TY - JOUR
T1 - A case of Ollier's disease associated with two intracranial gliomas
AU - Rawlings, C. E.
AU - Bullard, D. E.
AU - Burger, P. C.
AU - Friedman, A. H.
PY - 1987
Y1 - 1987
N2 - Ollier's disease or multiple enchondromatosis is a deforming dysplastic disease of cartilage involving primarily the metaphyses and diaphyses of long bone. 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.
AB - Ollier's disease or multiple enchondromatosis is a deforming dysplastic disease of cartilage involving primarily the metaphyses and diaphyses of long bone. 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.
UR - http://www.scopus.com/inward/record.url?scp=0023195706&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0023195706&partnerID=8YFLogxK
U2 - 10.1227/00006123-198709000-00023
DO - 10.1227/00006123-198709000-00023
M3 - Review article
C2 - 3313094
AN - SCOPUS:0023195706
SN - 0148-396X
VL - 21
SP - 400
EP - 403
JO - Neurosurgery
JF - Neurosurgery
IS - 3
ER -