A rare case of cauda equina paraganglioma histologically simulating ependymoma

Background: Paragangliomas of the cauda equina are rare neuroendocrine tumors that can mimic ependymomas on imaging and histology, creating diagnostic challenges and influencing surgical planning. Case Description: A 41-year-old man presented with progressive low back pain and bilateral leg numbness. MRI showed a well-circumscribed, enhancing intradural extramedullary mass at L3. He underwent L3–L4 laminectomy with gross-total microsurgical excision. Histopathology revealed pseudorosettes and uniform tumor cells, initially suggesting ependymoma; however, immunohistochemistry demonstrated strong synaptophysin and S100 positivity with negative GFAP and EMA, confirming a cauda equina paraganglioma. Postoperatively, the patient recovered fully and remained asymptomatic at 1-year follow-up. Conclusion: Paragangliomas can closely resemble ependymomas, and accurate diagnosis requires immunohistochemistry. Complete surgical excision yields excellent outcomes, and individualized long-term imaging follow-up is recommended because recurrence, although uncommon, can occur.