4.5 Article

Cerebellar anaplastic astrocytoma in a teenager with Ollier Disease

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JOURNAL OF NEURO-ONCOLOGY
卷 89, 期 1, 页码 59-62

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SPRINGER
DOI: 10.1007/s11060-008-9583-8

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Ollier; astrocytoma; screening; PTHR1; PTEN

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Introduction Ollier Disease is a sporadic skeletal disorder with a predisposition to oncogenesis. It is estimated at around 1/100,000. We are presenting a young patient with Ollier Disease and high-grade astrocytoma. Case report A 14-year-old, Caucasian male with Ollier Disease presented with a history of headaches, vomiting, blurred vision, and unsteady gait. Brain MRI with contrast showed a 41 x 55 mm mass in the posterior fossa with spotty enhancement, which pathology proved to be anaplastic astrocytoma. Conclusion Despite the universal acceptance that Ollier Disease carries a high risk of developing malignancy there is very little in the literature about systematic screening. We recommended a cost-effective screening regime for these patients.

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