Accounts for 5% of all female genital cancers and 1% of all malignancies in women.
2500 cases per year and 500 deaths per year in the U.S.
Any vulvar lesion discovered by a physical examination should be biopsied to rule out a neoplasm.
Most vulvar neoplasia are diagnosed in early stages.
Histology include melanoma, extramammary Paget’s, disease, Bartholin gland adenocarcinoma, verrucous carcinoma, basal cell carcinoma, with most cases being squamous cell carcinoma.
Approximately 39% of patients with vulvar cancer are diagnosed with advanced stages III or IV.
87% of lesions are squamous cancers and 6% are malignant melanoma.
Incidence of squamous cell vulvar cancer 2.5 per 100,000 women.
Risk factors include: increasing age, infection with HPV, cigarette smoking, inflammatory conditions affecting the vulva and immunodeficiency.
Standard treatment for early stage squamous cell carcinoma is wide local excision and unilateral or bilateral inguinofemoral lymphadenectomy.
In locally advanced vulvar cancers, preoperative chemoradiation is delivered.
The most common agents used for preoperative chemotherapy/radiation include cisplatinum, fluorouracil, and Mitomycin C.
Well significant prognostic factor for vulvar squamous cell carcinoma is lymph node status.
Factors associated with poor outcomes besides lymph nodes status include age, tumor diameter, tumor depth, tumor class, histologic grade, lymphovascular space invasion, margin distance, and clitoral involvement.
Half a women who undergo surgical treatment for vulvar cancer have wound complications.
Lymphedema and cellulitis following lymphadenectomy, can be lifelong issues.
Sentinel lymph node biopsy is a reasonable alternative to inguinal femoral lymphadenectomy in selected patients.
Studies have demonstrated a superiority of groin and pelvic radiotherapy compared to pelvic lymphadenectomy in patients undergoing radical pelvic vulvectomy with groin metastases.
Locally advanced vulvar tumors that are unresectable without removing the proximal urethra/bladder/anus should undergo external beam radiation with concurrent chemotherapy: inguinal femoral lymphadenectomy may be required, if abnormal radiographic nodal studies are present.
Adjuvant chemoradiation therapy is recommended for patients with nodal disease.
Most recurrences of vulvar cancer occur within the first one to two years.
Among patients with recurrence about 37% develop recurrent vulvar squamous cell carcinoma.
Over half of recurrences are vulvar followed by inguinal at 18.7%, multisite at 14.2%, distant at 7.9% and pelvic at 5.7%.
Survival at five years is 60% for vulvar recurrences, 27% for inguinal/pelvic and 15% distant sites and 14% from multiple sites.
No clear standard of care exists for recurrent vulvar carcinoma with treatment and outcome, depending on the site and extent of recurrent disease.
Treatment options include surgery, radiation, chemotherapy, and checkpoint inhibitors.