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Vulvar cancer


Table of Contents

GENERAL INFORMATION
CELLULAR CLASSIFICATION
STAGE INFORMATION
TREATMENT OPTION OVERVIEW
STAGE 0 VULVAR CANCER
STAGE I VULVAR CANCER
STAGE II VULVAR CANCER
STAGE III VULVAR CANCER
STAGE IV VULVAR CANCER
RECURRENT VULVAR CANCER

GENERAL INFORMATION

Vulvar cancer is highly curable when diagnosed in an early stage. Survival is most dependent on the pathologic status of the inguinal nodes. In operable patients without nodal involvement, the overall survival rate is 90%. However, with nodal involvement, the overall 5-year survival rate is approximately 50%- 60%.[1] 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.[1-5] Overall, about 30% of operable patients 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 3 or more unilateral nodes or 2 or more bilateral nodes had a 29% 5-year survival rate. Intermediate groups with intermediate survival were also identified.[1] These discriminants were most useful within FIGO stage III disease. 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, although other histologic types do occur.

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 most common site of involvement is the labia majora (about 50% of cases). The labia minora accounts for 15%-20% of cases. The clitoris and Bartholin's glands are less frequently involved.[6]

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. 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). American Journal of Obstetrics and Gynecology 164(4): 997-1004, 1991.

  2. Boyce J, Fruchter RG, Kasambilides E, et al.: Prognostic factors in carcinoma of the vulva. Gynecologic Oncology 20(3): 364-377, 1985.

  3. Sedlis A, Homesley H, Bundy BN, et al.: Positive groin lymph nodes in superficial squamous cell vulvar cancer: a Gynecologic Oncology Group study. American Journal of Obstetrics and Gynecology 156(5): 1159-1164, 1987.

  4. Binder SW, Huang I, Fu YS, et al.: Risk factors for the development of lymph node metastasis in vulvar squamous cell carcinoma. Gynecologic Oncology 37(1): 9-16, 1990.

  5. 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). Gynecologic Oncology 49(3): 279-283, 1993.

  6. Macnab JC, Walkinshaw SA, Cordiner JW, et al.: Human papillomavirus in clinically and histologically normal tissue of patients with genital cancer. New England Journal of Medicine 315(17): 1052-1058, 1986.


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) I. mild dysplasia (formerly mild atypia) II. moderate dysplasia (formerly moderate atypia) III. severe dysplasia (formerly severe atypia) IV. carcinoma in situ Paget's disease of the vulva (characteristic large pale cells in epithelium and skin adnexa) Other histologies (basal cell carcinoma, verrucous carcinoma, sarcoma, histiocytosis X, or malignant melanoma)


STAGE INFORMATION

The diagnosis is made by biopsy, which can often be done on an outpatient basis. The patient may be examined under anesthesia if necessary. 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 d'Obstetrique (FIGO) and the American Joint Committee on Cancer's (AJCC) TNM classifications.[1,2] Staging is now 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.[3]



-- TNM definitions --

Primary tumor (T)
  TX:  Primary tumor cannot be assessed
  T0:  No evidence of primary tumor
  Tis:  Carcinoma in situ (preinvasive carcinoma)
  T1:  Tumor confined to the vulva and/or perineum, 2 cm or less in greatest
       dimension
     T1a:  Tumor confined to the vulva and/or perineum, 2 cm or less in
           greatest dimension, and with stromal invasion no greater than 1 mm*
     T1b:  Tumor confined to the vulva and/or perineum, 2 cm or less in
           greatest dimension, and with stromal invasion greater than 1 mm*
  T2:  Tumor confined to the vulva and/or perineum, more than 2 cm in greatest
       dimension
  T3:  Tumor of any size with adjacent spread to the lower urethra and/or
       vagina or anus
  T4:  Tumor invades any of the following:  upper urethral mucosa, 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:  Unilateral regional lymph node metastasis
  N2:  Bilateral regional lymph node metastasis

Distant metastasis (M)
  MX:  Distant metastasis cannot be assessed
  M0:  No distant metastasis
  M1:  Distant metastasis (including pelvic lymph node metastasis)

-- AJCC stage groupings --

-- Stage 0 --

   Tis, 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

-- FIGO stage groupings --

-- Stage 0 -- 
Stage 0 vulvar cancer is defined as carcinoma in situ, intraepithelial
carcinoma.

-- Stage I -- 
Stage I vulvar cancer is defined as lesions 2 cm or less confined to the vulva
or perineum.  There are no lymph node metastases.
  Stage IA:  Stage IA vulvar cancer is defined as lesions 2 cm or less in size  
             confined to the vulva or perineum with stromal invasion no greater 
             than 1.0 mm.*  There are no nodal mestastases.
  Stage IB:  Stage IB vulvar cancer is defined as lesions 2 cm or less in size
             confined to the vulva or perineum and with stromal invasion
             greater than 1.0 mm.*  There are no nodal metastases.

-- Stage II --
Stage II vulvar cancer is defined as tumor confined to the vulva and/or
perineum or more than 2 cm in the greatest dimension with no nodal metastases.

-- Stage III --    
Stage III vulvar cancer is defined as tumor of any size arising on the vulva
and/or perineum with 1) adjacent spread to the lower urethra and/or the vagina,
or the anus; and/or 2) unilateral regional lymph node metastases.

-- Stage IV --      
Stage IV vulvar cancer can be designated as stage IVA or stage IVB.
  Stage IVA:  Stage IVA vulvar cancer is defined as tumor invading any of the
              following:  upper urethra, bladder mucosa, rectal mucosa, pelvic
              bone and/or bilateral regional nodal metastases.
  Stage IVB:  Stage IVB vulvar cancer is defined as any distant metastasis
              including pelvic lymph nodes.

*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.

References:

  1. Shepherd JH: Cervical and vulva cancer: changes in FIGO definitions of staging. British Journal of Obstetrics and Gynaecology 103(5): 405-406, 1996.

  2. Vulva. In: American Joint Committee on Cancer: AJCC Cancer Staging Manual. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 181-184.

  3. Hopkins MP, Reid GC, Johnston CM, et al.: A comparison of staging systems for squamous cell carcinoma of the vulva. Gynecologic Oncology 47(1): 34-37, 1992.

  4. International Society for the Study of Vulvar Diseases Task Force: Microinvasive cancer of the vulva: report of the ISSVD Task Force. Journal of Reproductive Medicine 29(7): 454-456, 1984.


TREATMENT OPTION OVERVIEW

Standard treatment in vulvar cancer is surgery or, for most with stage III/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 and 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.[4]

The designations in PDQ that treatments are "standard" or "under clinical evaluation" are not to be used as a basis for reimbursement determinations.

References:

  1. Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71(4, Suppl): 1673-1677, 1993.

  2. Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecologic Oncology 42(1): 9-21, 1991.

  3. Homesley HD, Bundy BN, Sedlis A, et al.: Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstetrics and Gynecology 68(6): 733-740, 1986.

  4. Keys H: Gynecologic Oncology Group randomized trials of combined technique therapy for vulvar cancer. Cancer 71(4, Suppl): 1691-1696, 1993.


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. 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.

Treatment options:

Treatment choice depends on the extent of disease.

Standard:
1. Wide local excision or laser beam therapy or a combination of both.

2. Skinning vulvectomy with or without grafting.

3. 5% fluorouracil cream (response rate of 50%-60%).[3]

References:

  1. Wright VC, Chapman W: Intraepithelial neoplasia of the lower female genital tract: etiology, investigation, and management. Seminars in Surgical Oncology 8(4): 180-190, 1992.

  2. Di Saia PJ, Rich WM: Surgical approach to multifocal carcinoma in situ of the vulva. American Journal of Obstetrics and Gynecology 140(2): 136-142, 1981.

  3. Woodruff JD, Julian C, Puray T, et al.: The contemporary challenge of carcinoma of the vulva. American Journal of Obstetrics and Gynecology 115(5): 677-686, 1973.


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.

Equivalent treatment options:

Standard:

1. For microinvasive lesions (<1 millimeter invasion) with no associated severe vulvar dystrophy, a wide (5-10 millimeters) 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 centimeters or less in diameter with 5 millimeters 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 millimeters.[6] In a Gynecologic Oncology Group (GOG) randomized trial, groin irradiation for clinical N0 patients led to an inferior survival secondary to an increased groin failure rate compared to groin dissection and adjuvant irradiation for positive groin nodes.[7] Unfortunately, because the protocol was poorly designed with regard to adequacy of dose at the depth of the groin nodes, the question of whether elective nodal irradiation has a better outcome than groin dissection was not satisfactorily answered by the GOG study. A retrospective study with similar patient numbers and superior irradiation design contradicts the GOG data and reports no significant survival advantage to groin dissection over groin irradiation.[8] Therefore, groin irradiation for clinical N0 patients 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]

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. Journal of Surgical Oncology 30(2): 124-131, 1985.

  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. Obstetrics and Gynecology 79(4): 490-497, 1992.

  3. Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71(4, Suppl): 1673-1677, 1993.

  4. Hoffman MS, Roberts WS, LaPolla JP, et al.: Recent modifications in the treatment of invasive squamous cell carcinoma of the vulva. Obstetrical and Gynecological Survey 44(4): 227-233, 1989.

  5. Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecologic Oncology 42(1): 9-21, 1991.

  6. Heaps JM, Fu YS, Montz FJ, et al.: Surgical-pathologic variables predictive of local recurrence in squamous cell carcinoma of the vulva. Gynecologic Oncology 38(3): 309-314, 1990.

  7. Stehman FB, Bundy BN, Thomas G, et al.: Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. International Journal of Radiation Oncology, Biology, Physics 24(2): 389-396, 1992.

  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. International Journal of Radiation Oncology, Biology, Physics 27(4): 963-967, 1993.

  9. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. British Journal of Radiology 62(734): 145-147, 1989.

  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): 3707-3716, 1993.

  11. Kumar PP, Good RR, Scott JC: Techniques for management of vulvar cancer by irradiation alone. Radiation Medicine 6(4): 185-191, 1988.


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%-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]

Treatment options:

Standard:

1. Modified radical vulvectomy with bilateral inguinal node and femoral node dissection. The lines of surgical resection should clear the tumor by 10 millimeters.[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 millimeters, capillary-lymphatic space invasion, and thickness greater than 5 millimeters, particularly if the patient also has positive nodes.[1,4] In a Gynecologic Oncology Group (GOG) randomized trial, groin irradiation for clinical N0 patients led to an inferior survival secondary to an increased groin failure rate compared to groin dissection and adjuvant irradiation for positive groin nodes.[5] Unfortunately, because the protocol was poorly designed with regard to adequacy of dose at the depth of the groin nodes, the question of whether elective nodal irradiation has a better outcome than groin dissection was not satisfactorily answered by the GOG study. A retrospective study with similar patient numbers and superior irradiation design contradicts the GOG data and reports no significant survival advantage to groin dissection over groin irradiation.[6] Therefore, groin irradiation for clinical N0 patients 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]

References:

  1. Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecologic Oncology 42(1): 9-21, 1991.

  2. Hacker NF, Van der Velden J: Conservative management of early vulvar cancer. Cancer 71(4, Suppl): 1673-1677, 1993.

  3. Hoffman MS, Roberts WS, LaPolla JP, et al.: Recent modifications in the treatment of invasive squamous cell carcinoma of the vulva. Obstetrical and Gynecological Survey 44(4): 227-233, 1989.

  4. Faul CM, Mirmow D, Huang Q, et al.: Adjuvant radiation for vulvar carcinoma: improved local control. International Journal of Radiation Oncology, Biology, Physics 38(2): 381-389, 1997.

  5. Stehman FB, Bundy BN, Thomas G, et al.: Groin dissection versus groin radiation in carcinoma of the vulva: a Gynecologic Oncology Group study. International Journal of Radiation Oncology, Biology, Physics 24(2): 389-396, 1992.

  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. International Journal of Radiation Oncology, Biology, Physics 27(4): 963-967, 1993.

  7. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. British Journal of Radiology 62(734): 145-147, 1989.

  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): 3707-3716, 1993.

  9. Kumar PP, Good RR, Scott JC: Techniques for management of vulvar cancer by irradiation alone. Radiation Medicine 6(4): 185-191, 1988.


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 groin and pelvic irradiation 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 groin and pelvic irradiation compared to pelvic node dissection.[3]

Treatment options:

Standard:

1. Modified radical vulvectomy with inguinal and femoral node dissection. Pelvic and groin irradiation 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 45-50 Gy may also be indicated when there is capillary-lymphatic space invasion and a thickness of greater than 5 millimeters, particularly if the nodes are involved.[1] Pelvic and groin irradiation 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 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%-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%-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 180 cGy and excessive total doses are not used.[1,8-11] Doses of at least 54 Gy, but less than 65 Gy, should be used.

References:

  1. Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecologic Oncology 42(1): 9-21, 1991.

  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). American Journal of Obstetrics and Gynecology 164(4): 997-1004, 1991.

  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). Gynecologic Oncology 49(3): 279-283, 1993.

  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. American Journal of Clinical Oncology 10(2): 171-181, 1987.

  5. Anderson JM, Cassady JR, Shimm DS, et al.: Vulvar carcinoma. International Journal of Radiation Oncology, Biology, Physics 32(5): 1351-1357, 1995.

  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): 3707-3716, 1993.

  7. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. British Journal of Radiology 62(734): 145-147, 1989.

  8. Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecologic Oncology 47(1): 14-20, 1992.

  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. Gynecologic Oncology 42(3): 197-201, 1991.

  10. Koh WJ, Wallace HJ, Greer BE, et al.: Combined radiotherapy and chemotherapy in the management of local-regionally advanced vulvar cancer. International Journal of Radiation Oncology, Biology, Physics 26(5): 809-816, 1993.

  11. Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecologic Oncology 34(3): 263-267, 1989.


STAGE IV VULVAR CANCER

In a randomized trial from the Gynecologic Oncology Group, patients with two or more pathologically positive groin nodes had significantly better survival with pelvic irradiation than with pelvic node dissection. 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 groin and pelvic irradiation compared to pelvic node dissection.[1]

Treatment options:

Standard:

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 45-50 Gy may also be indicated when there is capillary-lymphatic space invasion and thickness greater than 5 millimeters, particularly if the nodes are involved.[2] Pelvic and groin irradiation should be performed if two or more groin nodes are involved.[1]

3. radiation therapy of large primary lesion 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 Gynecologic Oncology Group 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 irradiation resulted in complete response rates of 53%-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%-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 180 cGy and excessive total doses are not used.[2,7-10] Doses of at least 54 Gy, but less than 65 Gy, should be used.

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. Obstetrics and Gynecology 68(6): 733-740, 1986.

  2. Thomas GM, Dembo AJ, Bryson SC, et al.: Changing concepts in the management of vulvar cancer. Gynecologic Oncology 42(1): 9-21, 1991.

  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. American Journal of Clinical Oncology 10(2): 171-181, 1987.

  4. Anderson JM, Cassady JR, Shimm DS, et al.: Vulvar carcinoma. International Journal of Radiation Oncology, Biology, Physics 32(5): 1351-1357, 1995.

  5. Slevin NJ, Pointon RC: Radical radiotherapy for carcinoma of the vulva. British Journal of Radiology 62(734): 145-147, 1989.

  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): 3707-3716, 1993.

  7. Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecologic Oncology 47(1): 14-20, 1992.

  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. Gynecologic Oncology 42(3): 197-201, 1991.

  9. Koh WJ, Wallace HJ, Greer BE, et al.: Combined radiotherapy and chemotherapy in the management of local-regionally advanced vulvar cancer. International Journal of Radiation Oncology, Biology, Physics 26(5): 809-816, 1993.

  10. Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecologic Oncology 34(3): 263-267, 1989.

  11. Keys H: Gynecologic Oncology Group randomized trials of combined technique therapy for vulvar cancer. Cancer 71(4, Suppl): 1691-1696, 1993.


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]

Treatment options:

Standard:

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]

4. There is no standard chemotherapy or other systemic treatment effective in patients with metastatic disease. Such patients should be considered for clinical trials. Refer to PDQ or to CancerNet (http://cancernet.nci.nih.gov) for information on clinical trials for patients with vulvar cancer.

Under clinical evaluation:
Clinical trials.

References:

  1. Piura B, Masotina A, Murdoch J, et al.: Recurrent squamous cell carcinoma of the vulva: a study of 73 cases. Gynecologic Oncology 48(2): 189-195, 1993.

  2. Hopkins MP, Reid GC, Morley GW: The surgical management of recurrent squamous cell carcinoma of the vulva. Obstetrics and Gynecology 75(6): 1001-1005, 1990.

  3. Miyazawa K, Nori D, Hilaris BS, et al.: Role of radiation therapy in the treatment of advanced vulvar carcinoma. Journal of Reproductive Medicine 28(8): 539-541, 1983.

  4. Russell AH, Mesic JB, Scudder SA, et al.: Synchronous radiation and cytotoxic chemotherapy for locally advanced or recurrent squamous cancer of the vulva. Gynecologic Oncology 47(1): 14-20, 1992.

  5. Thomas G, Dembo A, DePetrillo A, et al.: Concurrent radiation and chemotherapy in vulvar carcinoma. Gynecologic Oncology 34(3): 263-267, 1989.

  6. Podratz KC, Symmonds RE, Taylor WF, et al.: Carcinoma of the vulva: analysis of treatment and survival. Obstetrics and Gynecology 61(1): 63-74, 1983.

  7. Shimm DS, Fuller AF, Orlow EL, et al.: Prognostic variables in the treatment of squamous cell carcinoma of the vulva. Gynecologic Oncology 24(3): 343-358, 1986.

Date Last Modified: 02/1999



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