Ollier's disease and Maffucci's syndrome: distinct entities or a continuum. Case report: enchondromatosis complicated by an intracranial glioma.
A patient with the diagnosis of enchondromatosis (Ollier's disease) presented with seizures. CT examination showed an avascular right frontal lobe lesion. Surgery revealed a histologically verified grade II astrocytoma. This case is consistent with recent evidence that questions the distinction between the two forms of enchondromatosis (Ollier's disease and Maffucci's syndrome). Maffucci's syndrome is associated with a high rate of malignancy, including intracranial gliomas. Ollier's disease has not been associated with malignant change, other than sarcomatous transformation of the enchondromas. However, Ollier's disease and Maffucci's syndrome may be a spectrum of the same disease process, and therefore patients with diagnosed Ollier's disease are at a higher risk for malignancy than has been previously recognized.
Mellon
C D
CD
Department of Medicine (Neurology), University of Texas Health Science Center, San Antonio 78284.
Carter
J E
JE
Owen
D B
DB
D000328:Adult; D001254:Astrocytoma / Q000209:etiology* / Q000601:surgery; D001932:Brain Neoplasms / Q000209:etiology* / Q000601:surgery; D003937:Diagnosis, Differential; D004687:Enchondromatosis / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D008297:Male; D010009:Osteochondrodysplasias / Q000175:diagnosis*