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

• Autor(es): Casal, D ; Mavioso, C ; Mendes, MM ; Mouzinho, MM
• Data: 2010
• Identificador Persistente: http://hdl.handle.net/10400.17/143
• Origem: Repositório do Centro Hospitalar de Lisboa Central, EPE
• Assunto(s): Doença de Ollier; Adulto; Condrossarcoma; Mão; Síndrome de Maffuci
• Tipo de Documento: Artigo
• Idioma: Inglês

Descrição

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.