MAFFUCCIS-SYNDROME ASSOCIATED WITH A CRANIAL BASE CHONDROSARCOMA - CASE-REPORT AND LITERATURE-REVIEW

OBJECTIVE AND IMPORTANCE: Our objective was to study the diagnosis and management of this rare condition. A review of the literature concern ing 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 li terature, The differential diagnosis between Oilier's disease and Maff ucci's syndrome and the causes of these conditions are not clear. INTE RVENTION: An 18-year-old female patient presented with a giant tumor i nvolving the posterior fossa, clivus, middle fossa, and cavernous sinu s. The lesion could be totally removed through a transzygomatic approa ch. The histological diagnosis was chondrosarcoma. It was confirmed by immunohistochemical studies. There were no postoperative complication s. CONCLUSION: Maffucci's syndrome is a rare clinical condition that p resents difficulties concerning its diagnosis and management. It is ch aracterized by the presence of multiple enchondromas and cutaneous hem angiomas. Intracranial chondrosarcomas may be associated with this syn drome. Immunohistochemical studies are necessary to differentiate chon drosarcomas from chordomas. The treatment of choice for cranial base c hondrosarcomas is total removal of the lesion. Total removal may be ve ry difficult to achieve because of the involvement of neurovascular st ructures. Alternative therapies, such as proton beam radiosurgery, sho uld be considered. In this case, radical removal of the tumor was poss ible using a transzygomatic approach. Cross total removal of large cra nial base chondrosarcomas is possible, but a longer follow-up period i s necessary to ascertain that radical resection was achieved.