Purpose of This PDQ Summary
General Information
Cellular Classification
Stage Information

TNM Definitions AJCC Stage Groupings

Treatment Option Overview
Stage 0 Vulvar Cancer

Current Clinical Trials

Stage I Vulvar Cancer

Current Clinical Trials

Stage II Vulvar Cancer

Current Clinical Trials

Stage III Vulvar Cancer

Current Clinical Trials

Stage IV Vulvar Cancer

Current Clinical Trials

Recurrent Vulvar Cancer

Current Clinical Trials

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Changes to This Summary (05/22/2008)
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Purpose of This PDQ Summary

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.

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General Information

Note: Estimated new cases and deaths from vulvar cancer in the United States in 2008:[1]

• New cases: 3,460.
• Deaths: 870.

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.[2-6] 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

1. American Cancer Society.: Cancer Facts and Figures 2008. Atlanta, Ga: American Cancer Society, 2008. Also available online. Last accessed October 1, 2008. 
   
   
2. 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]
   
   
3. Boyce J, Fruchter RG, Kasambilides E, et al.: Prognostic factors in carcinoma of the vulva. Gynecol Oncol 20 (3): 364-77, 1985.  [PUBMED Abstract]
   
   
4. 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]
   
   
5. 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]
   
   
6. 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]
   
   
7. 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]
   
   

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Cellular Classification

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)

1. Mild dysplasia (formerly mild atypia).
2. Moderate dysplasia (formerly moderate atypia).
3. Severe dysplasia (formerly severe atypia).
4. 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.

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Stage Information

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]

TNM Category/FIGO Stage

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

1. Vulva. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 243-9. 
   
   
2. 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]
   
   

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Treatment Option Overview

Standard treatment in vulvar cancer is surgery or, for most patients with stage III or IV disease, surgery supplemented by external-beam radiation therapy.[1-3] 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

1. Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71 (4 Suppl): 1673-7, 1993.  [PUBMED Abstract]
   
   
2. 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]
   
   
3. 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]
   
   

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Stage 0 Vulvar Cancer

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.

Standard treatment options:

1. Wide local excision or laser beam therapy or a combination of both.
2. Skinning vulvectomy with or without grafting.
3. Use of 5% fluorouracil cream (response rate of 50%–60%).[3]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage 0 vulvar cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Wright VC, Chapman W: Intraepithelial neoplasia of the lower female genital tract: etiology, investigation, and management. Semin Surg Oncol 8 (4): 180-90, 1992 Jul-Aug.  [PUBMED Abstract]
   
   
2. Di Saia PJ, Rich WM: Surgical approach to multifocal carcinoma in situ of the vulva. Am J Obstet Gynecol 140 (2): 136-45, 1981.  [PUBMED Abstract]
   
   
3. Woodruff JD, Julian C, Puray T, et al.: The contemporary challenge of carcinoma in situ of the vulva. Am J Obstet Gynecol 115 (5): 677-86, 1973.  [PUBMED Abstract]
   
   

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Stage I Vulvar Cancer

Radical vulvectomy has been associated with 5-year survival rates in excess of 90%. The choice of treatment depends on various tumor and patient factors.

Standard treatment options:

1. For microinvasive lesions (<1 mm invasion) with no associated severe vulvar dystrophy, a wide (5–10 mm) excision is indicated. For all other stage I lesions, if well lateralized, without diffuse severe dystrophy, and with clinically negative nodes, a radical local excision with complete unilateral lymphadenectomy should be performed.[1] Candidates for this procedure should have lesions 2 cm or less in diameter with 5 mm or less invasion, no capillary lymphatic space invasion, and clinically uninvolved nodes.[2] A literature review suggests that the local recurrence rate is 7.2% after radical local excision compared with 6.3% after radical vulvectomy.[3]



2. Radical vulvectomy with bilateral inguinal and femoral node dissection. The morbidity of this operation can be reduced by using separate groin incisions and unilateral or superficial lymphadenectomy for select early lesions.[4] Also, the definition of radical vulvectomy is being extended with the realization that the effect of radical surgery is limited by the closest resection margin rather than the achievement of total organ ablation.[5] One study suggested that the margin of clearance of the tumor is the best predictor of local recurrence. All of the recurrences were with surgically free margins less than 8 mm.[6]

   In a Gynecologic Oncology Group (GOG) randomized trial, radiation therapy to the groin for patients with clinical N0 disease led to an inferior survival secondary to an increased groin failure rate compared with groin dissection and adjuvant radiation therapy for positive groin nodes.[7] Unfortunately, because the clinical trial was poorly designed with regard to adequacy of dose at the depth of the groin nodes, the question of whether elective nodal radiation therapy has a better outcome than groin dissection was not satisfactorily answered. A retrospective study with similar patient numbers and superior radiation therapy design contradicts the GOG data and reports no significant survival advantage to groin dissection versus radiation therapy to the groin.[8] Therefore, radiation therapy to the groin for patients with clinical N0 disease is an alternative to groin dissection for women who refuse or are deemed medically unfit to withstand groin dissections.




3. For those few patients unable to tolerate radical vulvectomy or deemed unsuitable for surgery because of site or extent of disease, radical radiation therapy may result in long-term survival.[8-11]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I vulvar cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Malfetano JH, Piver MS, Tsukada Y, et al.: Univariate and multivariate analyses of 5-year survival, recurrence, and inguinal node metastases in stage I and II vulvar carcinoma. J Surg Oncol 30 (2): 124-31, 1985.  [PUBMED Abstract]
   
   
2. Stehman FB, Bundy BN, Dvoretsky PM, et al.: Early stage I carcinoma of the vulva treated with ipsilateral superficial inguinal lymphadenectomy and modified radical hemivulvectomy: a prospective study of the Gynecologic Oncology Group. Obstet Gynecol 79 (4): 490-7, 1992.  [PUBMED Abstract]
   
   
3. Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71 (4 Suppl): 1673-7, 1993.  [PUBMED Abstract]
   
   
4. Hoffman MS, Roberts WS, Lapolla JP, et al.: Recent modifications in the treatment of invasive squamous cell carcinoma of the vulva. Obstet Gynecol Surv 44 (4): 227-33, 1989.  [PUBMED Abstract]
   
   
5. 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]
   
   
6. Heaps JM, Fu YS, Montz FJ, et al.: Surgical-pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva. Gynecol Oncol 38 (3): 309-14, 1990.  [PUBMED Abstract]
   
   
7. Stehman FB, Bundy BN, Thomas G, et al.: Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. Int J Radiat Oncol Biol Phys 24 (2): 389-96, 1992.  [PUBMED Abstract]
   
   
8. Petereit DG, Mehta MP, Buchler DA, et al.: Inguinofemoral radiation of N0,N1 vulvar cancer may be equivalent to lymphadenectomy if proper radiation technique is used. Int J Radiat Oncol Biol Phys 27 (4): 963-7, 1993.  [PUBMED Abstract]
   
   
9. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989.  [PUBMED Abstract]
   
   
10. Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993.  [PUBMED Abstract]
    
    
11. Kumar PP, Good RR, Scott JC: Techniques for management of vulvar cancer by irradiation alone. Radiat Med 6 (4): 185-91, 1988 Jul-Aug.  [PUBMED Abstract]
    
    

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Stage II Vulvar Cancer

Radical vulvectomy and bilateral inguinal and femoral node dissection, with care taken to ensure tumor-free margins, is the standard therapy and has been associated with 5-year survival rates of 80% to 90%, depending on the size of the primary tumor. The definition of radical vulvectomy is being extended with the realization that the effect of radical surgery is limited by the closest resection margin rather than the achievement of total organ ablation.[1]

Standard treatment options:

1. Modified radical vulvectomy with bilateral inguinal node and femoral node dissection. The lines of surgical resection should clear the tumor by 10 mm.[2] The morbidity of this operation can be reduced by using separate groin incisions and unilateral or superficial lymphadenectomy for select early lesions.[3] Adjuvant local radiation therapy may be indicated for surgical margins less than 8 mm, capillary-lymphatic space invasion, and thickness greater than 5 mm, particularly if the patient also has positive nodes.[1,4]

   In a Gynecologic Oncology Group (GOG) randomized trial, radiation therapy to the groin for patients with clinical N0 disease led to an inferior survival secondary to an increased groin failure rate compared with groin dissection and adjuvant radiation therapy for positive groin nodes.[5] Unfortunately, because the clinical trial was poorly designed with regard to adequacy of dose at the depth of the groin nodes, the question of whether elective nodal radiation therapy has a better outcome than groin dissection was not satisfactorily answered. A retrospective study with similar patient numbers and superior radiation therapy design contradicts the GOG data and reports no significant survival advantage to groin dissection versus radiation therapy to the groin.[6] Therefore, radiation therapy to the groin for patients with clinical N0 disease is an alternative to groin dissection for women who refuse or are deemed medically unfit to withstand groin dissections.




2. For those few patients unable to tolerate radical surgery or deemed unsuitable for surgery because of site or extent of disease, radical radiation therapy may result in long-term survival.[6-9]

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II vulvar cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. 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]
   
   
2. Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71 (4 Suppl): 1673-7, 1993.  [PUBMED Abstract]
   
   
3. Hoffman MS, Roberts WS, Lapolla JP, et al.: Recent modifications in the treatment of invasive squamous cell carcinoma of the vulva. Obstet Gynecol Surv 44 (4): 227-33, 1989.  [PUBMED Abstract]
   
   
4. Faul CM, Mirmow D, Huang Q, et al.: Adjuvant radiation for vulvar carcinoma: improved local control. Int J Radiat Oncol Biol Phys 38 (2): 381-9, 1997.  [PUBMED Abstract]
   
   
5. Stehman FB, Bundy BN, Thomas G, et al.: Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. Int J Radiat Oncol Biol Phys 24 (2): 389-96, 1992.  [PUBMED Abstract]
   
   
6. Petereit DG, Mehta MP, Buchler DA, et al.: Inguinofemoral radiation of N0,N1 vulvar cancer may be equivalent to lymphadenectomy if proper radiation technique is used. Int J Radiat Oncol Biol Phys 27 (4): 963-7, 1993.  [PUBMED Abstract]
   
   
7. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989.  [PUBMED Abstract]
   
   
8. Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993.  [PUBMED Abstract]
   
   
9. Kumar PP, Good RR, Scott JC: Techniques for management of vulvar cancer by irradiation alone. Radiat Med 6 (4): 185-91, 1988 Jul-Aug.  [PUBMED Abstract]
   
   

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Stage III Vulvar Cancer

Radical vulvectomy with inguinal and femoral lymphadenectomy is the standard therapy. The definition of radical vulvectomy is being extended with the realization that the effect of radical surgery is limited by the closest resection margin, rather than the achievement of total organ ablation.[1] Nodal involvement is a key determinant of survival. The 5-year survival rate for patients with unilateral nodal involvement is 70%, with a decrease to 30% for those with three or more unilateral nodes involved.[2]

In a randomized trial from the Gynecologic Oncology Group, patients with two or more pathologically positive groin nodes had significantly better survival with radiation therapy to the groin and pelvis than with pelvic node dissection. Patients on both arms of the trial received radical vulvectomy and bilateral inguinal and femoral groin node dissections. Patterns of failure have shown a significant decrease in groin failures with radiation therapy to the groin and pelvis compared with pelvic node dissection.[3]

Standard treatment options:

1. Modified radical vulvectomy with inguinal and femoral node dissection. Radiation therapy to the pelvis and groin should be performed if inguinal nodes are positive.



2. Radical vulvectomy with inguinal and femoral node dissection followed by radiation therapy to the vulva in patients with large primary lesions and narrow margins. Localized adjuvant radiation therapy consisting of 45 Gy to 50 Gy may also be indicated when there is capillary-lymphatic space invasion and a thickness of greater than 5 mm, particularly if the nodes are involved.[1] Radiation therapy to the pelvis and groin should be performed if two or more groin nodes are involved.[3]



3. Preoperative radiation therapy may be used in selected cases to improve operability and even decrease the extent of surgery required.[4,5] A radiation dose of up to 55 Gy with concomitant fluorouracil (5-FU) has been suggested.[1]



4. For those patients unable to tolerate radical vulvectomy or who are deemed unsuitable for surgery because of site or extent of disease, radical radiation therapy may result in long-term survival.[6,7] Where radiation therapy is being tested for primary definitive treatment of vulvar cancer, some prefer to add concurrent 5-FU or 5-FU and cisplatin.[1,8-11] Four phase II trials of concurrent 5-FU with or without cisplatin with radiation resulted in complete response rates of 53% to 89% for primary unresectable disease or for those who would require exenterative surgery.[8-11] With a median follow-up of 37 months, two series report crude disease-free survival rates of 47% to 84%.[9,10] Radiation complications of late fibrosis, atrophy, telangiectasia, and necrosis are minimized if the radiation fraction size is less than or equal to 1.8 Gy and excessive total doses are not used.[1,8-11] Doses of at least 54 Gy but less than 65 Gy should be used.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III vulvar cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. 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]
   
   
2. 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]
   
   
3. 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]
   
   
4. Boronow RC, Hickman BT, Reagan MT, et al.: Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer. II. Results, complications, and dosimetric and surgical considerations. Am J Clin Oncol 10 (2): 171-81, 1987.  [PUBMED Abstract]
   
   
5. Anderson JM, Cassady JR, Shimm DS, et al.: Vulvar carcinoma. Int J Radiat Oncol Biol Phys 32 (5): 1351-7, 1995.  [PUBMED Abstract]
   
   
6. Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993.  [PUBMED Abstract]
   
   
7. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989.  [PUBMED Abstract]
   
   
8. Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecol Oncol 47 (1): 14-20, 1992.  [PUBMED Abstract]
   
   
9. Berek JS, Heaps JM, Fu YS, et al.: Concurrent cisplatin and 5-fluorouracil chemotherapy and radiation therapy for advanced-stage squamous carcinoma of the vulva. Gynecol Oncol 42 (3): 197-201, 1991.  [PUBMED Abstract]
   
   
10. Koh WJ, Wallace HJ 3rd, Greer BE, et al.: Combined radiotherapy and chemotherapy in the management of local-regionally advanced vulvar cancer. Int J Radiat Oncol Biol Phys 26 (5): 809-16, 1993.  [PUBMED Abstract]
    
    
11. Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecol Oncol 34 (3): 263-7, 1989.  [PUBMED Abstract]
    
    

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Stage IV Vulvar Cancer

In a randomized trial from the Gynecologic Oncology Group (GOG), patients with two or more pathologically positive groin nodes had significantly better survival with radiation therapy to the pelvis than with pelvic node dissection.[1] Patients in both arms of the trial received radical vulvectomy and bilateral superficial and deep groin node dissections. Patterns of failure have shown a significant decrease in groin failure with radiation therapy to the groin and pelvis compared with pelvic node dissection.

Standard treatment options:

1. Radical vulvectomy and pelvic exenteration.



2. Surgery followed by radiation therapy to the vulva for large resected lesions with narrow margins. Localized adjuvant radiation therapy consisting of 45 Gy to 50 Gy may also be indicated when there is capillary-lymphatic space invasion and thickness greater than 5 mm, particularly if the nodes are involved.[2] Radiation therapy to the pelvis and groin should be performed if two or more groin nodes are involved.[1]



3. Radiation therapy of large primary lesions to improve operability followed by radical surgery.[3,4] A radiation dose of up to 55 Gy with concomitant fluorouracil (5-FU) has been suggested.[2]



4. For those patients unable to tolerate radical vulvectomy or who are deemed unsuitable for surgery because of site or extent of disease, radical radiation therapy may result in long-term survival.[5,6] Where radiation therapy is being tested for primary definitive treatment of vulvar cancer, some prefer to add concurrent 5-FU or 5-FU and cisplatin.[2,7-10] The GOG is investigating the feasibility of preoperative chemotherapy plus radiation therapy given as a neoadjuvant to surgery for advanced vulvar cancer.[11] Four phase II trials of concurrent 5-FU with or without cisplatin with radiation therapy resulted in complete response rates of 53% to 89% for primary unresectable disease or for those who would require exenterative surgery.[7-10] With a median follow-up of 37 months, two series report crude disease-free survival rates of 47% to 84%.[7-10] Radiation complications of late fibrosis, atrophy, telangiectasia, and necrosis are minimized if the radiation fraction size is less than or equal to 1.8 Gy and excessive total doses are not used.[2,7-10] Doses of at least 54 Gy but less than 65 Gy should be used.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV vulvar cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. 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]
   
   
2. 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]
   
   
3. Boronow RC, Hickman BT, Reagan MT, et al.: Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer. II. Results, complications, and dosimetric and surgical considerations. Am J Clin Oncol 10 (2): 171-81, 1987.  [PUBMED Abstract]
   
   
4. Anderson JM, Cassady JR, Shimm DS, et al.: Vulvar carcinoma. Int J Radiat Oncol Biol Phys 32 (5): 1351-7, 1995.  [PUBMED Abstract]
   
   
5. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. Br J Radiol 62 (734): 145-7, 1989.  [PUBMED Abstract]
   
   
6. Perez CA, Grigsby PW, Galakatos A, et al.: Radiation therapy in management of carcinoma of the vulva with emphasis on conservation therapy. Cancer 71 (11): 3707-16, 1993.  [PUBMED Abstract]
   
   
7. Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecol Oncol 47 (1): 14-20, 1992.  [PUBMED Abstract]
   
   
8. Berek JS, Heaps JM, Fu YS, et al.: Concurrent cisplatin and 5-fluorouracil chemotherapy and radiation therapy for advanced-stage squamous carcinoma of the vulva. Gynecol Oncol 42 (3): 197-201, 1991.  [PUBMED Abstract]
   
   
9. Koh WJ, Wallace HJ 3rd, Greer BE, et al.: Combined radiotherapy and chemotherapy in the management of local-regionally advanced vulvar cancer. Int J Radiat Oncol Biol Phys 26 (5): 809-16, 1993.  [PUBMED Abstract]
   
   
10. Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecol Oncol 34 (3): 263-7, 1989.  [PUBMED Abstract]
    
    
11. Keys H: Gynecologic Oncology Group randomized trials of combined technique therapy for vulvar cancer. Cancer 71 (4 Suppl): 1691-6, 1993.  [PUBMED Abstract]
    
    

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Recurrent Vulvar Cancer

Patients should be followed carefully to detect recurrent disease as early as possible. Both treatment and outcome depend on the site and extent of recurrence.[1] Radical excision of localized recurrence provides an approximate 5-year survival rate of 56% when the regional nodes are not involved.[2] Palliative radiation therapy is used in some patients. Radiation therapy with or without 5-FU may be curative in some patients with a small local recurrence.[3-5] When local recurrence occurs more than 2 years after primary treatment, a combination of radiation therapy and surgery may result in a 5-year survival rate of greater than 50%.[6,7]

Standard treatment options:

1. Wide local excision with or without radiation in those patients with local recurrence.



2. Radical vulvectomy and pelvic exenteration.



3. Synchronous radiation and cytotoxic chemotherapy with or without surgery.[4]

There is no standard chemotherapy or other systemic treatment effective in patients with metastatic disease. Such patients should be considered for clinical trials.

Treatment options under clinical evaluation:

• Clinical trials.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent vulvar cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

1. Piura B, Masotina A, Murdoch J, et al.: Recurrent squamous cell carcinoma of the vulva: a study of 73 cases. Gynecol Oncol 48 (2): 189-95, 1993.  [PUBMED Abstract]
   
   
2. Hopkins MP, Reid GC, Morley GW: The surgical management of recurrent squamous cell carcinoma of the vulva. Obstet Gynecol 75 (6): 1001-5, 1990.  [PUBMED Abstract]
   
   
3. Miyazawa K, Nori D, Hilaris BS, et al.: Role of radiation therapy in the treatment of advanced vulvar carcinoma. J Reprod Med 28 (8): 539-41, 1983.  [PUBMED Abstract]
   
   
4. Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecol Oncol 47 (1): 14-20, 1992.  [PUBMED Abstract]
   
   
5. Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecol Oncol 34 (3): 263-7, 1989.  [PUBMED Abstract]
   
   
6. Podratz KC, Symmonds RE, Taylor WF, et al.: Carcinoma of the vulva: analysis of treatment and survival. Obstet Gynecol 61 (1): 63-74, 1983.  [PUBMED Abstract]
   
   
7. Shimm DS, Fuller AF, Orlow EL, et al.: Prognostic variables in the treatment of squamous cell carcinoma of the vulva. Gynecol Oncol 24 (3): 343-58, 1986.  [PUBMED Abstract]
   
   

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Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

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Search the NCI Web site

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

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Changes to This Summary (05/22/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

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More Information

About PDQ

• PDQ® - NCI's Comprehensive Cancer Database.

  Full description of the NCI PDQ database.

Additional PDQ Summaries

• PDQ® Cancer Information Summaries: Adult Treatment

  Treatment options for adult cancers.

• PDQ® Cancer Information Summaries: Pediatric Treatment

  Treatment options for childhood cancers.

• PDQ® Cancer Information Summaries: Supportive and Palliative Care

  Side effects of cancer treatment, management of cancer-related complications and pain, and psychosocial concerns.

• PDQ® Cancer Information Summaries: Screening/Detection (Testing for Cancer)

  Tests or procedures that detect specific types of cancer.

• PDQ® Cancer Information Summaries: Prevention

  Risk factors and methods to increase chances of preventing specific types of cancer.

• PDQ® Cancer Information Summaries: Genetics

  Genetics of specific cancers and inherited cancer syndromes, and ethical, legal, and social concerns.

• PDQ® Cancer Information Summaries: Complementary and Alternative Medicine

  Information about complementary and alternative forms of treatment for patients with cancer.

Important:

This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

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