A Rare Documented Case of Advanced Metastatic Dermatofibrosarcoma Protuberans (DFSP) with Extensive Intrathoracic Dissemination

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous soft-tissue sarcoma characterized by low metastatic potential. However, rare fibrosarcomatous transformation (FS-DFSP) significantly increases the risk of systemic spread; although metastases—particularly pulmonary ones—are infrequent compared to other skin tumors, they have been sporadically described in the medical literature. Objective: This report documents a rare clinical case of extensive intrathoracic dissemination secondary to advanced metastatic progression in an elderly patient with a long-standing history of recurrent DFSP managed in a resource-constrained setting and the first documented primary pulmonary metastasis in the Gambia. Methods: We conducted a retrospective clinical analysis of an 81-year-old male presenting with severe, progressive dyspnea following a 14-year history of recurrent, histologically confirmed DFSP across the limbs and torso. The evaluation involved a comprehensive clinical examination, review of historical dermatological records, and detailed thoracic imaging to establish the extent of visceral involvement. Results: Clinical and radiological evaluations revealed massive intrathoracic tumor burden consistent with pulmonary and pleural metastases originating from the transformed cutaneous sarcoma. Despite the indolent initial presentation of DFSP, the development of late-stage visceral dissemination underscores the aggressive biological potential of the tumor over extended timelines. Managing this complex presentation was further compounded by limited local diagnostic and therapeutic resources. Conclusion: This case highlights the critical necessity for long-term clinical vigilance regarding indolent dermatological conditions, outlines the diagnostic challenges in linking chronic skin lesions to visceral metastasis, and emphasizes the urgent need for improved diagnostic pathways, heightened surveillance, and multidisciplinary care models to effectively manage complex soft-tissue sarcomas in resource-limited environments.