Dermatofibrosarcoma Protuberans of the Scalp and Lid - A Huge Challenge: Case Report

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a low grade locally invasive soft tissue sarcoma that rarely metastasizes; it mostly affects the  trunk and extremities, and less often the head and neck. The nonspecificity of early presentation in terms of symptoms and signs  makes it difficult to differentiate from other benign soft tissue tumours. The main modality of treatment is total surgical resection with or without adjuvant radiotherapy and oral medication-, imatinib mesylate. We report a case of aggressive DFSP involving the right part of  head and right upper eye lid in a 35year old house wife. She subsequently had excisional biopsy and Culter-Beard procedure to construct  the right upper eyelid. This study was done to create awareness, challenges and the role of Oculoplasty in the management of this rare  condition. Dermatofibrosarcoma protuberans is a rare and difficult tumour to treat. The role of the Oculoplastic Surgeon in managing  this tumor is underscored.