Vulvar cancer overview

# 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.