Supra-sellar clear cell ependymoma in a 2-year-old female: A case report

Clear cell ependymomas (CCEs) are rare glial tumors, with suprasellar localization being extremely uncommon in pediatric populations. Their nonspecific presentation often overlaps with more common tumors such as optic pathway gliomas and craniopharyngiomas, making early and accurate diagnosis particularly challenging.

A 2-year-old female presented with progressive visual disturbances and developmental delay. MRI revealed a T2-hyperintense, contrast-enhancing lobulated sellar and suprasellar mass. The initial radiologic impression favored optic pathway glioma. A transcranial approach enabled maximal safe resection. Histopathological analysis confirmed clear cell ependymoma, showing clear cytoplasm, perivascular pseudorosettes, and GFAP positivity. Critical structures including the optic nerves, pituitary stalk, and hypothalamus were preserved.

Suprasellar CCEs are exceptionally rare in children and can mimic more prevalent pediatric brain tumors radiologically. Histopathologic examination remains essential for definitive diagnosis. The lesion's location necessitates careful surgical planning to minimize morbidity. Due to the potential for recurrence, adjuvant therapy and vigilant long-term follow-up are recommended.

This case highlights the importance of including rare tumors like CCE in the differential diagnosis of pediatric suprasellar masses. A multidisciplinary approach involving imaging, histopathology, surgery, and adjuvant therapy is essential for accurate diagnosis and optimal patient outcomes.