A case of Ollier's disease associated with two intracranial gliomas.
Ollier's disease or multiple enchondromatosis is a deforming dysplastic disease of cartilage involving primarily the metaphyses and diaphyses of long bones. It is only rarely associated with sarcomatous degeneration of the enchondromas or other generalized neoplasms. A related disease, Maffucci's syndrome, is, however, associated with generalized tumors. We present the case of a 29-year-old, albino, black man with Ollier's disease who, as a child, underwent a number of orthopedic procedures for multiple limb deformities and fractures. At age 25, he developed hydrocephalus, progressive cranial nerve palsies, and a large enchondroma of the skull base. He subsequently underwent multiple shunt procedures and two suboccipital craniectomies. Eighteen months later, a brain computed tomographic (CT) scan revealed an intracerebellar mass, which was found to be an anaplastic astrocytoma. Two years later, he developed a right hemiparesis and sensory dysfunction with a diffuse supratentorial mass on CT scan. A stereotactic biopsy showed this to be a similar anaplastic astrocytoma. The literature concerning Ollier's disease is reviewed, and the intracerebral lesions associated with both Ollier's disease and Maffucci's syndrome are examined.
Rawlings
C E
CE
Department of Surgery, Duke University Medical Center, Durham, North Carolina.
Bullard
D E
DE
Burger
P C
PC
Friedman
A H
AH
D000328:Adult; D001254:Astrocytoma / Q000150:complications* / Q000175:diagnosis; D001932:Brain Neoplasms / Q000150:complications* / Q000175:diagnosis; D002528:Cerebellar Neoplasms / Q000150:complications / Q000175:diagnosis; D004687:Enchondromatosis / Q000150:complications*; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000175:diagnosis; D010009:Osteochondrodysplasias / Q000150:complications*