation cephalosporin is used as first-line prophylaxis, while doxycycline or clindamycin can be used in patients with penicillin allergy. A single dose is sufficient, but only if the procedure does not last beyond 3 hours or there is no excessive blood loss (> 1500 mL); in such cases, the dose should be repeatedly administered at appropriate intervals on the basis of the half-life of the antibiotic.

Bowel surgery is not a routine part of radical hysterectomy with vaginectomy; however, when the dorsal parametria or posterior vaginal wall has been affected, then the dissection of the tumor may be associated with a risk of rectum perforation. For this reason, mechanical bowel preparation was previously recommended for all patients scheduled for vaginectomy or type C2 radical hysterectomy. This practice is largely being abandoned according to critical analyses of available data showing that bowel preparation is not required, even in patients with anticipated bowel procedure.²⁹ Preoperative bowel preparation has not been shown to reduce the risk of anastomotic leak or contamination of the surgical field and may have adverse events, including renal failure.

In general, patients with pelvic cancer undergoing surgery are at increased risk of deep venous thrombosis and pulmonary embolism. Thromboembolic prophylaxis must be employed in all candidates for surgery, including both mechanic and pharmacologic methods. Most often, the combination of compression stocking and low-molecular weight heparin dosed according to BMI, starting 2 hours prior to surgery and continuing at least 7 days postoperatively. Occasionally, an inferior vena cava filter should be preoperatively placed for a patient with a recent or current thromboembolism.

Preoperative Procedure Description

Each part of the procedure and its extent (radicality) should be carefully specified prior to the procedure. The preoperative plan should contain a description of the following:

1. 
   

   Lymph node staging (ie, pelvic lymphadenectomy/sentinel lymph node (SLN) biopsy/low or complete paraaortic lymphadenectomy/removal of bulky pelvic nodes)

   
   
2. 
   

   Procedure on adnexa (ie, bilateral salpingo-oophorectomy (BSO)/adnexa transposition)

   
   
3. 
   

   Type of parametrectomy (B, C1, C2)

   
   
4. 
   

   Length of vaginal resection (ie, proximal vaginectomy, total vaginectomy)

   
   
5. 
   

   Any reconstructive procedures

Procedure Performance

The patient is positioned in the low dorsal lithotomy position using Allen-type stirrups to facilitate vaginal exposure should a perineal phase of the operation be required. The abdominal approach is initiated through a low vertical midline or transverse incision, depending on the anticipated scope of resection and patient body habitus. A self-retaining retractor is placed and the pelvis and abdomen are explored to exclude the presence of extrapelvic metastatic disease.

Radical hysterectomy with en bloc vaginectomy usually begins with pelvic lymphadenectomy, with or without previous sentinel lymph node biopsy, to ascertain the pelvic lymph node status as well as to define the anatomy of the central pelvis and sidewalls. The ureters are identified and tagged, and the paired paravesical and pararectal spaces are developed using a combination of blunt and sharp dissection to expose the external and internal (hypogastric) vascular systems (Figure 3-1). In Figure 3-1, the uterus is retracted medially to show the right lateral parametria stretching between the paravesical and pararectal spaces. The uterine artery and superior vesicle artery are skeletonized, doubly suture ligated, and divided at their origin, just proximal to the continuation of the umbilical ligament. In this fashion, the vessels and associated parametrium are completely resected at their origin from the internal iliac artery, reflected medially, and the remaining attachments divided along the medial aspect of the internal iliac vein up to the sacral bone. Thus, the paravesical and pararectal spaces are unified; the combined space resulting from connecting the paravesical and pararectal spaces is shown in Figure 3-2, which illustrates complete resection of the lateral parametria from the pelvic sidewall (surgical resection is the blue dotted line). Next, the vesicovaginal space is further developed and the urinary bladder is sharply dissected from the cervix and upper vagina deep into the anterior pelvis. Figure 3-3 demonstrates the position of the distal ureter running within the ureteral tunnel in the lateral parametria and the ureteral entrance into the bladder ventrally, running on the ventral vaginal wall. For a complete ventral parametrial resection, the ureter must be completely dissected from the cervix as well as from the ventral parametria. Figure 3-4 demonstrates the completely freed ureter and the resection line on the lateral parametria as it is opened above the ureter. In the next step, the ureter is retracted, and the ventral parametrium is skeletonized and resected on the bladder wall, which is best identified by palpation (Figure 3-5). After mobilization and resection of the ventral parametrium, the ureter is completely free from its surrounding attachments, the lateral bladder wall is exposed, and the lateral margins of resection are clearly defined (Figure 3-6).