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Dermatofibrosarcoma protuberans of the vulva. A case report
J R Karlen1, K Johnson, S Kashkari
1Department of Obstetrics and Gynecology, Northeastern Ohio Universities College of Medicine, Rootstown, USA.
Background:
Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that is uncommon anywhere on the body but is especially uncommon on the vulva. This cancer rarely metastasizes but has a high incidence of local recurrence because of its tendency to grow in fingerlike projections away from the primary tumor.
Case:
A 36-year-old, black woman was the 10th and youngest patient with DFSP of the vulva to be reported. Treatment of the 5-cm, mobile, well-circumscribed lesion consisted of local excision. Several adjacent microscopic lesions showing DFSP were found in the pathologic specimen, however, so an even wider excision was performed. Twenty-seven months following removal, there was no evidence of recurrence.
Conclusion:
Survival rates for DFSP of the skin range from 91% to 100% in reported series, but local recurrence rates of 20-49% have also been noted. The reported deaths from the disease have resulted from extensive local spread due to inadequate excision and, only rarely, metastases. Because DFSP tends to spread to microscopic projections away from the visible lesions, very wide local excision is required for tumor control.