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Types of Cancer > Gynecologic Cancers > Vulvar Cancer > NCI Resources
NCI/PDQ® Health professionals: Vulvar Cancer Treatment (PDQ®)
Affiliation:
National Cancer Institute
Last Modified: July 1, 2009
TABLE OF CONTENTS
Purpose of This PDQ® Summary
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This PDQ® cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of vulvar cancer. This summary is reviewed regularly and updated as necessary by the PDQ® Adult Treatment Editorial Board.
Information about the following is included in this summary:
- Epidemiology.
- Cellular classification.
- Staging.
- Treatment options by cancer stage.
This summary is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Some of the reference citations in the summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ® Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either standard or under clinical evaluation. These classifications should not be used as a basis for reimbursement determinations.
This summary is available in a patient version, written in less technical language, and in Spanish.
Note: Estimated new cases and deaths from vulvar cancer in the United States in 2009: 1
- New cases: 3,580.
- Deaths: 900.
Vulvar cancer is primarily a disease of elderly women but has been observed in premenopausal women as well. It is most commonly squamous cell carcinoma in type, though other histologic types do occur. Vulvar cancer is highly curable when diagnosed in an early stage.
Survival is most dependent on the pathologic status of the inguinal nodes. In patients with operable disease without nodal involvement, the overall survival (OS) rate is 90%; however, in patients with nodal involvement, the 5-year OS rate is approximately 50% to 60%. 2 Risk factors for node metastasis are clinical node status, age, degree of differentiation, tumor stage, tumor thickness, depth of stromal invasion, and presence of capillary-lymphatic space invasion. 2 3 4 5 6 Overall, about 30% of patients with operable disease have nodal spread. A multifactorial analysis of risk factors in squamous vulvar cancer demonstrated that nodal status and primary lesion diameter, when considered together, were the only variables associated with prognosis. Patients with negative inguinal nodes and lesions no more than 2 cm had a 98% 5-year survival rate, while those with any size lesion with three or more unilateral nodes or two or more bilateral nodes had a 29% 5-year survival rate. Intermediate groups with intermediate survival were also identified. 2 These discriminants were most useful as assessment criteria for stage III disease in the Federation Internationale de Gynecologie et Obstetrique staging system.
In many cases, the development of vulvar cancer is preceded by condyloma or squamous dysplasias. The prevailing evidence favors human papillomavirus (HPV) as a causative factor in genital tract carcinomas. The labia majora is the most common site of involvement and accounts for about 50% of cases. The labia minora accounts for 15% to 20% of cases. The clitoris and Bartholin glands are less frequently involved. 7
The pattern of spread is influenced by the histology. Well-differentiated lesions tend to spread along the surface with minimal invasion, while anaplastic lesions are more likely to be deeply invasive. Spread beyond the vulva is either to adjacent organs such as the vagina, urethra, and anus, or via the lymphatics to the inguinal and femoral lymph nodes, followed by the deep pelvic nodes. Hematogenous spread appears to be uncommon.
References:
- American Cancer Society.: Cancer Facts and Figures 2009. Atlanta, Ga: American Cancer Society, 2009. Also available online [PUBMED Abstract]
- Homesley HD, Bundy BN, Sedlis A, et al.: Assessment of current International Federation of Gynecology and Obstetrics staging of vulvar carcinoma relative to prognostic factors for survival (a Gynecologic Oncology Group study). Am J Obstet Gynecol 164 (4): 997-1003; discussion 1003-4, 1991. [PUBMED Abstract]
- Boyce J, Fruchter RG, Kasambilides E, et al.: Prognostic factors in carcinoma of the vulva. Gynecol Oncol 20 (3): 364-77, 1985. [PUBMED Abstract]
- Sedlis A, Homesley H, Bundy BN, et al.: Positive groin lymph nodes in superficial squamous cell vulvar cancer. A Gynecologic Oncology Group Study. Am J Obstet Gynecol 156 (5): 1159-64, 1987. [PUBMED Abstract]
- Binder SW, Huang I, Fu YS, et al.: Risk factors for the development of lymph node metastasis in vulvar squamous cell carcinoma. Gynecol Oncol 37 (1): 9-16, 1990. [PUBMED Abstract]
- Homesley HD, Bundy BN, Sedlis A, et al.: Prognostic factors for groin node metastasis in squamous cell carcinoma of the vulva (a Gynecologic Oncology Group study) Gynecol Oncol 49 (3): 279-83, 1993. [PUBMED Abstract]
- Macnab JC, Walkinshaw SA, Cordiner JW, et al.: Human papillomavirus in clinically and histologically normal tissue of patients with genital cancer. N Engl J Med 315 (17): 1052-8, 1986. [PUBMED Abstract]
Cellular Classification
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Presented below is an adaptation of the histologic classification of vulvar disease and precursor lesions of cancer of the vulva developed by the International Society for the Study of Vulvar Disease.
- Non-neoplastic epithelial disorders of skin and mucosa
- Lichen sclerosus (lichen sclerosus et atrophicus).
- Squamous cell hyperplasia (formerly hyperplastic dystrophy).
- Other dermatoses.
- Classification of vulvar intraepithelial neoplasia (VIN)
- Mild dysplasia (formerly mild atypia).
- Moderate dysplasia (formerly moderate atypia).
- Severe dysplasia (formerly severe atypia).
- Carcinoma in situ.
- Paget disease of the vulva
- Characteristic large pale cells in epithelium and skin adnexa.
- Other histologies
- Basal cell carcinoma.
- Verrucous carcinoma.
- Sarcoma.
- Histiocytosis X.
- Malignant melanoma.
The diagnosis of vulvar cancer is made by biopsy, which can often be done on an outpatient basis. The patient may be examined under anesthesia. Cystoscopy, proctoscopy, x-ray examination of the lungs, and intravenous urography as needed, are used for staging purposes. Suspected bladder or rectal involvement must be confirmed by biopsy.
Stages are defined by the Federation Internationale de Gynecologie et Obstetrique (FIGO) and the American Joint Committee on Cancer's (AJCC) TNM classifications. 1 The definitions of the T categories correspond to the stages accepted by the FIGO and both systems are included for comparison. Staging is on a surgical rather than a clinical basis. The 1988 FIGO staging system provides far better discrimination of survival between stages than the 1970 FIGO clinical staging system. 2
- Primary tumor (T)
- TX: Primary tumor cannot be assessed
- T0: No evidence of primary tumor
- Tis/0: Carcinoma in situ (preinvasive carcinoma)
- T1/I: Tumor confined to the vulva or vulva and perineum, 2 cm or less in greatest dimension
- T1a/IA: Tumor confined to the vulva or vulva and perineum, 2 cm or less in greatest dimension, and with stromal invasion no greater than 1 mm.
- T1b/IB: Tumor confined to the vulva or vulva and perineum, 2 cm or less in greatest dimension, and with stromal invasion greater than 1 mm.
- T2/II: Tumor confined to the vulva or vulva and perineum, more than 2 cm in greatest dimension
- T3/III: Tumor of any size with contiguous spread to the lower urethra and/or vagina or anus
- T4/IVA: Tumor invades any of the following: upper urethra, bladder mucosa, rectal mucosa, or is fixed to the pubic bone
[Note: The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion.]
- Regional lymph nodes (N)
- NX: Regional lymph nodes cannot be assessed
- N0: No regional lymph node metastasis
- N1/III: Unilateral regional lymph node metastasis
- N2/IVA: Bilateral regional lymph node metastasis
Every effort should be made to determine the site and laterality of lymph node metastases. However, if regional lymph node metastases, NOS is the final diagnosis, then the patient should be staged as N1.
- Distant metastasis (M)
- MX: Distant metastasis cannot be assessed
- M0: No distant metastasis
- M1/IVB: Distant metastasis (including pelvic lymph node metastasis)
- Stage 0
- Tis, N0, M0
- Stage I
- T1, N0, M0
- Stage IA
- T1a, N0, M0
- Stage IB
- T1b, N0, M0
- Stage II
- T2, N0, M0
- Stage III
- T1, N1, M0
- T2, N1, M0
- T3, N0, M0
- T3, N1, M0
- Stage IVA
- T1, N2, M0
- T2, N2, M0
- T3, N2, M0
- T4, any N, M0
- Stage IVB
- Any T, any N, M1
References:
- Vulva. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 243-9. [PUBMED Abstract]
- Hopkins MP, Reid GC, Johnston CM, et al.: A comparison of staging systems for squamous cell carcinoma of the vulva. Gynecol Oncol 47 (1): 34-7, 1992. [PUBMED Abstract]
Treatment Option Overview
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Standard treatment in vulvar cancer is surgery or, for most patients with stage III or IV disease, surgery supplemented by external-beam radiation therapy. 1 2 3 Newer strategies integrate possible therapeutic advantages of surgery, radiation therapy, and chemotherapy and tailor the treatment to the extent of clinical and pathologic disease. Because of the psychosexual consequences and significant morbidity associated with standard radical vulvectomy, there is a definite trend toward vulvar conservation and individualized management of patients with early vulvar cancer. Since invasive and preinvasive neoplasms of the vulva may be HPV-induced and the carcinogenic effect may be widespread in the vulvar epithelium, close follow-up of patients is mandatory so that early detection of recurrent or second tumors is possible. Because there are few patients with far advanced disease, and they are often elderly, minimal data has been generated on responses, and therefore there is no standard chemotherapy for patients with this stage of disease. Physicians should consider including patients with stage III or IV disease in clinical trials evaluating the following adjuncts to standard surgical procedures: radiation sensitizers, chemotherapy in phase II trials, and combined modality studies. The Gynecologic Oncology Group is investigating the feasibility of preoperative chemotherapy plus radiation therapy as a neoadjuvant to surgery for advanced vulvar cancer.
References:
- Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71 (4 Suppl): 1673-7, 1993. [PUBMED Abstract]
- Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecol Oncol 42 (1): 9-21, 1991. [PUBMED Abstract]
- Homesley HD, Bundy BN, Sedlis A, et al.: Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstet Gynecol 68 (6): 733-40, 1986. [PUBMED Abstract]
Simple vulvectomy gives a 5-year survival rate of essentially 100% but is seldom indicated. Other more limited surgical procedures produce equivalent results and are less deforming. The choice of treatment depends on the extent of the disease.
Vulvar intraepithelial neoplasia (VIN) occupying nonhairy areas can be considered an epithelial disease; however, VIN occupying hairy sites usually involves the pilosebaceous apparatus and requires a greater depth of destruction or excision. 1 Whatever procedure is used, a significant number of patients develop a recurrence with the most common sites being the perianal skin, presacral area, and clitoral hood. 2 The use of topical fluorouracil is not a reliable first choice for treatment.
- Wide local excision or laser beam therapy or a combination of both.
- Skinning vulvectomy with or without grafting.
- Use of 5% fluorouracil cream (response rate of 50%60%). 3