Successful Novel Treatment of a Paraspinal Primitive Neuroectodermal Tumor with Predominantly Glial Differentiation: A 3-Year Follow-Up After Surgery, Intensity-Modulated Radiation Therapy and Oral Temozolomide

Abstract

Background: Paraspinal masses are a relatively uncommon but diverse group of lesions that can be neoplastic or non-neoplastic. Peripheral primitive neuroectodermal tumors of the lumbar paraspinal region with diffuse and strong glial differentiation have never been reported before.

Case description: We report a primary paraspinal primitive neuroectodermal tumor with overwhelming glial differentiation in a 23-year-old female patient who presented with intractable right lower extremity pain. The patient underwent a 2-staged operation with gross total resection of the mass followed by intensity-modulated radiation therapy and oral temozolomide, a regimen employed for high-grade intracranial gliomas. Serial imaging revealed no evidence of recurrence after 3 years.

Conclusions: Although these lesions appear to be exceptionally rare, an approach similar to that of intracranial high-grade glial tumors was effective in our experience. Our patient had no evidence of recurrence at 3-year follow-up.

Keywords: Chemotherapy; High glial differentiation; Paraspinal mass; Paraspinal neoplasm; Paraspinal primitive neuroectodermal tumor; Radiation; Surgery.