Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1

Matthias Kolberg, Maren Høland, Trude H. Agesen, Helge R. Brekke, Knut Liestøl, Kirsten S. Hall, Fredrik Mertens, Piero Picci, Sigbjørn Smeland, Ragnhild A. Lothe

Research output: Contribution to journalReview article

106 Citations (Scopus)

Abstract

There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have performed meta-analyses testing the effect of NF1 status on MPNST survival based on publications from the last 50 years, including only nonoverlapping patients reported from each institution. In addition, we analyzed survival characteristics for 179 MPNST patients from 3 European sarcoma centers. The meta-analyses including data from a total of 48 studies and >1800 patients revealed a significantly higher odds ratio for overall survival (OR OS) and disease-specific survival (ORDSS) in the non-NF1 group (OROS 5 1.75, 95% confidence interval [CI] 5 1.28- 2.39, and OR DSS 5 1.68, 95% CI 5 1.18-2.40). However, in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis. For our own MPNST patients, NF1 status had no effect on overall or disease-specific survival. The compiled literature from 1963 to the present indicates a significantly worse outcome of MPNST in patients with NF1 syndrome compared with non-NF1 patients. However, survival for the NF1 patients has improved in the last decade, and the survival difference is diminishing. These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status.

Original languageEnglish (US)
Pages (from-to)135-147
Number of pages13
JournalNeuro-oncology
Volume15
Issue number2
DOIs
StatePublished - Feb 1 2013

Fingerprint

Neurofibromatosis 1
Neurilemmoma
Survival Analysis
Meta-Analysis
Survival
Confidence Intervals
Neurofibromatoses
Sarcoma
Publications
Odds Ratio

Keywords

  • MPNST
  • Metaanalysis
  • NF1
  • Neurofibromatosis

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

Cite this

Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. / Kolberg, Matthias; Høland, Maren; Agesen, Trude H.; Brekke, Helge R.; Liestøl, Knut; Hall, Kirsten S.; Mertens, Fredrik; Picci, Piero; Smeland, Sigbjørn; Lothe, Ragnhild A.

In: Neuro-oncology, Vol. 15, No. 2, 01.02.2013, p. 135-147.

Research output: Contribution to journalReview article

Kolberg, Matthias ; Høland, Maren ; Agesen, Trude H. ; Brekke, Helge R. ; Liestøl, Knut ; Hall, Kirsten S. ; Mertens, Fredrik ; Picci, Piero ; Smeland, Sigbjørn ; Lothe, Ragnhild A. / Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. In: Neuro-oncology. 2013 ; Vol. 15, No. 2. pp. 135-147.
@article{8b6bc698d6bd4162b6f3e54e6126e1ea,
title = "Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1",
abstract = "There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have performed meta-analyses testing the effect of NF1 status on MPNST survival based on publications from the last 50 years, including only nonoverlapping patients reported from each institution. In addition, we analyzed survival characteristics for 179 MPNST patients from 3 European sarcoma centers. The meta-analyses including data from a total of 48 studies and >1800 patients revealed a significantly higher odds ratio for overall survival (OR OS) and disease-specific survival (ORDSS) in the non-NF1 group (OROS 5 1.75, 95{\%} confidence interval [CI] 5 1.28- 2.39, and OR DSS 5 1.68, 95{\%} CI 5 1.18-2.40). However, in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis. For our own MPNST patients, NF1 status had no effect on overall or disease-specific survival. The compiled literature from 1963 to the present indicates a significantly worse outcome of MPNST in patients with NF1 syndrome compared with non-NF1 patients. However, survival for the NF1 patients has improved in the last decade, and the survival difference is diminishing. These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status.",
keywords = "MPNST, Metaanalysis, NF1, Neurofibromatosis",
author = "Matthias Kolberg and Maren H{\o}land and Agesen, {Trude H.} and Brekke, {Helge R.} and Knut Liest{\o}l and Hall, {Kirsten S.} and Fredrik Mertens and Piero Picci and Sigbj{\o}rn Smeland and Lothe, {Ragnhild A.}",
year = "2013",
month = "2",
day = "1",
doi = "10.1093/neuonc/nos287",
language = "English (US)",
volume = "15",
pages = "135--147",
journal = "Neuro-Oncology",
issn = "1522-8517",
publisher = "Oxford University Press",
number = "2",

}

TY - JOUR

T1 - Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1

AU - Kolberg, Matthias

AU - Høland, Maren

AU - Agesen, Trude H.

AU - Brekke, Helge R.

AU - Liestøl, Knut

AU - Hall, Kirsten S.

AU - Mertens, Fredrik

AU - Picci, Piero

AU - Smeland, Sigbjørn

AU - Lothe, Ragnhild A.

PY - 2013/2/1

Y1 - 2013/2/1

N2 - There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have performed meta-analyses testing the effect of NF1 status on MPNST survival based on publications from the last 50 years, including only nonoverlapping patients reported from each institution. In addition, we analyzed survival characteristics for 179 MPNST patients from 3 European sarcoma centers. The meta-analyses including data from a total of 48 studies and >1800 patients revealed a significantly higher odds ratio for overall survival (OR OS) and disease-specific survival (ORDSS) in the non-NF1 group (OROS 5 1.75, 95% confidence interval [CI] 5 1.28- 2.39, and OR DSS 5 1.68, 95% CI 5 1.18-2.40). However, in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis. For our own MPNST patients, NF1 status had no effect on overall or disease-specific survival. The compiled literature from 1963 to the present indicates a significantly worse outcome of MPNST in patients with NF1 syndrome compared with non-NF1 patients. However, survival for the NF1 patients has improved in the last decade, and the survival difference is diminishing. These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status.

AB - There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have performed meta-analyses testing the effect of NF1 status on MPNST survival based on publications from the last 50 years, including only nonoverlapping patients reported from each institution. In addition, we analyzed survival characteristics for 179 MPNST patients from 3 European sarcoma centers. The meta-analyses including data from a total of 48 studies and >1800 patients revealed a significantly higher odds ratio for overall survival (OR OS) and disease-specific survival (ORDSS) in the non-NF1 group (OROS 5 1.75, 95% confidence interval [CI] 5 1.28- 2.39, and OR DSS 5 1.68, 95% CI 5 1.18-2.40). However, in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis. For our own MPNST patients, NF1 status had no effect on overall or disease-specific survival. The compiled literature from 1963 to the present indicates a significantly worse outcome of MPNST in patients with NF1 syndrome compared with non-NF1 patients. However, survival for the NF1 patients has improved in the last decade, and the survival difference is diminishing. These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status.

KW - MPNST

KW - Metaanalysis

KW - NF1

KW - Neurofibromatosis

UR - http://www.scopus.com/inward/record.url?scp=84875702670&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84875702670&partnerID=8YFLogxK

U2 - 10.1093/neuonc/nos287

DO - 10.1093/neuonc/nos287

M3 - Review article

C2 - 23161774

AN - SCOPUS:84875702670

VL - 15

SP - 135

EP - 147

JO - Neuro-Oncology

JF - Neuro-Oncology

SN - 1522-8517

IS - 2

ER -