Vaginal Cancer

Malignant diseases of the vagina are either primary vaginal cancers or metastatic from adjacent or distant organs. Primary vaginal cancers are defined as arising solely from the vagina with no involvement of the external cervical os superiorly or the vulva inferiorly. The importance of this definition lies in the different clinical approach in the treatment of upper and lower vaginal cancer. According to the International Federation of Gynecology and Obstetrics (FIGO), a vaginal lesion involving the external os of the cervix should be considered cervical cancer and treated as such; a tumor involving both vulva and vagina should be considered vulvar cancer. A patient with history of a preinvasive lesion or an invasive carcinoma arising from the cervix or the vulva requires that 5-10 years of disease-free interval have past before diagnosing a new vaginal lesion as primary vaginal carcinoma. This criterion is required to rule out recurrent cervical or vulvar disease.

About 80% of vaginal cancers are metastatic, primarily from the cervix or endometrium. Metastatic cancer from the vulva, ovaries, choriocarcinoma, rectosigmoid, and bladder are less common. These cancers usually invade the vagina directly. Cancers from distant sites that metastasize to the vagina through the blood or lymphatic system also occur, including colon cancer, renal cell carcinoma, melanoma, and breast cancer.

History of the Procedure

In 1946, Alexander Brunschwig published the first cases of pelvic exenteration. In his first series, 5 of 22 surgical patients died from the operation itself. The original procedure consisted of connecting the ureters to the colostomy. In 1950, Bricker modified the procedure by isolating a loop of ileum, closing one end, anastomosing the ureters to it, and bringing the patent end out as a stoma.Since then, several other modifications have improved the outcome of this procedure. Today, with vaginal reconstruction and continent vesicostomy, the procedure is widely accepted as a treatment in selective cases.

Frequency

Primary vaginal carcinoma is rare, constituting only 1-2% of all malignant gynecological tumors. It ranks fifth in frequency behind cancer of the uterus, cervix, ovary, and vulva. The age-adjusted incidence in the United States is 0.6 per 100,000 population. The strict criteria used in defining vaginal carcinoma contribute to this low incidence.

Etiology

The etiology of vaginal cancer has not been identified. Note that vaginal cancer is not histologically homogeneous; several types of lesions exist, each with its own characteristics, age predilection, aggressiveness, and prognosis (see the Table). This suggests that a single etiologic factor is unlikely. Although some histologic types of vaginal cancer have been associated with exposure to certain agents, so far no clear cause-and-effect relationship exists between any of those agents and vaginal carcinoma.

The strongest association is between squamous cell carcinoma and human papilloma virus (HPV) infection, which is similar to cervical carcinoma. HPV subtypes 16 and 18 have the highest oncogenic potential and are most commonly linked to dysplastic changes in the female genital tract. Because HPV is sexually transmitted, this association raises the question as to whether women who engage in high-risk sexual behaviors, such as sex with multiple partners, are at risk for developing vaginal cancer. Other associated infectious agents are herpes simplex virus (HSV) and Trichomonas vaginalis. In 2000, Lee and colleagues reported a case of rapidly progressive vaginal squamous cell carcinoma in a young woman with a 2-year history of human immunodeficiency virus (HIV) infection.They suggest that young women infected with both HIV and HPV are at increased risk for a more aggressive and less responsive vaginal cancer.

Another association that strengthens the link between HPV infection and vaginal cancer is the presence of a premalignant lesion in the vagina, known as vaginal intraepithelial neoplasia (VAIN). In 1991, Aho and coworkers reported that 5-9% of patients treated for VAIN progressed to invasive carcinoma.3 This suggests that VAIN may be a precursor to vaginal cancer even though the incidence of VAIN in the United States is 0.2-0.3 per 100,000 women, which is less than the incidence of diagnosed vaginal cancer. This is because of the fact that women with VAIN are usually asymptomatic and that screening for VAIN is not recommended for the general population. Still, the true malignant potential of VAIN needs to be identified.

A previous history of cervical intraepithelial neoplasia (CIN), invasive cervical carcinoma, or invasive vulvar carcinoma has also been associated with vaginal carcinoma. Several studies indicate that up to 30% of patients with primary vaginal carcinoma have a previous history of in situ or invasive carcinoma that was treated at least 5 years before diagnosis.

Long-term pessary use and chronic irritation of vaginal mucosa in women with procidentia have been associated with vaginal cancer. Other predisposing factors include cigarette smoking, immunosuppressive therapy, chemotherapy, and radiation therapy. Approximately 10% of women diagnosed with primary vaginal carcinoma have a previous history of irradiation to the pelvis. In a 2000 report, Carthew and colleagues demonstrated that tamoxifen, a chemotherapeutic drug, induced endometrial and vaginal cancer in rats in the absence of endometrial hyperplasia. In 1999, Pukkala and colleagues reported an association between low socioeconomic class in Finland and an increased incidence of cervical, endometrial, and vaginal cancer.

Diethylstilbestrol (DES), a drug previously used in the first trimester to prevent pregnancy loss, has a strong association with clear cell adenocarcinoma of the vagina. Herbst and colleagues first observed this association in 1971, which led to the discontinuation of DES that same year. By 1987, the Registry for Hormonal Transplacental Carcinogenesis, established by Herbst and Scully, identified 524 women with clear cell adenocarcinoma, but only 60% had a history of DES exposure. Disease in the other 40% of patients with no history of DES exposure could be explained by recall bias or exposure to other unidentified factors. Women with in utero exposure to DES are at higher risk of developing adenocarcinoma than the general population. The estimated risk in these women is 1 in 1000.

Although 59% of women with vaginal cancer had a prior hysterectomy, in a 1986 report, Herman and colleagues demonstrated that when age and prior cervical cancer are controlled for, risk of vaginal cancer is not increased following hysterectomy for benign disease.7 Note that hysterectomy by itself is not a risk factor, rather women who underwent hysterectomy were poorly monitored.

In a 2004 publication, Hellman et al in Sweden reviewed 341 cases of primary carcinoma of the vagina from 1956-1996 and suggested that the etiology of vaginal cancer may be age related.8 In younger women, the disease occurred in the upper part of the vagina and seemed to be related to cervical dysplasia and HPV infection, while in older patients, the tumors were exophytic. There was significant correlation with late menarche, suggesting hormonal factors and trauma to the vagina as probable etiologies.



Pathophysiology

The presence of different stages of histologic differentiation—VAIN, carcinoma in situ, possible microinvasive carcinoma, and invasive cancer—suggests a continuum of transformation from less malignant to more invasive, which is similar to the continuum described for cervical cancer. As reported by Ikenberg et al in 19909 and Ostraw et al in 1988, identification of HPV DNA in squamous cell cancer cells by in situ hybridization (21%) and southern blot hybridization (56%) strongly suggests an association with HPV infection and a possible role of HPV in the pathogenesis of squamous cell vaginal carcinoma.

On the other hand, the significant association with a previous history of cervical or vulvar cancer suggests that the entire genital tract is at risk for squamous cell carcinoma once malignancy has occurred anywhere along the tract; this is a phenomenon postulated by Marcus and is known as the field effect. HPV infection could explain this phenomenon because HPV is associated with cervical, vaginal, and vulvar disease. Koyamatsu et al did a comparative analysis of the presence of HPV types 16 and 18 by polymerase chain reaction (PCR) and expression of p53 gene and Ki-67 antigen using immunohistochemistry in cervical, vaginal, and vulvar cancer.They suggested that in cervical cancer, HPV 16 and 18 played a common causal role, and in vulvar cancer, p53 gene mutations were the main carcinogenic cause, while vaginal cancer has transitional characteristics between cervical and vulvar cancer. There was no significant difference in overexpression of Ki-67 antigen among the 3 cancers.

Another explanation for this association is that an occult residual disease, such as VAIN, is trapped within the vaginal cuff posthysterectomy and goes unnoticed until it develops into invasive carcinoma. This possibility illustrates the theory of the field effect and HPV infection because HPV has also been linked to VAIN. It also partially explains why women with previous hysterectomy go unnoticed until they present with advanced-stage vaginal carcinoma. The third possibility is radiation carcinogenesis.

The pathogenesis by which DES might play a role in inducing clear cell adenocarcinoma is unclear. In 1972, Forsberg and colleaguesproposed the possibility of estrogen-induced maturation arrest of the müllerian ducts, and in 1984 Robboy and colleaguessuggested that atypical vaginal adenosis and atypical cervical ectropion of the tuboendometrial type might act as the precursors of clear cell adenocarcinoma of the vagina and cervix.

Most vaginal cancers occur in the upper third of the vagina. Reports are contradictory as to whether the anterolateral wall or the posterior wall is the more frequent site. Reports suggesting that the upper posterior wall is the most common site favor the hypothesis that irritating substances, such as vaginal secretions and semen, pool in the posterior fornix and cause chronic irritation, which could lead to induction of a carcinogenic process.

The proximity of the bladder anteriorly and the rectum posteriorly to the vagina predisposes these organs to direct invasion by the tumor. Lymphatic dissemination follows the lymphatic drainage of the vagina. The middle-to-upper vagina communicates superiorly with the lymphatics of cervix and drains into the pelvic obturator node, the internal and external iliac chains, then to the para-aortic nodes. The distal third of vagina drains to the inguinal node then the pelvic node. Posterior wall lymphatics communicate with rectal lymphatics and drain to the inferior gluteal, sacral, and rectal nodes. Hematogenous dissemination to distant sites includes the lungs, liver, bone, and skin. A submucosal lesion suggests that the malignancy is metastatic via the vaginal lymphatics.

Presentation

Duration of symptoms averages 6-12 months before diagnosis, with a range of 0-11 years. Delay in diagnosis of vaginal carcinoma is not uncommon, and this is partially because of disease rarity and delay in relating patient symptoms to a vaginal origin. As expected, the longer the delay, the more advanced the cancer once the diagnosis is made, which results in a poorer outcome.

Painless vaginal bleeding is the most common symptom, accounting for 65-80% of all presentations. Bleeding is postmenopausal in about 70% of patients, which is consistent with the peak age of 60 years for squamous cell carcinoma, the most common type. Menorrhagia, intermenstrual bleeding, and postcoital bleeding have also been reported. Vaginal discharge occurs in 30% of patients, while 20% of patients report urinary symptoms, which are caused by an anterior lesion compressing or invading the bladder, the urethra, or both. This causes bladder pain, dysuria, urgency, and hematuria. About 15-30% of patients present with pelvic pain. Posterior lesions compress or invade the rectosigmoid, which causes tenesmus or constipation.

Only 10% of patients report a vaginal mass or vaginal prolapse. In 2000, Eltabbakh and coworkers reported a single patient who presented with a cystic pelvic mass arising from the posterior vaginal wall that mimicked an ovarian neoplasm. About 10-27% of patients are asymptomatic; diagnosis is made during routine pelvic examination. These patients tend to be caught at a much earlier stage than those presenting with symptoms, and their prognosis is much better.

Indications

Consensus as to the proper treatment of vaginal carcinoma is lacking, mainly because of the rarity of the disease. The most commonly used treatment modality is radiotherapy. Surgery, with or without concomitant radiation therapy, is indicated in the following conditions:

  • Squamous cell carcinoma
    • Stage I disease in the upper posterior vagina
    • Stage IVa disease, particularly in the presence of a rectovaginal or vesicovaginal fistula
    • Central recurrence after radiotherapy
    • Ovary transposition in young patients prior to radiotherapy
  • Clear cell adenocarcinoma: Although the etiology is different, the presentation may be similar to squamous cell carcinoma.
  • Verrucous carcinoma: Because radiation therapy is contraindicated, surgery is the only treatment.
  • Other cases
    • Melanoma
    • Sarcoma
    • Embryonal rhabdomyosarcoma
    • Endodermal sinus tumor

These indications are discussed in detail in Treatment.

Relevant Anatomy

The vagina is located in the true pelvis, which also contains the rest of the internal genital tract, the rectosigmoid, the bladder, the proximal urethra, and the pelvic portions of the ureters. The pelvic organs are partially covered by the peritoneum. The endopelvic fascia covers these organs and forms their supporting ligaments in conjunction with the pelvic vasculature and musculature. The pelvic cavity is divided into anterior and posterior compartments by the transversely positioned broad ligament. The uterus is centered within the broad ligament and is attached to the round ligaments, which run anterolaterally within the broad ligament from the uterus to the pelvic wall.

The anterior cul-de-sac, also known as the vesicouterine pouch, is located between the uterus and the bladder. It has small lateral recesses known as the paravesical fossae. This pouch ends where the cervix and the bladder connect and does not extend down to the vagina. The posterior cul-de-sac, known as the rectouterine pouch of Douglas, is located between the uterus posteriorly and the rectum anteriorly. It is continuous with the pararectal fossae and contrary to the anterior pouch. It extends about 1-2 cm down to the vagina, separating the cervix from the rectum.

The vagina is a muscular tube that extends from the cervix to the hymenal ring, penetrating the levator ani and the urogenital diaphragm. These latter structures provide vaginal support inferiorly. From the outermost to the innermost layers, the vagina is composed of an endopelvic fascia, which contains an abundant plexus of vessels, lymphatics, and nerves as well as outer longitudinal and inner circular smooth muscle layers, submucosa, and mucosa. The vagina is attached to the rectum posteriorly by the rectal pillars, while the bladder pillars provide anterior vaginal attachment to the bladder. During vaginal inspection with a speculum, the anterior and posterior sulci provide the anatomic landmark of the site of attachment of these pillars. These are most easily observed in nulliparous women.

The rectal and bladder pillars are paired, parallel, longitudinal, fibrovascular bundles containing extensive vascular and lymphatic networks between the vagina and the rectum and bladder, respectively. They both run the entire length of the vagina. The bladder pillars also contain the paravaginal tissues (paracolpium). As it joins the lower end of the cervix, the upper end of the bladder pillar forms the vesicouterine ligament. This ligament forms a tunnel through which the ureters run inferomedially to reach the inferolateral portion of the bladder. The tunnel divides the vesicouterine ligament into anterior and posterior leaves. This anatomic structure is important during radical hysterectomy when careful dissection of the ligament is needed to mobilize the ureters. The rectal pillars receive the middle rectal arteries from the cardinal ligament.

The cardinal ligaments are wedge-shaped fibrovascular bundles containing the uterine, vaginal, inferior vesical, and middle rectal arteries and veins as well as the lymphatic system. On each side, they run from the lateral aspect of the cervix to the lateral pelvic sidewall, traversing the pelvic plane at a 30º angle from transverse pelvic diameter and dividing the paravesical and paravaginal spaces from the pararectal spaces. On the pelvic wall, they insert on the endopelvic fascia and the hypogastric vasculature. The anterior part of the cardinal ligament is more vascular, while the posterior part is more fibrous and contains the autonomic system of the bladder and rectum. An important landmark is the uterine artery that crosses the anterior-most portion of the cardinal ligament. The ureter enters the upper portion of the ligament beneath this artery (water under the bridge) and 1-2 cm lateral to the isthmus of the uterus. The uterine veins cross below the ureters.

The uterosacral ligaments run from the posterolateral aspect of the cervix to the anterolateral part of the rectum. They are in close contact to the rectal pillars and straddle the posterior cul-de-sac. Several avascular tissue planes are developed during pelvic surgery. The paravesical space is bordered by the symphysis pubis anteriorly, the cardinal ligaments posteriorly, the obliterated umbilical artery along the bladder medially, and the obturator internus laterally. The pararectal space is bordered by the cardinal ligament anteriorly, sacrum posteriorly, rectum medially, and hypogastric artery laterally. The rectovaginal space is bounded by the vagina anteriorly and the rectum posteriorly, while the rectal pillars form its lateral walls. The vesicovaginal space is limited laterally by the bladder pillars, anteriorly by the bladder, and posteriorly by the vagina. To develop this space, the peritoneal reflection of the anterior cul-de-sac is entered.

The levator ani forms the major support of the pelvic structures and is the major component of the pelvic diaphragm. It is penetrated anteriorly by the rectum, vagina, and urethra. It forms the floor of all the planes discussed above. The upper part of the vagina receives its blood supply from the uterine and the internal pudendal arteries, from which the vaginal artery arises. The inferior rectal artery and other branches arising from the internal pudendal artery supply the lower vagina. The vaginal venous plexus mainly drains into the pelvic wall through the parametrial veins, and to a lesser degree to the vesical and rectal plexuses.

Crossover of the vaginal lymphatic system is extensive. The middle-to-upper vagina communicates superiorly with the cervical lymphatics and drains into the pelvic obturator node, the internal and external iliac chains, and then the para-aortic nodes. The distal third of the vagina drains to the inguinal then the pelvic nodes. The posterior wall lymphatics communicate with the rectal lymphatics and drain to the inferior gluteal, sacral, and rectal nodes. The vagina stays in close proximity to the bladder and urethra anteriorly, which increases risk of accidental injury to these structures during surgery. The sigmoid, on the other hand, reflects away from the posterior vaginal wall at its midpoint, facilitating approaching the vagina posteriorly through the posterior cul-de-sac and a developed rectovaginal plane.



Contraindications

Metastasis and extension to pelvic sidewalls are contraindications for exenteration. Microscopic pelvic node involvement is more of a controversy than a contraindication, and patients with positive pelvic nodes and no other poor prognostic factors can be considered candidates for exenteration. Involvement of both the pelvic and para-aortic nodes should warrant aborting the surgery.

Treatment Medical Therapy

Until the discovery of radium, surgical excision was the only available treatment. In 1929, Taussig first reported radiation therapy at the Barnard Free Skin and Cancer Clinic in Boston, where he treated 18 patients with vaginal carcinoma. Only 2 of the patients survived longer than 5 years. During October of 1934, Taussig stated before the Clinical Congress of the American College of Surgeons that "primary cancer of the vagina is very rare and almost universally fatal. We acknowledge our total inability to do anything effective" to treat this disease.

Since then, radiation therapy has largely replaced surgical excision; today, radiotherapy is the treatment of choice for most cases of primary vaginal carcinoma. Still, no consensus exists regarding the proper treatment of this particular cancer, partly because of the rarity of the disease. Treatment is individualized and depends on the histologic type, stage, and location of the lesion; the presence or absence of the uterus; and whether the patient has received previous irradiation. It also depends on the medical condition of the patient and a risk-benefit analysis of all possible treatment modalities. Treatment consists of radiation therapy, surgery, or a combination of both with occasional chemotherapy.

Psychological and anatomic considerations are important in planning the treatment regimen. For example, the physician must ask the patient if she can withstand the physical and physiological alterations of an exenteration. Other issues that must be addressed are radical versus conservative approach, the patient's wishes regarding maintenance of a functional vagina, and the close proximity of the bladder and rectum to the vagina, which may limit the dose of radiation and restrict surgical margins.

Surgical Therapy

The following are treatment options and surgical procedures in several types of primary vaginal cancer.

Squamous cell carcinoma

Stage I disease involving the upper posterior vagina is treated by radical hysterectomy, partial vaginectomy, and bilateral pelvic lymphadenectomy. Lymphadenectomy is required to ensure that metastatic disease is not present. If the patient had a previous hysterectomy, then a radical upper vaginectomy with pelvic lymphadenectomy is performed after the paravesicular and pararectal spaces are developed to avoid injury to the bladder and rectum, respectively. Each ureter is also dissected out to its point of entry into the bladder. If the lesion is multifocal or if it extends to the lower third of the vagina, inguinal lymphadenectomy should also be performed, and a total vaginectomy is required. If the depth of the invasion is questioned during the operation, then a frozen section from the margins should be taken to ensure that tumor resection was adequate.

In general, tumors of the upper posterior wall are more operable because the sigmoid reflects away from the posterior vaginal wall, while the entire length of the anterior vaginal wall stays in close proximity to the bladder. A lower vaginal lesion can be treated with radical hemivulvectomy and lower vaginectomy with bilateral inguinal node dissection. Radiation therapy is commonly used as an alternative to surgery. Stages II and III are treated with radiation therapy. In premenopausal patients, a pretreatment laparotomy is performed in order to transpose the ovaries away from the field of radiation and resect any enlarged lymph nodes. If the patient has a central recurrence with no signs of metastasis after radiotherapy, then pelvic exenteration is the only option.

Patients with stage IVa disease have the option of radiation therapy or pelvic exenteration. The latter is highly recommended if a rectovaginal or vesicovaginal fistula is present. Stage IVb is a contraindication for surgery.

Clear cell adenocarcinoma

Therapeutic considerations are very similar to those for patients with squamous cell carcinoma, although most patients are young and every effort should be made to preserve functional ovaries and vagina. Surgery is the primary treatment modality. In stage I and early stage II disease, radical hysterectomy, pelvic lymphadenectomy, and vaginectomy with split-thickness skin graft have been successful. Alternatively, in 1987, Senekjian and colleagues reported a 5-year survival of 92% for patients with very early small lesions treated by wide local excision, laparotomy for retroperitoneal lymphadenectomy, and local irradiation to the immediate adjacent tissues.The best candidates are patients with tumors less than 2 cm in diameter, a predominant tubulocystic pattern, and depth of invasion less than 3 cm. If radiation is used as the sole treatment, then transposition of at least one ovary up into the paracolic gutter beyond the radiation field should be done with pelvic lymph node dissection. Local excision without radiation is not recommended since Herbst and colleagues reported that 16% of patients with stage I disease have positive pelvic nodes. Pelvic exenteration is done for central recurrences after primary irradiation.

Matthews et al presented a case report of radical abdominal trachelectomy and upper vaginectomy performed on a 22-year-old woman with clinical stage I vaginal clear cell adenocarcinoma in the left fornix. The woman had no evidence of recurrence with regular menstrual cycles 28 months after initial surgery. The authors concluded that this procedure could be considered to conserve fertility in young women.

Melanoma

The best treatment for vaginal melanoma remains controversial. Radical surgery has been the main treatment modality, although a more conservative approach has been advocated by some authors. In 1989 Reid et aland Buchanan et al in 1998, for example, showed no significant difference in 5-year survival rates or disease-free intervals for radical versus conservative surgery. On the other hand, in 1994 Van Nostrand and colleagues demonstrated that radical surgery had a significant 2-year survival advantage over conservative surgery (48% vs 20%); they recommend a radical approach to patients with lesions smaller than 10 cm2.

Recently, detection of nodal involvement prior to radical procedures has been suggested because positive lymph nodes indicate poor prognosis; radical surgery might be unjustified. Siu et al used laparoscopic ultrasonography to successfully detect enlarged pelvic lymph nodes.Rodier et al used 99mTc-sulfur colloid injected around the lesion and detected the sentinel lymph node with hot spot by lymphoscintigraphy.Nakagawa et al succeeded in evaluating the sentinel lymph node to decide the extent of surgery using a dye injection method; 1 mL of methylene blue was injected into the subcutaneous layer at the boundary between the lesion and the vaginal mucosa, followed by incision in the ipsilateral groin to detect the stained lymph node.

Radical surgery varies depending on tumor size and location. Small lesions in the upper vagina are treated by radical hysterectomy, subtotal vaginectomy, and pelvic lymphadenectomy. Lesions in the lower vagina are managed by partial vaginectomy, total or partial vulvectomy, and bilateral inguinal lymphadenectomy. Larger and more invasive lesions (>3 mm) are treated with exenterative surgery. Note that whenever vaginal mucosa is left in situ after partial or subtotal vaginectomy, frozen sections should be obtained to exclude lateral superficial spread because the most common site of initial recurrence is the vagina.

Conservative management includes wide local excision (WLE) and simple hysterectomy combined with radiotherapy and/or chemotherapy. Radiation therapy with high-dose fractions (>400 cGy/fx) has been effective in selected patients. This type of response is consistent with the higher response rate seen with cutaneous melanoma when large individual fractions are compared to conventional fractionation. Irvin et al reported in their case series a higher locoregional control using WLE followed by high-dose fractionation teletherapy compared to more radical surgical resection.

Verrucous carcinoma

As mentioned previously, radiation therapy is contraindicated because it tends to induce aggressive cancer types. The only treatment option is surgical resection. If the lesion is small, a wide surgical excision is performed. With larger lesions, vaginectomy or exenteration is recommended. Because this tumor rarely metastasizes, dissecting the lymph nodes is unnecessary unless they appear enlarged.

Sarcoma botryoides

Because the typical patient is prepubertal, preserve ovarian function and reproductive organs. Currently, a conservative approach is used instead of an exenterative surgery. Preoperative and/or postoperative chemotherapy and radiotherapy improve the outcome. For small easily resectable tumors, the lesion is excised. Chemotherapy VAC (vincristine, actinomycin D, and cyclophosphamide) and radiotherapy follow. If the tumor is bulky, preoperative chemotherapy or radiotherapy is administered before the lesion is excised.

Endodermal sinus tumor

This very rare tumor is treated with chemotherapy VAC to reduce the tumor size. Chemotherapy is followed by partial colpectomy, radiotherapy, or both.

Vaginal leiomyosarcoma

These tumors vary in their malignancy depending on how well they are differentiated. Well-differentiated tumors are less likely to metastasize and are managed by surgical excision. Frozen sections are taken to ensure that the tumor is well contained within the surgical margins. Poorly differentiated tumors should receive adjuvant radiotherapy.

Preoperative Details

Patient selection and preoperative evaluation

The first and most important requirement for exenterative surgery is that the patient should have no underlying medical illnesses. The patient must be fit for a prolonged operation with potential blood loss and major fluid shifts. Psychological evaluation is necessary because a stable personality and supportive social environment are required because of postoperative physical and physiologic changes. In addition to tumor resectability, patient evaluation centers on whether the patient can physiologically and psychologically withstand the surgery.

Signs of systemic spread should be absent. Evaluation starts with physical examination, which includes palpation of all peripheral lymph nodes, especially the inguinal and supraclavicular nodes. The clinical triad of unilateral leg edema, sciatic pain, and ureteral obstruction suggest involvement of the posterolateral pelvic sidewall, which is a sign of lack of resectability. Each sign by itself is not a contraindication for exploratory laparotomy, although each is associated with decreased probability of resection and decreased probability of long-term survival even if resected with clear margins. Chest radiography or CT scanning of the chest, upper abdomen, and pelvis are mandatory to rule out lung, liver, and para-aortic metastasis, respectively. Any suspicious lymph node should undergo fine-needle aspiration cytology to rule out metastasis. In a 1989 report, Manetta and colleagues dismissed the need to biopsy nonsuspicious supraclavicular lymph nodes in a random fashion.

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