Huge supratentorial extraventricular anaplastic ependymoma presenting with massive calcification--case report

Area of Science:

• Neuro-oncology
• Pediatric Neurosurgery
• Neuroradiology

Background:

• Ependymomas are tumors arising from ependymal cells, typically found in the ventricles or spinal cord.
• Supratentorial extraventricular ependymomas are rare and can present with varied imaging characteristics.
• Massive intratumoral calcification is an unusual feature for ependymomas, complicating diagnosis.

Observation:

• A 15-year-old boy presented with headache and visual defect due to a large, calcified supratentorial mass.
• Imaging revealed a 7 cm extraventricular mass with significant calcification and perilesional edema.
• Histopathological and immunohistochemical analysis confirmed anaplastic ependymoma (WHO grade 3).

Findings:

• The tumor exhibited high cellularity, atypia, necrosis, mitotic activity, and perivascular pseudorosettes.
• Immunohistochemistry showed positivity for GFAP, S-100, vimentin, and EMA, with a high MIB-1 index (26.5%).
• Complete tumor resection was achieved without neurological deterioration.

Implications:

• This case underscores that ependymoma should be considered in the differential diagnosis of supratentorial extraventricular masses with extensive calcification.
• The unusual presentation emphasizes the need for comprehensive histopathological evaluation for accurate tumor classification.
• Understanding rare presentations of brain tumors aids in improving diagnostic accuracy and treatment strategies.