Il tumore maligno della guaine dei nervi periferici in eta pediatrica. Un'esperienza del Gruppo Cooperativo Italiano Sarcomi della Parti Molli

Translated title of the contribution: Malignant peripheral nerve sheath tumor in childhood. An experience of the Italian Cooperative Group on Soft Tissue Sarcoma

G. Bisogno, G. Cecchetto, G. Sotti, S. Dallorso, A. Mancini, A. Donfrancesco, L. Cordero Di Montezemolo, N. Santoro, P. Tamaro, L. Masiero, M. Carli

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Abstract

The term Malignant Peripheral Nerve Sheath Tumor (MPNST) designs malignant neoplasms arising from cells which are part of the peripheral nerve. It is a rare tumor of childhood, strongly associated Neurofibromatosis type I. This report represents the experience on MPNST acquired from the Italian Cooperative Group on Soft Tissue Sarcoma. We analyzed 29 patients registered from January 1979 until December 1995 in two consecutive protocols designed as RMS79 and RMS88. Despite multidisciplinary treatment comprehensive of surgery, chemotherapy and radiotherapy, MPNST is a very aggressive neoplasm with unsatisfying progression free and overall survival (respectively 28% and 43.6%). Some tumor features (size, site, invasiveness and stage) as well as patient characteristics (sex and presence of neurofibromatosis) have shown a prognostic value. After multivariate analysis only tumor invasiveness maintains a statistical significance. Radical surgery at diagnosis is the mainstay in the treatment of MPNST. Good results have been obtained with an Ifosfamide containing regimen in tumors not resectable at diagnosis: 46% of patients have shown a reduction of tumour size with a consequently radical conservative surgery. The role of radiotherapy remains controversial. In order to improve our knowledge about these rare and aggressive tumors, specific protocols are needed, possibly throughout an international cooperation.

Original languageItalian
Pages (from-to)118-124
Number of pages7
JournalRivista Italiana di Pediatria
Volume23
Issue number1
Publication statusPublished - Feb 1997

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ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Bisogno, G., Cecchetto, G., Sotti, G., Dallorso, S., Mancini, A., Donfrancesco, A., Cordero Di Montezemolo, L., Santoro, N., Tamaro, P., Masiero, L., & Carli, M. (1997). Il tumore maligno della guaine dei nervi periferici in eta pediatrica. Un'esperienza del Gruppo Cooperativo Italiano Sarcomi della Parti Molli. Rivista Italiana di Pediatria, 23(1), 118-124.