2012 Volume 122 Issue 12 Pages 2891-2897
From January 2001 to July 2010, we studied 101 cases (67 males and 34 females; ages between 41 and 85 years; mean age, 69.9 years) who underwent treatment at the Division of Dermatology, National Cancer Center Hospital, Japan. Tumors were located on the vulva in 89 cases, perianal region in 6 cases, and axilla in 5 cases. Multiple tumors were present in the vulva/axilla in 1 case. Surgery was the first choice of treatment if no distal metastasis was detected during preoperative examination. A sentinel lymph node biopsy or lymph node biopsy was performed when the patient showed pathological dermal invasion histopathologically and subcutaneous nodules clinically. Patients with lymph node metastasis underwent regional lymph node dissection. Significant differences were observed in 5-year survival rates; 100% of the patients with 1 or 2 metastatic lymph nodes survived for 5 years, but none of the patients with more than 3 metastatic lymph nodes did. However, the results also revealed that lymph node dissection resulted in a poor prognosis in cases of bilateral inguinal lymph node metastases or those of multiple metastases in vulval extramammary Paget’s disease. Thus, reduction surgery should be considered to prevent damage to the patient’s quality of life. A FECOM (5-FU/Epirubicin/Carboplatin/Vincristine/Mitomycin C) regimen or treatment with taxanes is often employed in cases of unresectable lymph node metastasis or distal metastasis; however, resistance to these treatments is common, and prognosis is extremely poor.