Myxopapillary ependymoma

Myxopapillary ependymoma is a slow-growing, low-grade (WHO grade I/II) tumor of the central nervous system that arises from ependymal cells, which line the ventricles of the brain and the central canal of the spinal cord. This tumor occurs almost exclusively in the conus medullaris, cauda equina, and filum terminale regions of the lower spinal cord. It is a subtype of ependymoma characterized by a distinctive histological pattern featuring mucin-rich (myxoid) papillary structures. Myxopapillary ependymoma predominantly affects young adults, with a slight male predominance. The most common symptoms include progressive lower back pain, which may be present for months or even years before diagnosis. As the tumor grows, it can compress nearby nerve roots, leading to leg pain (sciatica), numbness or weakness in the lower extremities, and bowel or bladder dysfunction. Some patients may also experience sensory changes or difficulty walking. Because symptoms develop gradually, diagnosis is often delayed. The primary treatment for myxopapillary ependymoma is surgical resection, with gross total removal being the goal, as complete excision is associated with favorable long-term outcomes and low recurrence rates. When complete surgical removal is not achievable, or in cases of recurrence or dissemination through the cerebrospinal fluid, adjuvant radiation therapy may be recommended. The overall prognosis is generally good, though local recurrence can occur, particularly after subtotal resection. Long-term follow-up with periodic MRI imaging is essential to monitor for recurrence. Chemotherapy has a limited role and is generally reserved for refractory or recurrent cases. Despite its typically benign behavior, rare cases of cerebrospinal fluid dissemination and even distant metastasis have been reported.

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