Maffucci' s syndrome complicated by intracranial chondrosarcoma: two new illustrative cases
Arq. neuropsiquiatr
;
65(3b): 816-821, set. 2007. ilus
Article
in English
| LILACS
| ID: lil-465186
ABSTRACT
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.
RESUMO
Síndrome de Maffucci é uma condição congênita rara, às vezes confundida com a doença de Ollier, caracterizada por encondromas múltiplos associados com hemangiomas e flebectasia. A concomitância com neoplasias primárias tem sido relatada esporadicamente. Nós relatamos dois casos de síndrome de Maffucci associada a condrossarcoma da base do crânio, enfatizando aspectos fisiopatológicos e o manejo desafiador dos condrossarcomas intracranianos. Em revisão da literatura, podemos encontrar o relato de apenas doze casos similares.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Chondrosarcoma
/
Skull Base Neoplasms
/
Enchondromatosis
Type of study:
Diagnostic study
Limits:
Adult
/
Humans
/
Male
Language:
English
Journal:
Arq. neuropsiquiatr
Journal subject:
Neurology
/
Psychiatry
Year:
2007
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Hospital Centenário/BR
/
Hospital Cristo Redentor/BR
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