Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico-radiological presentations.

Abstract

Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5cm, most commonly (n=6) occurred over chest wall and abdominal wall. History of previous local trauma or surgery was identified in the majority of cases (n=4). There was poor correlation between pre-imaging clinical diagnoses and pathological diagnoses. Local recurrence and tumour de-differentiation with sarcomatous changes occurred in the minority of cases (n=2). Acommon radiological 'claw' sign at the lesion/skin interface formed by elongated appendages of the tumour superficially was appreciated in most cases (n=6). A history of previous local trauma or surgery serves as a possible etiological factor for the development of DFSP. High clinical suspicion for the entity is essential in its detection and differentiation from simple wound complications and local recurrence of other benign lesions. The radiological 'claw' sign at the lesion/skin interface might serve as a tell-tale sign for cutaneous tumour involvement. A comprehensive analysis of imaging findings in conjunction with individual clinical presentations is the key to accurate diagnoses and proper management.