Endoscopic treatment and radiotherapy of a skull base chondrosarcoma in a patient with type II enchondromatosis

Abstract

Maffucci syndrome is a rare, congenital mesenchymal displasia characterized by multiple enchondromata and hemangiomata, both of which can undergo malignant transformation. Intracranial involvement is uncommon. There is no medical treatment for enchondromatosis. Surgery is indicated in case of complications (pathological fractures, severe functional defects, malignant transformation). Here, we describe a large chondrosarcoma involving nose, paranasal sinuses and clivus treated by endoscopic transnasal resection and radiotherapy, in a 25-year-old patient with Maffucci's syndrome.