OA
Archives of Clinical Case Reports and Reviews
Case Report: The First Documented Case of Advanced Metastatic Dermatofi- brosarcoma Protuberans (DFSP) with Extensive Intrathoracic Dissemination and Mediastinal Shift in West Africa.
Authors: Asmell Ramos Cabrera, Hector O. Victoria Barzaga, Jennifer Shallop, Momodu Kabir Manneh, Syeda Qurat-ul-Ain Hasan, Al-Heri Bulus Mika Doctor
Publication Year: 2026
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous malignancy with a low propensity for distant metastasis. When systemic dissemination occurs, it primarily targets the lungs, often secondary to a fibrosarcomatous transformation (FS-DFSP). We report the case of an 81-year-old Gambian male presenting with severe progressive dyspnea. He had a 14-year history of recurrent, disseminated cutaneous tumors across his limbs and torso, histologically confirmed as DFSP. Advanced thoracic imaging revealed a massive, space-occupying left intrathoracic mass causing complete lung collapse and a severe rightward mediastinal shift, alongside contralateral pulmonary nodules. To our knowledge, this represents the first documented report of advanced metastatic DFSP with extensive intrathoracic dissemination in West Africa. This case highlights the profound diagnostic challenges associated with the slow-growing nature of the tumor and the clinical difficulty less experienced physicians face in linking indolent dermatological conditions to internal organ affection. Furthermore, it underscores the logistical hurdles of managing complex soft-tissue sarcomas in resource-limited environments where specialized diagnostic validation and targeted therapies are unavailable.