Dermatofibrosarcoma protuberans: clinicopathologic presentation in Nigerians.

Abstract

IntroductionDermatofibrosarcoma protuberance (DFSP) is in general a rare low grade malignant sarcoma and possesses a tendency for local recurrence. It has a site predilection for the trunk. Occurrence in the facial area is extremely rare. Ample knowledge of its clinical, histological and biologic characteristics is vital for accurate and prompt recognition.MethodsOver 13 years, clinicohistologic information of cases was retrieved. Histological and immunohistochemical re-evaluation were performed to re-confirm diagnosis. Data collected and analyzed with SPSS Statistics version 20 were presented as frequency tables, charts and proportions as appropriate.ResultsOf 191 soft tissue sarcomas, a total of 28 cases were diagnosed as DFSP (14.7%). Facial types occurred in 3 cases (1.6%). Tumour had age and site predilections for the 4th decade and trunk respectively. There was an equal gender distribution among cases. Most common clinical presentation was in form of painless protruding nodular mass. General histologic presentation revealed cellular lesions composed of spindle to oval neoplastic cells arranged in a storiform pattern. Mitotic figures were rare. All cases showed positive expressions to CD34.ConclusionFacial DFSP is rare among Nigerians. Its clinical appearance may mimic other common benign lesions of the head and neck region often resulting in misdiagnoses. A comprehensive knowledge of its clinical and histologic presentations and biologic behavior, combined with its identification with the aid of advanced histologic and radiographic techniques results in prompt confirmatory diagnosis. Appropriate treatment should include adequate surgical excision techniques combined with adjuvant radiotherapy or chemotherapy.