Ollier disease and Maffucci syndrome are rare syndromes in which there is deforming dysplasia of cartilage, primarily but not exclusively involving the metaphyses and diaphyses of long bones. In a minority of patients, dysplasia can lead to sarcomatous degeneration, producing chondrosarcomas. There also appears to be an association with other neoplasms. Little has been written about the association between Ollier disease and intracranial tumors, and these papers have largely consisted of case reports in adults. The authors present the case of a 6-year-old girl with left arm osseous changes consistent with Ollier disease and a biopsy-proven thalamic glioblastoma multiforme. They then examine the co-occurrence of brain tumors in conjunction with a dyschondroplasia syndrome in children and adolescents to assess the presentation, treatment offered, and disease course of similar cases.
Eight other such cases were identified, 6 in patients with Ollier disease (ranging in age from 7 to 18 years), and 2 with Maffucci syndrome (both in late adolescence). Including our own patient, 7 of the 9 cases of comorbid dyschondroplasia and intracranial malignancy occurred in girls. Some patients presented soon after the acute onset of symptoms, and others had a more subtle, protracted course over as many as 2 years. Some tumors were deemed resectable and others not. In only 1 instance was follow-up beyond 1 year reported.
Abbreviations used in this paper: CGE = cobalt Gy equivalent; GBM = glioblastoma multiforme.
HofmanSHeegMKleinJPKrikkeAP: Simultaneous occurrence of a supra- and an infratentorial glioma in a patient with Ollier's disease: more evidence for non-mesodermal tumor predisposition in multiple enchondromatosis. Skeletal Radiol27:688–6911998
HofmanS, HeegM, KleinJP, KrikkeAP: Simultaneous occurrence of a supra- and an infratentorial glioma in a patient with Ollier's disease: more evidence for non-mesodermal tumor predisposition in multiple enchondromatosis. 27:688–691, 1998)| false
NoëlGFeuvretLCalugaruVHadadiKBailletFMazeronJJ: Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome—three case reports and review of the literature. Acta Oncol43:705–7102004
NoëlG, FeuvretL, CalugaruV, HadadiK, BailletF, MazeronJJ, : Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome—three case reports and review of the literature. 43:705–710, 2004)| false