RESUMO
OBJECTIVE AND IMPORTANCE: Our objective was to study the diagnosis and management of this rare condition. A review of the literature concerning chondrosarcomas related to Maffucci's syndrome is reported. Cause and management are discussed. CLINICAL PRESENTATION: We report a case of Maffucci's syndrome associated with a cranial base chondrosarcoma. To our knowledge, only five similar cases have been reported in the literature. The differential diagnosis between Ollier's disease and Maffucci's syndrome and the causes of these conditions are not clear. INTERVENTION: An 18-year-old female patient presented with a giant tumor involving the posterior fossa, clivus, middle fossa, and cavernous sinus. The lesion could be totally removed through a transzygomatic approach. The histological diagnosis was chondrosarcoma. It was confirmed by immunohistochemical studies. There were no postoperative complications. CONCLUSION: Maffucci's syndrome is a rare clinical condition that presents difficulties concerning its diagnosis and management. It is characterized by the presence of multiple enchondromas and cutaneous hemangiomas. Intracranial chondrosarcomas may be associated with this syndrome. Immunohistochemical studies are necessary to differentiate chondrosarcomas from chordomas. The treatment of choice for cranial base chondrosarcomas is total removal of the lesion. Total removal may be very difficult to achieve because of the involvement of neurovascular structures. Alternative therapies, such as proton beam radiosurgery, should be considered. In this case, radical removal of the tumor was possible using a transzygomatic approach. Gross total removal of large cranial base chondrosarcomas is possible, but a longer follow-up period is necessary to ascertain that radical resection was achieved.