Pediatric thalamic glioblastoma associated with Ollier disease (multiple enchondromatosis): a rare case of concurrence

Case report

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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.

Article Information

Address correspondence to: Adrianna Ranger, M.D., Children's Hospital, London Health Sciences Center, 800 Commissioners Road East, Room E6-315, London, Ontario, Canada N6A 5W9. email: aranger@uwo.ca.

© AANS, except where prohibited by US copyright law.

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Figures

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    Left: Enchondromatous change involving the distal end of the left radius in a 6-year-old girl with Ollier disease. Right: Enchondromatous change involving the proximal end of the left humerus in the same patient.

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    Sagittal T1-weighted MR imaging study obtained with Gd contrast in a 6-year-old girl with Ollier disease, revealing a large, heterogeneous tumor arising from the left thalamus and extending into the upper brainstem.

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    Histological slides prepared from biopsy specimens obtained in the present case. A: Tumor cells were randomly arranged on a fibrillary background. Nuclear pleomorphism and mitotic figures were present. B: Endothelial hyperplasia and glomeruloid vascular tufts were noted. C: The tumor cells expressed glial fibrillary acidic protein. D: The DNA polymerase activity was high, at 10–15% in selected fields. H & E (A and B), anti–glial fibrillary acidic protein immunoperoxidase (C), anti–Ki 67 immunoperoxidase (D). Bar = 50 μm.

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