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Ollier disease

Ollier disease is a rare disorder characterised by the development of enchondromatosis in long bones.

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.

Case reports


Pearce et al. present a 19-year-old man with Ollier disease who also developed three synchronous brain tumours. Craniotomy, biopsy and debulking was performed for one lesion followed by a period of observation, and 9 months later he underwent a second craniotomy and debulking for symptomatic progression. Histopathological examination revealed a diagnosis of multifocal diffuse glioma (World Health Organization grade II). This report highlights the increased incidence of primary brain tumours in patients with Ollier disease and identifies the importance of screening patients with Ollier disease for primary neoplasms 1).


Mitchell et al. describe an unusual case of oligodendroglial GC diagnosed in a 16-year-old boy with Ollier disease. This is the first case of GC reported in a child with Ollier disease 2).


Ranger et al. 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 3).

Pearce P, Robertson T, Ortiz-Gomez JD, Rajah T, Tollesson G. Multifocal supratentorial diffuse glioma in a young patient with Ollier disease. J Clin Neurosci. 2012 Mar;19(3):477-8. doi: 10.1016/j.jocn.2011.06.019. Epub 2012 Jan 16. PubMed PMID: 22249016.
Mitchell RA, Ye JM, Mandelstam S, Lo P. Gliomatosis cerebri in a patient with Ollier disease. J Clin Neurosci. 2011 Nov;18(11):1564-6. doi: 10.1016/j.jocn.2011.03.025. Epub 2011 Aug 24. PubMed PMID: 21868231.
Ranger A, Szymczak A, Hammond RR, Zelcer S. Pediatric thalamic glioblastoma associated with Ollier disease (multiple enchondromatosis): a rare case of concurrence. J Neurosurg Pediatr. 2009 Oct;4(4):363-7. doi: 10.3171/2009.5.PEDS08422. PubMed PMID: 19795968.
ollier_disease.txt · Last modified: 2018/07/05 16:36 by administrador