Maffucci' s syndrome complicated by intracranial chondrosarcoma: two new illustrative cases

Arquivos de Neuro-Psiquiatria

Leandro Infantini Dini²  Gustavo Rassier Isolan²  Glauco Andre Saraiva²  Simone Afonso Dini¹  Pasquale Gallo¹ 

[1] ,Hospital Centenário Department of Neurosurgery

关键词: Maffucci;    s syndrome;    enchondromatosis;    hemangiomas;    Ollier;    s disease;    skull base;    chondrosarcoma;    síndrome de Maffucci;    encondromatose;    hemangiomas;    doença de Ollier;    base de crânio;    condrossarcoma;   

DOI  :  10.1590/S0004-282X2007000500016

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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