Cerebellar anaplastic astrocytoma in a teenager with Ollier Disease.

BACKGROUND

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.

METHODS

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.

CONCLUSIONS

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.