Maffucci's syndrome complicated by intracranial chondrosarcoma: two new illustrative cases.
Maffucci's syndrome is a rare congenital condition, sometimes misdiagnosed as Ollier's disease, characterized by multiple enchondromas combined with hemangiomas and phlebectasia. Coexisting primary malignancies have been described sporadically. We report two cases of Maffucci's syndrome associated with cranial base chondrosarcoma, emphasizing pathophysiological features and the challenging management of intracranial chondrosarcomas. To the best of our knowledge, only twelve similar cases have been reported in the literature.
Dini
Leandro Infantini
LI
Department of Neurosurgery, Hospital Centenário, Avenida Theodomiro Porto da Fonseca 799, 93020-080 São Leopoldo, RS, Brazil. lenadrodini@ig.com.br
Isolan
Gustavo Rassier
GR
Saraiva
Glauco Andre
GA
Dini
Simone Afonso
SA
Gallo
Pasquale
P
D000328:Adult; D002813:Chondrosarcoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D004687:Enchondromatosis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019292:Skull Base Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D014057:Tomography, X-Ray Computed