eMedicine Specialties > Obstetrics and Gynecology > Gynecologic Surgery

Surgical Treatment of Vaginal Cancer: Workup

Author: Tarek Bardawil, MD, Assistant Professor, Department of Obstetrics and Gynecology, University of Miami Miller School of Medicine
Coauthor(s): Alberto Manetta, MD, Professor, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Department of Internal Medicine, Division of Epidemiology, University of California at Irvine College of Medicine
Contributor Information and Disclosures

Updated: Dec 10, 2007

Workup

Laboratory Studies

  • Screening
    • Routine screening for vaginal carcinoma is not justified for all patients because it is not cost effective. Women at risk, however, particularly those with a history of cervical neoplasia and risky sexual behavior, should receive a Papanicolaou test on a regular basis.
    • Screening women with previous hysterectomy is controversial. In 1990, Manetta and colleagues suggested that women with previous hysterectomy should be counseled to continue their gynecologic cancer surveillance program.15 They reported that 63% of patients who were diagnosed after the onset of their symptoms and who tended to have an unfavorable prognosis had undergone a prior hysterectomy. On the other hand, in 2000 Videlefsky et al16 and Fetters et al concluded that routine vaginal cuff testing for most patients who underwent hysterectomy for benign conditions is not indicated.
    • Young girls who were exposed to DES in utero should be routinely examined starting at puberty or at the age of 14 years. Examination includes cytologic screening of the cervix and vagina, followed by careful inspection and palpation of the genital tract. Staining with half-strength Lugol iodine helps mark areas of adenosis. As long as cytology findings are negative, colposcopy is unnecessary.

Diagnostic Procedures

  • During routine vaginal examination, the speculum blades should be rotated laterally in order to visualize the anterior and posterior vaginal walls. Inspect all vaginal mucosa while withdrawing the speculum. Vaginal cancer is multifocal and, although typically located in the vaginal apex, disease might involve any part of the vagina. Visual inspection alone is not enough, and careful circumferential palpation of the entire vagina is required in order to feel any raised or hardened areas.
  • Vaginal lesions, particularly when small and located in the lower third of the vagina, are often missed during the first vaginal inspection because the blades of the speculum normally cover the anterior and posterior vaginal walls. Frick and colleagues reported that about 19% of cases of vaginal cancer were missed on initial examination, which contributes to delay in diagnosis. Other reasons for delay are the rarity of vaginal carcinoma (1-2%) and attribution of patient symptoms to more common diseases, such as postmenopausal bleeding and endometrial cancer.
  • All visible lesions should be biopsied using either Eppendorf or Kevorkian punch biopsy forceps or similar instruments. Although the procedure is uncomfortable, local anesthesia is not recommended because it is as uncomfortable as the biopsy itself. In elderly patients, particularly those with some degree of vaginal stenosis, the examination should be performed under general anesthesia to allow adequate biopsy.
  • Patients with carcinoma in situ or very early invasive carcinoma are usually asymptomatic. Diagnosis is made when a routine Pap smear identifies abnormal cells. If the cervix is present, then the physician must rule out cervical neoplasia because cervical cancer is much more common than vaginal cancer. After a cervical origin has been ruled out through colposcopy and cervical cone biopsy, then the physician should perform a vaginal colposcopy. Because this is a time-consuming and difficult procedure, especially in elderly patients, it should be done under general anesthesia. Lugol iodine solution can help identify regions to obtain biopsies from; malignant cells lack glycogen and do not stain dark brown as does healthy vaginal mucosa. Because healthy vaginal epithelium needs to be estrogenized in order to have sufficient glycogen, use of local estrogen cream for 1-2 weeks before examination may be helpful for postmenopausal patients. Estrogen cream should be discontinued 2 days prior to colposcopy.
  • Patients with previous hysterectomy and abnormal cytology findings should undergo vaginal colposcopy as mentioned above. If no lesion is observed, then resect the entire vaginal vault because the lesion may be buried in the closed vaginal vault at the time of hysterectomy.

Histologic Findings

As mentioned before, primary vaginal carcinoma is not homogeneous. It is classified into several histologic types, each with its own characteristics (see the Table). The following are brief descriptions of the most common types.

Squamous cell carcinoma

Squamous cell carcinoma is by far the most common type, accounting for 85-87% of all primary vaginal carcinoma. It generally occurs in women older than 50 years and peak incidence is in people aged 60 years; however, several cases were reported in women as young as 18 years.

Grossly, it appears as an ulcerating lesion (50%), a fungating mass (30%), or an annular constricting mass (20%). Secondary infections in an ulcerating tumor are common. Histologically, it resembles squamous cell carcinoma arising from the cervix, confusing the physician as to whether the lesion is cervical or vaginal in origin. This illustrates the need for a strict definition of primary vaginal carcinoma.

The most common site of occurrence is the upper third of the vagina. Because of the thin vaginal wall, squamous cell carcinoma tends to spread early by directly invading the bladder and rectal walls. It also metastasizes through the blood or lymphatics. In 1981, Al-Kurdi and coworkers reported that about 28.6% of patients had pelvic lymph node involvement upon diagnosis.17 Squamous cell carcinoma can metastasize to virtually any organ, and in 1999 cutaneous metastasis was reported by Plataniotis and colleagues.18

Verrucous carcinoma

Verrucous carcinoma is rare in the vagina and is more commonly observed in the vulva. It is observed in women older than 50 years and is considered a variant of squamous cell carcinoma. Its clinical and pathological characteristics are similar to their vulvar counterparts. On visual examination, it has a large, warty, cauliflowerlike appearance similar to condylomata acuminata, but the papillary fronds lack a central core of connective tissue. They are slow growing, locally aggressive, and rarely metastatic. Radiation is contraindicated because it has been implicated in potentiating this tumor to a more malignant phenotype.

Clear cell adenocarcinoma

This is the second most common type and accounts for about 9% of all primary vaginal carcinoma. Unlike squamous cell carcinoma, clear cell adenocarcinoma manifests in patients at a very early age, usually after age 14 years, and peak incidence is in people aged 19 years. As discussed previously, an association with intrauterine exposure to DES has been established. The estimated risk in the exposed population is about 1 in 1000.

Clear cell adenocarcinoma is thought to arise mainly from areas of vaginal adenosis but may also arise in wolffian rest elements, periurethral glands, and foci of endometriosis. In non–DES-exposed patients, ectopic cervical glands are a possible origin.

Grossly, vaginal adenosis appears as multiple cysts 0.5-4 cm in diameter or as a diffuse erythematous granular mucosal lesion. The cancerous lesion appears polypoid, papillary, flat, or ulcerated. Microscopically, 3 histological patterns are predominant: tubulocystic, solid, and papillary. The tubulocystic pattern has the most favorable outcome. The tumor can spread by local invasion or by hematogenous or lymphatic dissemination.

Upon presentation, 70% of cases are stage I disease, but recurrence is frequent and can occur as late as 20 years after initial therapy. Secondary tumors from the colon, endometrium, cervix, breast, or ovary should be considered.

Melanoma

Vaginal melanoma is rare and accounts for 0.5-2% of all primary vaginal cancers. Fewer than 150 cases have been reported. Vaginal melanoma usually manifests in women older than 50 years, peak incidence is in women aged 60 years, and it tends to occur in white women.

Melanoma is most commonly found in the lower anterior vaginal wall. Grossly, it appears as blue or black, soft, mucosal or submucosal nodules, but it may also be nonpigmented and is frequently ulcerated, mimicking squamous cell carcinoma. Histologically, it is similar to cutaneous melanoma except that it is more invasive. It is thought to arise from melanocytes, which are present in 3% of healthy vaginas. The source probably is an aberrant melanocyte migration or melanocyte metaplasia.

The Breslow and Clark systems are used as part of staging; however, because deep invasion is invariably present upon presentation, tumor size rather than depth is a more significant prognostic factor. Tumors tend to recur locally, and metastasis to the lungs is common.

Sarcoma botryoides (embryonal rhabdomyosarcoma)

Although this tumor is rare, it is the most common vaginal cancer in children. It manifests in girls younger than 8 years, and peak incidence is in girls aged 3 years. Sarcoma botryoides is highly malignant and very aggressive. Initially, it tends to invade locally, then it metastasizes to inguinal, pelvic, retroperitoneal, and mediastinal lymph nodes as well as the lungs, pericardium, liver, kidney, and bones.

Grossly, it occurs in 2 structural forms: solid and multicystic grapelike. (The term botryoides comes from the Greek word botrys, which means grapes.) It originates in the subepithelial layers and expands outward to fill the vaginal cavity. Histologically, it is characterized by a loose myxomatous stroma with malignant pleomorphic cells and characteristic cross-striated rhabdomyoblasts (strap cells), staining positively for muscle markers. Patients most commonly present with abnormal vaginal bleeding. Patients occasionally present with a polypoid mass protruding from the introitus.

Endodermal sinus tumor (yolk sac tumor)

This type of adenocarcinoma is very rare. It is classified as a germ cell tumor and most commonly occurs in the ovary. It manifests in patients at a very young age, usually girls younger than 2 years, and peak incidence is in babies aged 10 months. To date, about 20 vaginal cases have been documented. Characteristically, it secretes alpha-fetoprotein (AFP), which is frequently used as a marker of recurrence.

Vaginal leiomyosarcoma

This tumor of smooth muscle origin is rare and accounts for fewer than 2% of all primary vaginal cancers. It has a wide age range of 25-86 years.

Grossly, it manifests as a bulky submucosal lesion, mainly in the upper vagina. Histologically, it is similar to leiomyosarcoma of the uterus. In a 1979 report, Tavassoli and Norris established the following microscopic criteria to diagnose leiomyosarcoma of the vagina: moderate-to-marked atypia with 5 or more mitotic figures per 10 high-power fields (HPF). Histologic grade is the most important predictor of outcome.19 Leiomyosarcoma may follow radiation therapy to the genital tract.

Most Common Forms of Primary Carcinoma of the Vagina

Open table in new window

Table
Histologic TypeCases of Vaginal CarcinomaPeak AgeSpreadCharacteristics
Squamous cell carcinoma85-87%
60 y
Local, blood, lymphaticMost common in upper third of vagina
Verrucous carcinomaRare
60 y
LocalVariant of squamous cell, cauliflowerlike, aggressive, radiotherapy contraindicated
Clear cell adenocarcinoma9%
19 y
Local, blood, lymphaticAssociated with in utero exposure to DES, tubulocystic pattern most favorable prognosis, late recurrence common
Melanoma0.5-2%
60 y
Local, blood, lymphaticWhite women, lower anterior vaginal wall, size more prognostically significant than invasion, poor prognosis
Sarcoma botryoides (embryonal rhabdomyosarcoma)Rare
3 y
Local, blood, lymphaticMost common vaginal cancer among children, grapelike mass, strap cells
Endodermal sinus tumor (yolk sac tumor)Very rare
10 moLocalAggressive, AFP as marker
Leiomyosarcoma<2%
Wide range
Local, blood, lymphaticGrade is most important prognostic factor, can be secondary to pelvic irradiation
Histologic TypeCases of Vaginal CarcinomaPeak AgeSpreadCharacteristics
Squamous cell carcinoma85-87%
60 y
Local, blood, lymphaticMost common in upper third of vagina
Verrucous carcinomaRare
60 y
LocalVariant of squamous cell, cauliflowerlike, aggressive, radiotherapy contraindicated
Clear cell adenocarcinoma9%
19 y
Local, blood, lymphaticAssociated with in utero exposure to DES, tubulocystic pattern most favorable prognosis, late recurrence common
Melanoma0.5-2%
60 y
Local, blood, lymphaticWhite women, lower anterior vaginal wall, size more prognostically significant than invasion, poor prognosis
Sarcoma botryoides (embryonal rhabdomyosarcoma)Rare
3 y
Local, blood, lymphaticMost common vaginal cancer among children, grapelike mass, strap cells
Endodermal sinus tumor (yolk sac tumor)Very rare
10 moLocalAggressive, AFP as marker
Leiomyosarcoma<2%
Wide range
Local, blood, lymphaticGrade is most important prognostic factor, can be secondary to pelvic irradiation


Staging

Once the diagnosis of cancer is established, staging should proceed to determine the best treatment. As with the other gynecologic cancers, staging is done according to FIGO classification (see FIGO staging classification of vaginal carcinoma). For vaginal cancer, staging is clinical and based on findings during general examination, pelvic examination, cystoscopy (for anterior wall tumors), proctoscopy (for posterior wall tumors), and chest radiography. If the patient reports bone pain, then skeletal radiography should be performed to rule out bone involvement.

CT scanning or MRI of the upper abdomen and pelvis are not FIGO recommendations, although they are frequently performed because they help in establishing the presence of enlarged lymph nodes, ureteral compression, hydronephrosis, and liver metastasis. Oudouxa et al suggested that F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) provides a more accurate assessment of the extent of disease in a patient with malignant melanoma as compared to conventional methods.20

Baseline levels of carcinoembryonic antigen (CEA), cancer antigen–125 (CA-125), and squamous cell carcinoma antigen are recommended because they are elevated in patients with some carcinoma types. Patients in whom adenocarcinoma is diagnosed should undergo thorough exploration for possible metastasis, mainly in the uterus, cervix, ovary, and colon. In these patients, a fractional dilatation and curettage is indicated to rule out endometrial origin. A barium enema with either sigmoidoscopy or colonoscopy is also indicated to rule out colonic origin. Also, mammography, chest radiography, and CT scanning of the abdomen or pelvis should follow. CA-125 should be taken as baseline for post-treatment follow-up.

Surgical staging is not usually required, but it is performed in selected premenopausal patients prior to radiotherapy. In these patients, pretreatment laparotomy allows the transposition of at least one ovary away from the field of radiation. It also allows for better assessment of the extent of the disease through dissection of the pelvic lymph nodes. Also, for patients scheduled for exenterative surgery, an exploratory laparotomy is required to rule out metastasis or lateral spread to the pelvic sidewall before proceeding with exenteration.

FIGO staging classification of vaginal carcinoma is as follows:
  • Stage 0: Carcinoma is carcinoma in situ (VAIN).
  • Stage I: Carcinoma is limited to the vaginal wall.
  • Stage II: Carcinoma involves subvaginal tissue but has not extended to pelvic wall.
  • Stage III: Carcinoma extends to pelvic wall.
  • Stage IV: Carcinoma extends beyond the true pelvis or involves mucosa of bladder or rectum. Bullous edema as such precludes inclusion in the stage IV classification.
  • Stage IVa: Carcinoma invades bladder or rectal mucosa or directly extends beyond the true pelvis.
  • Stage IVb: Carcinoma spreads to distant organs.

More on Surgical Treatment of Vaginal Cancer

Overview: Surgical Treatment of Vaginal Cancer
Workup: Surgical Treatment of Vaginal Cancer
Treatment: Surgical Treatment of Vaginal Cancer
Follow-up: Surgical Treatment of Vaginal Cancer
References

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Further Reading

Keywords

cervical cancer, vulvar cancer, premalignant disease of the vagina, human papilloma virus, HPV, cervical carcinoma, herpes simplex virus, HSV, Trichomonas vaginalis, T vaginalis, human immunodeficiency virus, HIV, sexually transmitted diseases, STDs, melanoma, exenteration

Contributor Information and Disclosures

Author

Tarek Bardawil, MD, Assistant Professor, Department of Obstetrics and Gynecology, University of Miami Miller School of Medicine
Tarek Bardawil, MD is a member of the following medical societies: American Association of Gynecologic Laparoscopists and American College of Obstetricians and Gynecologists
Disclosure: Nothing to disclose.

Coauthor(s)

Alberto Manetta, MD, Professor, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Department of Internal Medicine, Division of Epidemiology, University of California at Irvine College of Medicine
Alberto Manetta, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Obstetricians and Gynecologists, American College of Surgeons, American Gynecological and Obstetrical Society, American Medical Association, and American Society of Clinical Oncology
Disclosure: Nothing to disclose.

Medical Editor

Serdar H Ural, MD, Associate Professor of Obstetrics and Gynecology and Radiology, Director, Division of Maternal Fetal Medicine, Medical Director, Labor and Delivery Suite, Penn State University College of Medicine
Serdar H Ural, MD is a member of the following medical societies: American Association of Gynecologic Laparoscopists, American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, Association of Professors of Gynecology and Obstetrics, and Society for Maternal-Fetal Medicine
Disclosure: GSK Honoraria Speaking and teaching; Ortho Honoraria Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

Frederick B Gaupp, MD, Consulting Staff, Department of Family Practice, Hancock Medical Center
Frederick B Gaupp, MD is a member of the following medical societies: American Academy of Family Physicians
Disclosure: Nothing to disclose.

Chief Editor

Michel E Rivlin, MD, Associate Professor, Coordinator, Quality Assurance/Quality Improvement, Department of Obstetrics and Gynecology, University of Mississippi School of Medicine
Michel E Rivlin, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Medical Association, Mississippi State Medical Association, and Royal College of Surgeons of Edinburgh
Disclosure: Nothing to disclose.

 
 
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