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59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

01. - 04.06.2008, Würzburg

Malignant peripheral nerve sheath tumors – considerations for a multimodal individually adapted treatment concept

Maligne periphere Nervenscheidentumoren – ein Ansatz für ein multimodales individuell zugeschnittenen Behandlungskonzept

Meeting Abstract

  • corresponding author C.P.G. Heinen - Neurochiurgische Abteilung der Universität Ulm am BKH zu Günzburg
  • U. M. Bäzner - Neurochiurgische Abteilung der Universität Ulm am BKH zu Günzburg
  • R. W. König - Neurochiurgische Abteilung der Universität Ulm am BKH zu Günzburg
  • T. Kretschmer - Neurochiurgische Abteilung der Universität Ulm am BKH zu Günzburg
  • G. Antoniadis - Neurochiurgische Abteilung der Universität Ulm am BKH zu Günzburg
  • J. Seidel - Neurochiurgische Abteilung der Universität Ulm am BKH zu Günzburg

Deutsche Gesellschaft für Neurochirurgie. Società Italiana di Neurochirurgia. 59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch). Würzburg, 01.-04.06.2008. Düsseldorf: German Medical Science GMS Publishing House; 2008. DocSO.01.09

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter: http://www.egms.de/de/meetings/dgnc2008/08dgnc009.shtml

Veröffentlicht: 30. Mai 2008

© 2008 Heinen et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielf&aauml;ltigt, verbreitet und &oauml;ffentlich zug&aauml;nglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

Objective: Malignant peripheral nerve sheath tumors (MPNST) represent 0,001% of all tumors, neurofibromatosis type 1 patients have an overall life-time risk of 10% to develop a MPNST. Despite their neuroectodermal origin, MPNST are listed under soft tissue sarcomas and treatment recommendations are based on this categorization. Similar histological types of MPNST seem to respond to treatment in different ways. Recent gene profiling data revealed that MPNST are a very diverse entity. In order to evaluate the effectivness of current and new treatment regimens, clinical data needs to be collected and correlated with the different genetic types. We present our experience with n=11 MPNST patients.

Methods: From 1993 until 2007 n=11 patient with MPNST were treated surgically. N=3 patients had an associated neurofibromatosis type 1. Median age was 49 years (ranging from 16 to 73 years).

Results: N=4 patients had an associated neurofibromatosis type 1. N=7 were located at the brachial plexus, n=1 each involved the median nerve, the sciatic n., the posterior femoral n., and the L3/4-spinal root level. N= 9 tumors could be resected completely at the first surgical session. N=3 were found to be WHO grade III, whereas n=8 presented with WHO grade IV. Treatment consisted of surgery alone in 6 patients, of surgery and radiation in 4 patients and of surgery,radiation and chemotherapy in 1 patient. N=8 patients died within 19 months on the average. The n=3 survivors had WHO grade III tumors, one of them did not receive radiochemotherapy. Comparing patients treated with surgery alone with those who had surgery and radiochemotherapy, we found a positive effect for radiation. N=8 patients died. The n=3 survivors had WHO grade III tumors. Among these, the median progression-free survival was 9 months and overall median survival was 19 months, being 14 months for the patients treated only with surgery, in contrast to 30 months for the patients with surgery plus radiation Comparing patients with surgery alone with those who had surgery plus radiation, we found a positive effect for radiation.

Conclusions: MPNST are a very aggressive and fast growing entity. Current literature and our own experience suggest a benefit from adjuvant therapy. Radical surgery remains the gold standard. However, there is no uniform treatment protocol and it is questionable whether MPNST should be treated as soft tissue sarcomas. Therefore, we propose the founding of a central registry in order to define each patient’s subtype of MPNST and thereby to facilitate the best multimodal therapy for the individual.