Primary Intracranial Extra-Axial Anaplastic Ependymomas.

Area of Science:

• Neuro-oncology
• Neurosurgery
• Pathology

Background:

• Intracranial extra-axial ependymomas (IEAEs) are rare, typically arising within the central nervous system.
• Only 17 cases of IEAEs have been previously reported in the literature.

Observation:

• This report details 2 cases involving 3 anaplastic IEAEs, initially misdiagnosed.
• Case 1: A 47-year-old male with a para-falcine lesion requiring gross total resection (GTR) after radiosurgery failure, followed by radiotherapy with no recurrence at 53 months.
• Case 2: A 30-year-old male with two IEAEs treated with staged surgeries (near total resection and GTR), radiotherapy, and no recurrence at 28 months.

Findings:

• IEAEs present diverse clinical and radiological features, making preoperative diagnosis challenging.
• Histopathological diagnosis confirmed anaplastic ependymoma in both reported cases.
• No recurrence was observed in patients who underwent GTR plus radiotherapy, or in previously reported cases with total resection.

Implications:

• Gross total resection combined with radiotherapy is crucial for favorable outcomes in IEAEs.
• Tailored, staged surgical resection followed by radiotherapy is recommended for patients with multiple IEAEs.
• Accurate preoperative diagnosis and timely treatment are essential for managing these rare tumors.