Dermatofibrosarcoma Protuberans of the Lid with Orbital Invasion: The Challenges of Late Presentation

Abstract

Background: Dermatofibrosarcoma Protuberans (DFSP) is a very rare painless intermediate to low grade malignancy of the subcutaneous tissue or dermis commonly seen in the young to middle age group and affecting the trunk and proximal extremities. The aim of this report is to present the rare lid affectation of the tumour and the challenges involved in managing a locally invasive tumour in the face of late presentation and financial constraints. This is a case report of a 35-year-old woman who presented a 10 year history of Dermatofibrosarcoma Protuberans (DFSP) of the right lower lid with facial, maxillary, and orbital invasion of the right side. She underwent multiple surgeries, including orbital exenteration. There were limitations in accessing and following primary surgical procedures with radiation therapy and chemotherapy that could have impacted the overall outcome of this case. Managing a DFSP in the presence of late presentation, marked local invasiveness and financial constraints is challenging. A multidisciplinary approach involving social workers must be employed if a cure is to be achieved.