myxopapillary

Myxopapillary describes a histopathological pattern characterized by abundant myxoid (mucoid) stroma with delicate vascular cores arranged in a papillary or tufted architecture, typically formed by cuboidal to elongated ependymal-like cells. This pattern is most commonly encountered in myxopapillary ependymoma, a variant of ependymoma.

Myxopapillary ependymoma (MPE) is the prototypical tumor associated with the term and is classified by the World Health Organization as a grade I ependymal neoplasm. It arises most often in the filum terminale of the spinal cord, cauda equina, and less commonly in other intradural sites. It tends to present in adults, often in the third to fifth decades.

Clinical presentation typically includes progressive low back pain, radicular leg symptoms, and signs of cauda equina syndrome such as leg weakness, sensory loss, or sphincter disturbance.

Diagnosis combines imaging and histology. MRI shows a well-circumscribed intradural extramedullary mass, typically in the lumbosacral region, with variable signal intensity and enhancement after gadolinium. Histology confirms a myxoid matrix with papillary architecture and ependymal-type cells; immunohistochemistry often shows GFAP positivity and characteristic EMA perinuclear dot staining; mitotic activity is typically low.

Treatment and prognosis focus on surgical management. Gross total resection is the primary treatment; radiotherapy is considered for residual, recurrent, or disseminated disease. Prognosis is generally favorable after complete resection, with recurrence possible, especially after subtotal resections. Long-term follow-up is advised due to potential late recurrences.