Dermatofibrosarcoma Protuberans: Experience with Management of Eighteen Cases

Abstract

Soft Tissue Sarcoma (STS) are a heterogeneous group of rare tumours that arise predominantly from the embryonic mesoderm. They account for about 0.7% of all adult malignancies but up to 15% of childhood malignancies1. In Nigeria it is said to constitute about 1.3% of solid malignancies2. Previous reports from this hospital showed that the commonest form of STS was fibrosarcoma3, 4. Dermatofibrosacoma protruberans (DFSP) is a variant of STS. DFSP is a cutaneous malignancy that arises from the dermis and invades deeper subcutaneous tissue (e.g. fat, fascia, muscle, bone). cellular origin of DFSP is not clear at this time. Evidence exists that supports the cellular origin being fibroblastic histiocytic or neuroectodermal. It is known for its locally aggressive growth and high rate of local recurrence. Our study investigates the age and sex distribution, clinical presentation and site at presentation in a Nigerian Teaching Hospital.