Pediatric Case of Xanthogranuloma in the Sellar Region Presenting a Visual Disturbance Successfully Treated with Endoscopic Endonasal Surgery.

Abstract

Xanthomatous pituitary diseases rarely occur in childhood. We report a rare pediatric case of a xanthogranuloma that developed in the sellar region, resulting in a visual disturbance that was treated successfully with endoscopic endonasal surgery. A 13-year-old boy came to us with a headache and visual disturbance that occurred 1 month prior. Clinical examination findings showed that he was alert with signs of bitemporal hemianopsia. An endocrinologic examination showed partial hypopituitarism, and brain magnetic resonance imaging revealed a cystic mass in the sellar turcica compressing the optic apparatus. Endoscopic endonasal surgery was performed to decompress the optic apparatus, and the mass was removed. Histopathologic analysis of the tumor demonstrated granulomatous tissue with cholesterol clefts, foamy macrophages, and multinucleated giant cells, with no epithelial component. The diagnosis was xanthogranuloma of the sellar region. The patient gradually recovered from the visual disturbance and was free from any neurologic signs or symptoms 6 months after surgery. Xanthogranuloma, although rare, should be considered as a differential diagnosis of a sellar or suprasellar lesion, even in children.