Vulvar cancer overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Musadiq Ali M.B.B.S.[2] Monalisa Dmello, M.B,B.S., M.D. [3]

Vulvar cancer Microchapters

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Patient Info

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Vulvar cancer from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Overview

Vulvar cancer accounts for about 5% of cancers of the female genital system in the United States. The vulva is the area immediately external to the vagina, including the mons pubis, labia, clitoris, bartholin glands, and perineum. The labia majora are the most common site of vulvar carcinoma involvement and account for about 50% of cases. The labia minora account for 15% to 20% of vulvar carcinoma cases. The clitoris and Bartholin glands are less frequently involved. Lesions are multifocal in about 5% of cases. About 90% of vulvar carcinomas are squamous cell cancers. The vulvar intraepithelial neoplasias (VIN), may be precursors to invasive squamous cell cancers. Common risk factors in the development of vulvar cancer are human papilloma virus, human immunodeficiency virus, vulvar intraepithelial neoplasia, and lichen sclerosus. Vulvar cancer may be classified according to histology into 3 groups: vulvar carcinoma, vulvar sarcoma, and vulvar malignant melanoma. If left untreated, patients with vulvar cancer may progress to develop ulcer on vulva or wart-like patches on vulva, and chronic pruritus of the vulva. Common complications of vulvar cancer include fistula and metastasis. Prognosis is generally good, and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%. Common physical examination findings of vulvar cancer include vulvar ulcers, mass, and inguinal lymphadenopathy. Vulvar biopsy is the confirmatory test for the diagnosis of vulvar cancer or precancerous lesions.The optimal therapy for vulvar cancer depends on the stage at diagnosis.The feasibility of surgery depends on the stage of vulvar cancer at diagnosis.


Classification

Vulvar cancer may be classified according to histology into 3 groups vulvar carcinoma, vulvar sarcoma, and vulvar malignant melanoma.

Pathophysiology

Development of vulvar cancer is the result of multiple genetic mutations.

Causes

Vulvar cancer may be caused by either HPV infection, or vulvar dermatoses.

Differential Diagnosis

Vulvar cancer must be differentiated from other neoplastic vulvar lesions, non neoplastic vulvar lesions, and infectious disease lesions of vulva.

Epidemiology and Demographics

In 2012, the incidence of vulvar cancer was estimated to be 2.4 per 100,000 females in the United States. The incidence of vulvar cancer increases with age, the median age at diagnosis is 68 years. Vulvar cancer is more prevalent in the caucasian race.

Risk factors

Common risk factors in the development of vulvar cancer are human papilloma virus, human immunodeficiency virus, vulvar intraepithelial neoplasia, and lichen sclerosus.

Screening

Screening for vulvar cancer is not recommended.

Natural History, Complications and Prognosis

If left untreated, patients with vulvar cancer may progress to develop ulcer on vulva or wart-like patches on vulva, and chronic pruritus of the vulva. Common complications of vulvar cancer include fistula and metastasis. Prognosis is generally good and the 5 year survival rate of patients with vulvar cancer is approximately 71.2%.

Diagnosis

Staging

According to the FIGO cancer staging system, there are 4 stages of vulvar cancer.

History and Symptoms

Symptoms of vulvar cancer include vulvar ulcers, lumps, discoloration, and chronic pruritis of the vulva.

Physical Examination

Common physical examination findings of vulvar cancer include vulvar ulcers, mass, and inguinal lymphadenopathy.

Chest X-ray

Chest radiography (CXR) may be helpful in the diagnosis of pulmonary metastasis of vulvar cancer. The characteristic findings of pulmonary metastasis are peripheral, rounded nodules of variable size scattered throughout both lungs.

CT scan

A CT scan for vulvar cancer may be helpful in determination of tumor size, tumor shape, and to detect metastasis of vulvar cancer.

MRI

MRI is useful in accurately assessing the size of vulval lesion and assessing groin lymph node metastasis.

Other Diagnostic Studies

Vulvar biopsy is the confirmatory test for the diagnosis of vulvar cancer or precancerous lesions.

Treatment

Medical therapy

The optimal therapy for vulvar cancer depends on the stage at diagnosis.

Surgery

The feasibility of surgery depends on the stage of vulvar cancer at diagnosis.

Primary Prevention

Prevention of vulvar cancer includes a comprehensive approach involving awareness, screening, and preventative vaccinations.

References