Vulvar sarcoma: clinical implications

Abstract

This is a review of the histories of 47 patients with vulvar sarcoma, consisting of leiomyosarcoma (25), malignant fibrous histiocytoma (5), epithelioid sarcoma (8), dermatofibrosarcoma protuberans (9), including 7 from our own institute. When compared to the biological behaviour of sarcomas from other anatomic sites of the body, no essential differences were found. The prognosis after the appearance of regional or distant recurrence was poor and prevention of local recurrence by wide excision was the best way to improve the prognosis of leiomyosarcoma and dermatofibrosarcoma protuberans. However, the poor prognosis of epitheloid sarcoma did not change. Elective treatment of regional lymph nodes was not indicated in the four tumor types discussed and dissection of metastatic inguinal nodes was rarely beneficial. However, distressing local problems were prevented in a patient with epithelioid sarcoma and lasting benefit was seen in a patient with malignant fibrous histiocytoma who developed an inguinal metastasis after a 3-year disease-free interval. The beneficial effect of resection of pulmonary metastasis needs more attention.