Tanycytic ependymoma is a rare subtype of ependymoma. Reports of this tumor in neurofibromatosis type 2 (NF-2) are rare. A 16-year-old girl presented with gait disturbance and a palpable neck mass, which had been present for 2 years. MRI revealed an intramedullary lesion within the upper cervical spinal cord, which was removed surgically. Pathological investigation revealed an uncommon form of tanycytic ependymoma associated with NF-2. This rare morphology of tanycytic ependymoma could be misinterpreted as pilocytic astrocytoma or other tumor types that exhibit elongated cells. Increased awareness of this transitional form of intramedullary ependymoma among neurosurgeons and pathologists might avoid incorrect surgical approaches and postoperative treatments.
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