Vulvar Cancer

Published on 31/05/2015 by admin

Filed under Obstetrics & Gynecology

Last modified 22/04/2025

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Chapter 79 Vulvar Cancer

INTRODUCTION

Description: Squamous cell cancer of the vulva generally presents as an exophytic ulcer or hyperkeratotic plaque. It may arise as a solitary lesion or develop hidden within hypertrophic or other vulvar skin changes, making diagnosis difficult and often delayed.
Prevalence: Fewer than 5000 cases per year, 4% of gynecologic malignancies. (The incidence of vulvar cancer increased by approximately 20% between 1973 and 2000, likely related to increased exposure to human papillomavirus [HPV].)
Predominant Age: In situ 40 to 49, invasive 65 to 70.
Genetics: No genetic pattern.

ETIOLOGY AND PATHOGENESIS

Causes: Unknown. Thought to be associated with human papillomavirus.
Risk Factors: Infection with human papillomavirus (molecular analysis had detected human papillomavirus DNA in 40% of vulvar cancers), smoking, immunosuppression, lichen sclerosus.

CLINICAL CHARACTERISTICS

Signs and Symptoms

• Itching, irritation, cracking, or bleeding of the vulva, most common on the posterior two thirds of the labia majus
• Ulcerated exophytic lesion or hyperkeratotic plaque (late in disease)

DIAGNOSTIC APPROACH

Differential Diagnosis

• Hypertrophic vulvar dystrophy
• Lichen sclerosus

Associated Conditions: Hyperplastic vulvar dystrophy.

Workup and Evaluation

Laboratory: No evaluation indicated.
Imaging: No imaging indicated.
Special Tests: Biopsy of any suspicious lesion.
Diagnostic Procedures: History, physical examination, and vulvar biopsy.

Pathologic Findings

Histologic types include squamous cell (90%), melanoma (5%), basaloid, warty, verrucous, giant cell, spindle cell, acantholytic squamous cell (adenoid squamous), lymphoepithelioma-like, basal cell, and Merkel cell. Sarcoma accounts for approximately 2% of vulvar cancers. Metastatic tumors from other sources are rare, but they do occur.

MANAGEMENT AND THERAPY

Nonpharmacologic

General Measures: Early evaluation (generally by biopsy). (The majority of women have had symptoms for more than 6 months before a diagnosis is made.)
Specific Measures: Initial treatment consists of wide local excision (1-cm margins). Subsequent therapy, including node dissections and adjunctive therapy (radiation), is determined by the stage of disease, cell type, and surgical margins.
Diet: No specific dietary changes indicated.
Activity: No restriction, except as dictated by surgical therapy.
Patient Education: American College of Obstetricians and Gynecologists Patient Education Pamphlet AP088 (Disorders of the Vulva).

Drug(s) of Choice

None.

FOLLOW-UP

Patient Monitoring: Careful follow-up for recurrence or additional new lesions. Any patient who has had human papillomavirus–related dysplasia should avoid the vulvar use of topical steroids because this may increase the risk of recurrence.
Prevention/Avoidance: None.
Possible Complications: Distant spread and disease progression, secondary infection. Wound breakdown after surgical excision is common.
Expected Outcome: If tumor invasion is less than 1 mm, the risk of lymph node involvement is essentially 0 and high success rates may be expected. Five-year survival rates decline with advancing stage: 20% with deep node involvement. Overall 5-year survival is 70%.

MISCELLANEOUS

Pregnancy Considerations: No effect on pregnancy, although the presence of a pregnancy may affect surgical therapeutic options.
ICD-9-CM Codes: Specific to cell type and location.

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REFERENCES

Level II

Ansink AC, Sie-Go DM, van der Velden J, et al. Identification of sentinel lymph nodes in vulvar carcinoma patients with the aid of a patent blue V injection: a multicenter study. Cancer. 1999;86:652.

Maggino T, Landoni F, Sartori E, et al. Patterns of recurrence in patients with squamous cell carcinoma of the vulva. A multicenter CTF Study. Cancer. 2000;89:116.

Rhodes CA, Cummins C, Shafi MI. The management of squamous cell vulval cancer: a population based retrospective study of 411 cases. Br J Obstet Gynaecol. 1998;105:200.

Level III

Canavan TP, Cohen D. Vulvar cancer. Am Fam Physician. 2002;66:1269.

DiSaia PJ. Management of superficially invasive vulvar carcinoma. Clin Obstet Gynecol. 1985;28:196.

Frumovitz M, Bodurka DC. Neoplastic disease of the vulva. In: Katz VL, Lentz GM, Lobo RA, Gershenson DM, editors. Comprehensive Gynecology. 5th ed. Philadelphia: Mosby/Elsevier; 2007:790.

Hacker NF, Van der Velden J. Conservative management of early vulvar cancer. Cancer. 1993;71:1673.

Kurman RJ, Norris HJ, Wilkinson EJ Rosai J, editor. Atlas of tumor pathology: tumors of the cervix, vagina and vulva, Vol 4. Washington, DC: AFIP, 1992.

Tyring SK. Vulvar squamous cell carcinoma: guidelines for early diagnosis and treatment. Am J Obstet Gynecol. 2003;189:S17.

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