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Skull Base 1997; 7(1): 49-55
DOI: 10.1055/s-2008-1058625
DOI: 10.1055/s-2008-1058625
Case Reports
© Thieme Medical Publishers, Inc., 381 Park Avenue South, New York, NY 10016
Maffucci's Syndrome and Intracranial Chondrosarcoma
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Publikationsverlauf
Publikationsdatum:
03. März 2008 (online)
![](https://www.thieme-connect.de/media/sbs/199701/lookinside/thumbnails/10.1055-s-2008-1058625-1.jpg)
Abstract
Maffucci's syndrome is a rare, congenital mesenchymal dysplasia characterized by multiple enchondromata and hemangimata, both of which may undergo malignant degeneration. Intracranial involvement is uncommon. A literature review yielded only six cases of Maffucci's syndrome with intracranial chondrosarcoma and two cases of Ollier's disease (enchondromata alone) with intracranial chondrosarcoma. We report the seventh case of Maffucci's syndrome in a patient with a very large and extensive skull base chondrosarcoma requiring staged operations for removal.