A myxoid chondrosarcoma associated with an anti-Hu-positive paraneoplastic encephalomyelitis.

Extraskeletal myxoid chondrosarcoma is a rare tumor with less than 100 cases reported in the literature. The prevalence of anti-Hu positive myxoid chondrosarcoma-associated paraneoplastic subacute cerebellar degeneration is exceedingly rare. We present a report of a patient with confirmed myxoid chondrosarcoma-associated paraneoplastic subacute cerebellar degeneration, who exhibited marked improvement within 1 week of receiving chemotherapy, intravenous immunoglobulin (IVIG), and hydrocortisone treatment.