Vulvar Cancer

3-5% of all female genital cancers and 1% of all malignancies in women. 2000-3000 new cases annually. The average age at diagnosis is approximately 65 years, but there is a trend to younger age at diagnosis.

Risk Factors

The cause of vulvar cancer appears to be multifactorial but some associated risk factors appear to be advanced age, low socioeconomic class, hypertension, diabetes, prior lower genital tract malignancy (cervical cancer), and immunosuppression. HPV association is not as strong as that of cervix cancer.

Pathology

Approximately 85% of vulvar malignancies are of the squamous cell variety. The second most common common histologic type is malignant melanoma representing 10% of cases of vulvar malignancies.

Routes of Spread

1. Direct extension
2. Lymphatic dissemination
3. Hematogenous dissemination

Clinical Features

Symptoms: Chronic pruritis, ulceration, or nodule are the most common complaints.

Physical Findings: Generally these lesions arise from the labia majora (40%), labia minora (20%), periclitoral area (10%), and perineum/posterior fourchette (15%). They may appear as a dominant mass, warty area, ulcerated area, or thickened white epithelium. It is estimated that only 5% of cases are multi-focal. A general rule of thumb is to biopsy the center of any suspicious area.

Pretreatment Workup

1. Physical exam with special attention to the groin nodes and measurement of primary lesion
2. Routine labs
3. CXR
4. Cystoscopy/Proctoscopy (depending on site and extent of lesion)

Staging

Since 1988 Vulvar cancer has been staged using a surgical system which utilizes the TNM classification with modifications added in 1995. 

Stage I and Stage IA:
T1N0M0- Tumor confined to the vulva and/or perineum 2cm or less in diameter with stromal invasion no greater than 1.0 mm, nodes are negative.

Stage IB:
T1N0M0- Tumor confined to the vulva and/or perineum 2cm or less in diameter with stromal invasion greater than 1.0 mm, nodes are negative.

Stage II:
T2N0M0- Tumor confined to the vulva and/or perineum >2cm in diameter, nodes are negative.

Stage III:
T3N0M0- Tumor of any size with Adjacent spread to the lower urethra or the anus
T1N1M0 2. Unilateral regional lymph node metastases
T2N1M0
T3N1M0

Stage IVA:
Tumor invades any of the following:
Upper urethra, bladder mucosa, rectal mucosa, pelvic bone or bilateral regional node mets
T1N2M0
T2N2M0
T3N2M0
T4 any N M0

Stage IV B:
Any distant mets including pelvic nodes.
Any T, any N, M1

Stage I- (<1mminvasion=microinvasive) wide local excision.  All other Stage I lesions require a radical wide local excision with a traditional 2cm gross margin and superficial dissection of the corresponding groin. If the lesion is close to the midline, bilateral superficial groins are dissected.

Stage II- Radical vulvectomy with bilateral node dissection including superficial and deep inguinal nodes.  Radiotherapy may be indicated if high risk pathologic features are found ..

Stage III- Several treatment options exist and must be individualized in each patient:
1. Modified radical vulvectomy (Radical wide local excision is used in some institutions) with inguinal and femoral node dissection. Pelvic and groin radiation with positive groin nodes.
2. Preoperative radiotherapy (+/-radiosensitizer) may be used to increase operability and decrease the extent of resection followed by radical excision with bilateral superficial and deep groin node dissection.
3. Radiotherapy alone if the patient or extent of lesion deemed unsuitable for radical surgery.

 Stage IV- Treatment options include the following and must be individualized:
1. Radical vulvectomy and pelvic exenteration
2. Radical vulvectomy followed by radiotherapy
3. Preoperative radiotherapy (+/- radiosensitizer) followed by radical surgical excision.
4. In cases of patient or lesion inoperability primary radiotherapy may be used (+/- radiosensitizer)

Recurrent Disease- Treatment depends on the site and extent of recurrence. For local recurrences a radical wide excision may be used with radiotherapy (dependent upon prior treatment). In selected cases, pelvic exenteration may be considered. Patients with regional or distant metastasis are more difficult to manage and often palliative therapy is the only option.

Prognostic Factors

1. Stage (including lesion size)
2. Inguinal node metastases (this is the single most important prognostic variable)
3. Lymph-Vascular Space Invasion
4. Other important factors include site of lesion, histologic grade, and depth of invasion.

Survival

Stage I: 95%
Stage II: 75-85%
Stage III: 55%
Stage IV: A-20%, B-5%

Recommended Follow-Up

Physical examination every 3 months for 2 years, every 6 months for years 3, 4 and 5, and annually thereafter.