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Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report
David John Yeh1, Richard B Hessler, E Andrew Stevens
1Department of Neurosurgery, Medical College of Georgia, BI-3088, 1120 15th Street, Augusta, GA 30912, USA. dyeh@mail.mcg.edu
Objective And Importance:
Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a recently recognized, rare type of neoplasm that most commonly presents as a temporal seizure focus among male patients less than 30 years of age. This case represents the only reported suprasellar presentation, with the youngest reported age at diagnosis.
Clinical Presentation:
We present the case of a 12-year-old boy with new-onset diplopia who was diagnosed as having a large suprasellar mass, which was presumptively designated a craniopharyngioma on the basis of its clinical and radiological appearance.
Intervention:
Gross total resection of a well-encapsulated tumor with adjacent cysts was achieved, without postoperative neurological deficits. Frozen-section studies suggested pilocytic astrocytoma; however, the final histological diagnosis was composite PXA-GG.
Conclusion:
Composite PXA-GG, although extremely rare, may present as a pediatric suprasellar mass. The histopathological diagnosis is contingent on the demonstration of distinct coincident PXA and GG components. The prognosis after gross subtotal resection is likely favorable; however, long-term follow-up monitoring is indicated for these rare neoplasms.