Background:
Pleomorphic xanthoastrocytoma (PXA) is a rare form of astrocytic neoplasm most commonly found in children and young adults. This neoplasm, which is classified as a Grade II tumor by the World Health Organization classification of tumors of the central nervous system, carries a relatively favorable outcome. It is usually found supratentorially in cortical regions of the cerebral hemispheres, and as such, presenting symptoms are similar to other supratentorial cortical neoplasms; with seizures being a common initial symptom. Due to the rarity of this type of neoplasm, PXA arising elsewhere in the brain is often not included in the initial differential diagnosis.
Case description:
This report presents an extremely rare patient with PXA arising in the suprasellar region who presented with progressive peripheral vision loss. Magnetic resonance imaging of the brain demonstrated a heterogeneous suprasellar mass with cystic and enhancing components initially; the most likely differential diagnosis was craniopharyngioma. The patient underwent endoscopic endonasal resection of the tumor. Microscopically, the tumor was consistent with a glial neoplasm with variable morphology. Based on these findings along with further immunohistochemical workup, the patient was diagnosed with a PXA arising in the suprasellar region. At the 1-year follow-up, the patient remained free of recurrence. Although rare PXA originating in other uncommon locations, such as the spinal cord, cerebellum, the ventricular system, and the pineal region have been previously described.
Conclusion:
Although rare, PXA should be included in the differential diagnosis for solid-cystic tumors arising in the suprasellar region in young adults.