Intramedullary PNET of the spine: long-term survival after combined modality therapy and subsequent relapse

Primitive neuroectodermal tumors (PNET) originating within the spinal cord are extraordinarily rare. We report a female who presented at age 21 with diffuse involvement of the lower spinal cord. After biopsy and successful treatment with radiation and chemotherapy, she recurred 10 years later with disease in her cerebellum. She was reinduced with chemotherapy and subsequently received high-dose chemotherapy with autologous stem cell support. She is alive and free of disease 11 years after her initial presentation. This represents the longest survival ever documented for a primary spinal PNET.