Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report.
OBJECTIVE
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.


METHODS
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.


METHODS
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.


CONCLUSIONS
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.
Yeh
David John
DJ
Department of Neurosurgery, Medical College of Georgia, BI-3088, 1120 15th Street, Augusta, GA 30912, USA. dyeh@mail.mcg.edu
Hessler
Richard B
RB
Stevens
E Andrew
EA
Lee
Mark R
MR
D001254:Astrocytoma / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D001932:Brain Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D002648:Child; D003397:Craniopharyngioma / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D018303:Ganglioglioma / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed