Hand Involvement in Ollier Disease and Maffucci Syndrome: a Case Series


Autoria(s): Casal, D; Mavioso, C; Mendes, MM; Mouzinho, MM
Data(s)

15/04/2011

15/04/2011

2010

Resumo

Ollier Disease and Maffucci Syndrome are two rare diseases that can cause tumors in several organs, having a special predilection for the hand. However, there have been very few reports in the literature focusing on hand manifestations of these diseases. We report the cases of three female patients: one with Ollier Disease, and two other with Maffucci Syndrome. All patients had hand involvement as their initial primary complaint. The Ollier Disease patient developed chondrosarcomas of two digits and had to have these fingers amputated. One of the Maffucci patients died one year after presentation from a brain glioblastoma. These cases emphasize the importance of early diagnosis of Ollier Disease and Maffucci Syndrome, as these two conditions are associated not only to crippling hand deformity, but also to a significant risk of chondrosarcoma, and other malignant tumors.

Identificador

Acta Reumatol Port 2010: 35 (3): 375-378

http://hdl.handle.net/10400.17/143

Idioma(s)

eng

Publicador

Sociedade Portuguesa de Reumatologia

Direitos

openAccess

Palavras-Chave #HSJ CPR #Enchondromatosis/diagnostic imaging #Enchondromatosis/pathology #Hand #Radiography
Tipo

article