gms | German Medical Science

56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)
3èmes journées françaises de Neurochirurgie (SFNC)

Deutsche Gesellschaft für Neurochirurgie e. V.
Société Française de Neurochirurgie

07. bis 11.05.2005, Strasbourg

A primary intracranial neuroendocrine carcinoma in a 2½-year-old boy: an extremely rare tumor entity

Ein primär intrakranielles neuroendokrines Karzinom bei einem 2½ Jahre alten Jungen: Beschreibung einer seltenen Tumorlokalisation

Meeting Abstract

  • corresponding author M. Schütze - Abteilung für Neurochirurgie, Universität Rostock
  • S. Landgraf - Abteilung für Pädiatrische Hämatologie und Onkologie, Universität Rostock
  • G. Eggers - Abteilung für pädiatrische Hämatologie und Onkologie, Universität Rostock
  • I. Leuschner - Institut für Paidopathologie, Universität Kiel
  • H. Nizze - Institut für Pathologie, Universität Rostock

Deutsche Gesellschaft für Neurochirurgie. Société Française de Neurochirurgie. 56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3èmes journées françaises de Neurochirurgie (SFNC). Strasbourg, 07.-11.05.2005. Düsseldorf, Köln: German Medical Science; 2005. DocP133

The electronic version of this article is the complete one and can be found online at:

Published: May 4, 2005

© 2005 Schütze et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.




Neuroendocrine neoplasms arising outside the appendix are very rarely observed in the pediatric population. Some authors describe primary sites of this tumor type in the lung or the liver. A neuroendocrine tumor with its primary location in the cranium has not been described so far.


A 2½-year-old boy was admitted to our hospital with the clinical sign of swollen lymph nodes in the cervical region. Initially he was diagnosed as an infective disease and was subsequently treated with antibiotics. During this treatment he deteriorated and a cranial MRI was performed. This showed a large tumor of the skullbase with an intracerebral cystic portion in the left frontal lobe. Tumor staging of the chest and the abdomen revealed no other tumors. An endoscopic guided biopsy of the tumor demonstrated an undifferentiated neuroendocrine carcinoma. The boy was treated with 3 cycles of chemotherapy and already after the second one the tumor showed retractable regression.


Because of the largely destroyed skull base an operation of the remaining parts was performed and the skull base was reconstructed 16 weeks after first diagnosis. The post operative course was uncomplicated and a further chemotherapy was attached. Several MRI scans during the following month showed a complete tumor regression. A last MRI one year after first diagnosis showed a wide meningeal spread of the tumor and in approval with the parents further therapy was cancelled.


In the actual literature a neuroendocrine carcinoma with its primary site at the skull base and a large intracerebral part isn’t described so far. Regarding to the different types of chemotherapy for different tumors 6 we highly recommend that even in young patients with a skull base tumor with difficulties in histological classification a neuroendocrine tumor should be considered as a differential diagnosis in such type of lesion.