Vaginal cancer
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Vaginal cancer is a type of cancer that forms in the tissues of the vagina. The vagina leads from the cervix (the opening of the uterus) to the outside of the body.
Carcinomas of the vagina are uncommon tumors comprising 1% to 2% of gynecologic malignancies. They can be effectively treated, and when found in early stages, are often curable. The histologic distinction between squamous cell carcinoma and adenocarcinoma is important because the two types represent distinct diseases, each with a different pathogenesis and natural history. Squamous cell vaginal cancer (approximately 85% of cases) initially spreads superficially within the vaginal wall and later invades the paravaginal tissues and the parametria. Distant metastases occur most commonly in the lungs and liver.
Adenocarcinoma (approximately 15% of cases) has a peak incidence between 17 and 21 years of age and differs from squamous cell carcinoma by an increase in pulmonary metastases and supraclavicular and pelvic node involvement. Rarely, melanoma and sarcoma are described as primary vaginal cancers. Adenosquamous carcinoma is a rare and aggressive mixed epithelial tumor comprising approximately 1% to 2% of cases.
Prevalance
Estimated new cases and deaths from vaginal (and other female genital) cancer in the United States in 2008:
- New cases: 2,210.
- Deaths: 760.
Types of vaginal cancer
Types of vaginal cancer, in order of prevalence, include:
- Vaginal squamous cell carcinoma arises from the thin, flat squamous cells that line the vagina. This is the most common type of vaginal cancer. It is found most often in women aged 60 or older.
- Vaginal adenocarcinoma arises from the glandular (secretory) cells in the lining of the vagina that produce some vaginal fluids. Adenocarcinoma is more likely than squamous cell cancer to spread to the lungs and lymph nodes. It is found most often in women aged 30 or younger, and has been found in a small percent of women whose mothers in the 1950s used diethylstilbestrol to prevent threatened abortions.
- Vaginal germ cell tumors (primarily teratoma and endodermal sinus tumor) are rare. They are found most often in infants and children.
- Sarcoma botryoides, a rhabdomyosarcoma also is found most often in infants and children.
Risk Factors
Clear cell adenocarcinomas are rare and occur most often in patients less than 30 years of age who have a history of in utero exposure to diethylstilbestrol (DES). The incidence of this disease, which is highest for those exposed during the first trimester, peaked in the mid-1970s, reflecting the use of DES in the 1950s.[3] Young women with a history of in utero DES exposure should prospectively be followed carefully to diagnose this disease at an early stage. In women who have been carefully followed and well-managed, the disease is highly curable.
Vaginal adenosis is most commonly found in young women who had in utero exposure to DES and may coexist with a clear cell adenocarcinoma, though it rarely progresses to adenocarcinoma. Adenosis is replaced by squamous metaplasia, which occurs naturally, and requires follow-up but not removal. The natural history, prognosis, and treatment of other primary vaginal cancers (sarcoma, melanoma, lymphoma, and carcinoid tumors) may be different, and specific references should be sought.
Presentation
Vaginal cancer occurs primarily in those over age 50. The disease usually presents first with abnormal vaginal bleeding or discharge.
Signs and Symptoms
The most common sign is abnormal vaginal bleeding, which may be postcoital, intermenstrual, prepubertal, or postmenopausal.[1] Other, less specific signs include difficult or painful urination, pain during intercourse, and pain in the pelvic area.
Diagnosis
Several tests are used to diagnose vaginal cancer, including:
- Physical exam and history
- Pelvic exam
- Pap smear
- Biopsy
- Colposcopy
Tests are done to find out if cancer cells have spread
The process used to find out if cancer has spread within the vagina or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process:
Biopsy:
A biopsy may be done to find out if cancer has spread to the cervix. A sample of tissue is cut from the cervix and viewed under a microscope. A biopsy that removes only a small amount of tissue is usually done in the doctor’s office. A woman may need to go to a hospital for a cone biopsy (removal of a larger, cone-shaped piece of tissue from the cervix and cervical canal). A biopsy of the vulva may also be done to see if cancer has spread there.
Chest x-ray:
An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
Cystoscopy:
A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Ureteroscopy:
A procedure to look inside the ureters to check for abnormal areas. A ureteroscope is inserted through the bladder and into the ureters. A ureteroscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue to be checked under a microscope for signs of disease. A ureteroscopy and cystoscopy may be done during the same procedure.
Proctoscopy:
A procedure to look inside the rectum to check for abnormal areas. A proctoscope is inserted through the rectum. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue to be checked under a microscope for signs of disease.
CT scan (CAT scan):
A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
MRI (magnetic resonance imaging):
A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Lymphangiogram:
A procedure used to x-ray the lymph system. A dye is injected into the lymph vessels in the feet. The dye travels upward through the lymph nodes and lymph vessels and x-rays are taken to see if there are any blockages. This test helps find out whether cancer has spread to the lymph nodes.
The following stages are used for vaginal cancer:
Stage 0 (Carcinoma in Situ)
In stage 0, abnormal cells are found in tissue lining the inside of the vagina. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.
Stage I
In stage I, cancer has formed and is found in the vagina only.
Stage II
In stage II, cancer has spread from the vagina to the tissue around the vagina.
Stage III
In stage III, cancer has spread from the vagina to the lymph nodes in the pelvis or groin, or to the pelvis, or both.
Stage IV
Stage IV is divided into stage IVA and stage IVB:
- Stage IVA: Cancer may have spread to lymph nodes in the pelvis or groin and has spread to one or both of the following areas:
- The lining of the bladder or rectum.
- Beyond the pelvis.
- Stage IVB: Cancer has spread to parts of the body that are not near the vagina, such as the lungs. Cancer may also have spread to the lymph nodes.
AJCC Stage Groupings
Stage 0
- Tis, N0, M0
Stage I
- T1, N0, M0
Stage II
- T2, N0, M0
Stage III
- T1, N1, M0
- T2, N1, M0
- T3, N0, M0
- T3, N1, M0
Stage IVA
- T4, any N, M0
Stage IVB
- Any T, any N, M1
Recurrent Vaginal Cancer
Recurrent vaginal cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the vagina or in other parts of the body.
Treatment
Therapeutic alternatives depend on stage; surgery or radiation therapy is highly effective in early stages, while radiation therapy is the primary treatment of more advanced stages. Chemotherapy has not been shown to be curative for advanced vaginal cancer, and there are no standard drug regimens.
Stage 0 Vaginal Cancer
Squamous Cell Carcinoma In Situ
This disease is usually multifocal and commonly occurs at the vaginal vault. Because vaginal intraepithelial neoplasia (VAIN) is associated with other genital neoplasias, the cervix (when present) and vulva should be carefully examined. The treatments listed below produce equivalent cure rates. The selection of treatment depends on patient factors and local expertise (e.g., anatomical distortion of the vaginal vault [related to wall closure at the time of hysterectomy] requires excision for technical reasons to exclude the possibility of invasion by buried disease). Lesions with hyperkeratosis respond better to excision or laser vaporization than to fluorouracil.
Standard treatment options:
- Wide local excision with or without skin grafting.
- Partial or total vaginectomy with skin grafting for multifocal or extensive disease.
- Intravaginal chemotherapy with 5% fluorouracil cream. Instillation of 1.5 g weekly for 10 weeks has been found to be as effective as more frequent use.
- Laser therapy.
- Intracavitary radiation therapy delivering 60 Gy to 70 Gy to the mucosa. The entire vaginal mucosa should be treated.
Stage I Vaginal Cancer
Squamous Cell Carcinoma
The treatments listed below produce equivalent cure rates. The selection of treatment depends on patient factors and local expertise.
Standard treatment options for superficial lesions less than 0.5 cm thick:
- Intracavitary radiation therapy. In most instances, 60 Gy to 70 Gy prescribed to 0.5 cm is delivered to the tumor for 5 to 7 days (external-beam radiation therapy [EBRT] is required for bulky lesions). For lesions of the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy is given to pelvic and/or inguinal lymph nodes.
- Surgery. Wide local excision or total vaginectomy with vaginal reconstruction, especially in lesions of the upper vagina. In cases with close or positive surgical margins, adjuvant radiation therapy should be considered.
Standard treatment options for lesions greater than 0.5 cm thick:
- Surgery. In lesions of the upper third of the vagina, radical vaginectomy and pelvic lymphadenectomy should be performed. Construction of a neovagina may be performed if feasible and if desired by the patient. In lesions of the lower third, inguinal lymphadenectomy should be performed. In cases with close or positive surgical margins, adjuvant radiation therapy should be considered.
- Radiation therapy. Combination of interstitial (single-plane implant) and intracavitary therapy to a dose of at least 75 Gy to the primary tumor. In addition to brachytherapy, EBRT is advocated for poorly differentiated or infiltrating tumors that may have a higher probability of lymph node metastasis. For lesions of the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy is given to the pelvic and/or inguinal lymph nodes.
Adenocarcinoma
Standard treatment options:
- Surgery. Because the tumor spreads subepithelially, total radical vaginectomy and hysterectomy with lymph node dissection are indicated. The deep pelvic nodes are dissected if the lesion invades the upper vagina, and the inguinal nodes are removed if the lesion originates in the lower vagina. Construction of a neovagina may be performed if feasible and if desired by the patient. In cases with close or positive surgical margins, adjuvant radiation therapy should be considered.
- Intracavitary and interstitial radiation as previously described for squamous cell cancer. For lesions of the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy is given to the pelvic and/or inguinal lymph nodes.
- Combined local therapy in selected cases, which may include wide local excision, lymph node sampling, and interstitial therapy.
Stage II Vaginal Cancer
Squamous Cell Carcinoma
Radiation therapy is the standard treatment for patients with stage II vaginal carcinoma.
Standard treatment options:
- Combination of brachytherapy and external-beam radiation therapy (EBRT) to deliver a combined dose of 70 Gy to 80 Gy to the primary tumor volume. For lesions of the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy is given to the pelvic and/or inguinal lymph nodes.
- Radical surgery (radical vaginectomy or pelvic exenteration) with or without radiation therapy.
Adenocarcinoma
Standard treatment options:
- Combination of brachytherapy and EBRT to deliver a combined dose of 70 Gy to 80 Gy to the primary tumor. For lesions of the lower third of the vagina, elective radiation therapy of 45 Gy to 50 Gy is given to the pelvic and/or inguinal lymph nodes.
- Radical surgery (radical vaginectomy or pelvic exenteration) with or without radiation therapy.
Stage III Vaginal Cancer
Squamous Cell Carcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and external-beam radiation therapy (EBRT). EBRT for a period of 5 to 6 weeks (including pelvic nodes) followed by an interstitial and/or intracavitary implant for a total tumor dose of 75 Gy to 80 Gy and a dose to the lateral pelvic wall of 55 Gy to 60 Gy.
- Rarely, surgery may be combined with the above.
Adenocarcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and EBRT as described for squamous cell cancer.
- Rarely, surgery may be combined with the above.
Stage IVA Vaginal Cancer
Squamous Cell Carcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and external-beam radiation therapy (EBRT).
- Rarely, surgery may be combined with the above.
Adenocarcinoma
Standard treatment options:
- Combination of interstitial, intracavitary, and EBRT.
- Rarely, surgery may be combined with the above.
Stage IVB Vaginal Cancer
Squamous Cell Carcinoma
Patients should be considered candidates for one of the ongoing clinical trials to improve therapeutic results. Standard treatment is inadequate.
Standard treatment options:
- Radiation (for palliation of symptoms) with or without chemotherapy.
Adenocarcinoma
Patients should be considered candidates for one of the ongoing clinical trials to improve therapeutic results.
Standard treatment options:
- Radiation (for palliation of symptoms) with or without chemotherapy.
Recurrent Vaginal Cancer
Recurrence carries a grave prognosis. In a large series only five of fifty patients with recurrence were salvaged by surgery or radiation therapy. All five of these salvaged patients originally presented with stage I or II disease and failed in the central pelvis. Most recurrences are in the first 2 years after treatment. In centrally recurrent vaginal cancers, some patients may be candidates for pelvic exenteration or radiation therapy. Neither cisplatin nor mitoxantrone has significant activity in recurrent or advanced squamous cell cancer. There is no standard chemotherapy.
Prognosis
Prognosis depends primarily on the stage of disease, but survival is reduced in patients who are greater than 60 years of age, are symptomatic at the time of diagnosis, have lesions of the middle and lower third of the vagina, or have poorly differentiated tumors. In addition, the length of vaginal wall involvement has been found to be significantly correlated to survival and stage of disease in squamous cell carcinoma patients
See also
References
- National Cancer Institute: Vaginal Cancer (public domain)
- Stenchever: Comprehensive Gynecology, 4th ed., Copyright © 2001 Mosby, Inc.
- ↑ "Vaginal Cancer". Gynocologic Malignancies. Armenian Health Network, Health.am. 2005. Retrieved 2007-11-08.