Dumbbell-shaped peripheral primitive neuroectodermal tumor of the spine--case report and review of the literature

J Neurooncol. 2009 Apr;92(2):211-7. doi: 10.1007/s11060-008-9744-9. Epub 2008 Dec 3.

Abstract

Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare. Here, we present a case study of a 29-year-old male with a dumbbell-shaped pPNET at the T9-10 spine level, including details of his examination, surgical procedures applied, histological and genetic findings, and his subsequent treatment. We discuss the clinical course, the pathology and treatment for this disease, the surgical approach to thoracic dumbbell tumors and we review the literature. To our knowledge, this is the first report of a case of a dumbbell-shaped intradural and spinal peripheral PNET.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Adult
  • Calmodulin-Binding Proteins
  • Chemotherapy, Adjuvant
  • Combined Modality Therapy
  • Gene Rearrangement
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Magnetic Resonance Imaging
  • Male
  • Neuroectodermal Tumors, Primitive, Peripheral / genetics
  • Neuroectodermal Tumors, Primitive, Peripheral / pathology*
  • Neuroectodermal Tumors, Primitive, Peripheral / surgery
  • Neurosurgical Procedures
  • RNA-Binding Protein EWS
  • RNA-Binding Proteins
  • Radiotherapy, Adjuvant
  • Spinal Neoplasms / genetics
  • Spinal Neoplasms / pathology*
  • Spinal Neoplasms / surgery
  • Thoracic Vertebrae

Substances

  • Calmodulin-Binding Proteins
  • EWSR1 protein, human
  • RNA-Binding Protein EWS
  • RNA-Binding Proteins