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

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.

journal_name

Acta Neurochir (Wien)

journal_title

Acta neurochirurgica

authors

Kobayashi H,Terasaka S,Yamaguchi S,Kubota K,Iwasaki Y

doi

10.1007/s00701-008-1601-2

subject

Has Abstract

pub_date

2008-08-01 00:00:00

pages

817-21

issue

8

eissn

0001-6268

issn

0942-0940

journal_volume

150

pub_type

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