Primary Ewing's sarcoma: peripheral primitive neuroectodermal tumour of the jugular foramen

Acta Neurochir (Wien). 2008 Aug;150(8):817-21. doi: 10.1007/s00701-008-1601-2. Epub 2008 Jun 12.

Abstract

We describe an extremely rare example of Ewing's sarcoma (ES)/peripheral primitive neuroectodermal tumour (pPNET) originating from the jugular foramen. The patient was a 10-year-old boy who presented with progressive symptoms due to right lower cranial nerve palsies. Computed tomographic (CT) scan and magnetic resonance imaging (MRI) revealed a tumour at the right jugular foramen which showed extra-cranial extension. Open biopsy of the extra-cranial lesion was performed, and diagnosis of ES/pPNET was made by histopathological, immunohistochemical and genetic investigations. The patient received a combination of multi-drug chemotherapy and irradiation. By 12 months after the diagnosis, MRI showed complete remission of the lesion, and the patient has been well apart from slight dysphagia. Previously, there was only one report of a jugular foramen ES/pPNET and in which treatment had failed. To our best knowledge, this is the first patient treated successfully with chemoradiotherapy.

Publication types

  • Case Reports

MeSH terms

  • 12E7 Antigen
  • Antigens, CD / analysis
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Biomarkers, Tumor / analysis
  • Biopsy
  • Cell Adhesion Molecules / analysis
  • Child
  • Combined Modality Therapy
  • Cyclophosphamide / administration & dosage
  • Dactinomycin / administration & dosage
  • Diagnosis, Differential
  • Humans
  • Image Processing, Computer-Assisted
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Invasiveness
  • Neuroectodermal Tumors, Primitive / diagnosis*
  • Neuroectodermal Tumors, Primitive / drug therapy
  • Neuroectodermal Tumors, Primitive / pathology
  • Neuroectodermal Tumors, Primitive / radiotherapy
  • Phosphopyruvate Hydratase / analysis
  • Sarcoma, Ewing / diagnosis*
  • Sarcoma, Ewing / drug therapy
  • Sarcoma, Ewing / pathology
  • Sarcoma, Ewing / radiotherapy
  • Skull Base Neoplasms / diagnosis*
  • Skull Base Neoplasms / drug therapy
  • Skull Base Neoplasms / pathology
  • Skull Base Neoplasms / radiotherapy
  • Synaptophysin / analysis
  • Tomography, X-Ray Computed
  • Vincristine / administration & dosage

Substances

  • 12E7 Antigen
  • Antigens, CD
  • Biomarkers, Tumor
  • CD99 protein, human
  • Cell Adhesion Molecules
  • Synaptophysin
  • Dactinomycin
  • Vincristine
  • Cyclophosphamide
  • Phosphopyruvate Hydratase

Supplementary concepts

  • VAC protocol