Malignant peripheral nerve sheath tumors: Analysis of the national cancer database

Alia Mowery, Daniel Clayburgh

Research output: Contribution to journalArticle

Abstract

Objectives: Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcoma (STS) that carries significant mortality. Given the rarity of MPNST, current knowledge on the disease is limited. We aim to characterize patient, tumor, and treatment factors, and determine variables associated with 5-year overall survival (OS) in individuals with MPNST. Materials and Methods: We utilized the National Cancer Database (NCDB) to identify adult patients diagnosed with MPNST from 2004 to 2015, and analyze patient, tumor, treatment, and survival data. American Joint Committee on Cancer (AJCC 8) staging criteria for STS was utilized. Results: In our cohort of 2,858 patients with MPNST, median age at diagnosis was 47. The 5-year OS rate was 52%. Age, race, tumor size, and treatment regimen significantly predicted 5-year OS on univariate analysis. AJCC 8 stage appeared to predict 5-year OS in head and neck tumors (though not significantly), but not trunk and extremity tumors. Conclusion: While our cohort of patients with MPNST are typically relatively young and healthy, 5-year OS is only 52%. Notably, age extremes, large tumor size, and certain treatment regimens are associated with worse outcomes. While AJCC 8 staging guidelines may have some value in predicting OS in head and neck tumors, they do not reliably risk stratify patients with trunk and extremity MPNST. Ultimately, MPNST is associated with high mortality with few modifiable risk factors, and survival may not be adequately predicted by current staging systems.

Original languageEnglish (US)
Pages (from-to)13-19
Number of pages7
JournalOral Oncology
Volume98
DOIs
StatePublished - Nov 1 2019

Fingerprint

Neurilemmoma
Databases
Survival
Neoplasms
Sarcoma
Neck
Extremities
Head
Mortality
Therapeutics
Survival Rate
Guidelines

Keywords

  • Head and neck neoplasms
  • Neoplasm staging
  • Sarcoma
  • Survival analysis

ASJC Scopus subject areas

  • Oral Surgery
  • Oncology
  • Cancer Research

Cite this

Malignant peripheral nerve sheath tumors : Analysis of the national cancer database. / Mowery, Alia; Clayburgh, Daniel.

In: Oral Oncology, Vol. 98, 01.11.2019, p. 13-19.

Research output: Contribution to journalArticle

@article{d5129b4e230f446c900ef68de96206b7,
title = "Malignant peripheral nerve sheath tumors: Analysis of the national cancer database",
abstract = "Objectives: Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcoma (STS) that carries significant mortality. Given the rarity of MPNST, current knowledge on the disease is limited. We aim to characterize patient, tumor, and treatment factors, and determine variables associated with 5-year overall survival (OS) in individuals with MPNST. Materials and Methods: We utilized the National Cancer Database (NCDB) to identify adult patients diagnosed with MPNST from 2004 to 2015, and analyze patient, tumor, treatment, and survival data. American Joint Committee on Cancer (AJCC 8) staging criteria for STS was utilized. Results: In our cohort of 2,858 patients with MPNST, median age at diagnosis was 47. The 5-year OS rate was 52{\%}. Age, race, tumor size, and treatment regimen significantly predicted 5-year OS on univariate analysis. AJCC 8 stage appeared to predict 5-year OS in head and neck tumors (though not significantly), but not trunk and extremity tumors. Conclusion: While our cohort of patients with MPNST are typically relatively young and healthy, 5-year OS is only 52{\%}. Notably, age extremes, large tumor size, and certain treatment regimens are associated with worse outcomes. While AJCC 8 staging guidelines may have some value in predicting OS in head and neck tumors, they do not reliably risk stratify patients with trunk and extremity MPNST. Ultimately, MPNST is associated with high mortality with few modifiable risk factors, and survival may not be adequately predicted by current staging systems.",
keywords = "Head and neck neoplasms, Neoplasm staging, Sarcoma, Survival analysis",
author = "Alia Mowery and Daniel Clayburgh",
year = "2019",
month = "11",
day = "1",
doi = "10.1016/j.oraloncology.2019.09.010",
language = "English (US)",
volume = "98",
pages = "13--19",
journal = "Oral Oncology",
issn = "1368-8375",
publisher = "Elsevier Limited",

}

TY - JOUR

T1 - Malignant peripheral nerve sheath tumors

T2 - Analysis of the national cancer database

AU - Mowery, Alia

AU - Clayburgh, Daniel

PY - 2019/11/1

Y1 - 2019/11/1

N2 - Objectives: Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcoma (STS) that carries significant mortality. Given the rarity of MPNST, current knowledge on the disease is limited. We aim to characterize patient, tumor, and treatment factors, and determine variables associated with 5-year overall survival (OS) in individuals with MPNST. Materials and Methods: We utilized the National Cancer Database (NCDB) to identify adult patients diagnosed with MPNST from 2004 to 2015, and analyze patient, tumor, treatment, and survival data. American Joint Committee on Cancer (AJCC 8) staging criteria for STS was utilized. Results: In our cohort of 2,858 patients with MPNST, median age at diagnosis was 47. The 5-year OS rate was 52%. Age, race, tumor size, and treatment regimen significantly predicted 5-year OS on univariate analysis. AJCC 8 stage appeared to predict 5-year OS in head and neck tumors (though not significantly), but not trunk and extremity tumors. Conclusion: While our cohort of patients with MPNST are typically relatively young and healthy, 5-year OS is only 52%. Notably, age extremes, large tumor size, and certain treatment regimens are associated with worse outcomes. While AJCC 8 staging guidelines may have some value in predicting OS in head and neck tumors, they do not reliably risk stratify patients with trunk and extremity MPNST. Ultimately, MPNST is associated with high mortality with few modifiable risk factors, and survival may not be adequately predicted by current staging systems.

AB - Objectives: Malignant peripheral nerve sheath tumor (MPNST) is a rare type of soft tissue sarcoma (STS) that carries significant mortality. Given the rarity of MPNST, current knowledge on the disease is limited. We aim to characterize patient, tumor, and treatment factors, and determine variables associated with 5-year overall survival (OS) in individuals with MPNST. Materials and Methods: We utilized the National Cancer Database (NCDB) to identify adult patients diagnosed with MPNST from 2004 to 2015, and analyze patient, tumor, treatment, and survival data. American Joint Committee on Cancer (AJCC 8) staging criteria for STS was utilized. Results: In our cohort of 2,858 patients with MPNST, median age at diagnosis was 47. The 5-year OS rate was 52%. Age, race, tumor size, and treatment regimen significantly predicted 5-year OS on univariate analysis. AJCC 8 stage appeared to predict 5-year OS in head and neck tumors (though not significantly), but not trunk and extremity tumors. Conclusion: While our cohort of patients with MPNST are typically relatively young and healthy, 5-year OS is only 52%. Notably, age extremes, large tumor size, and certain treatment regimens are associated with worse outcomes. While AJCC 8 staging guidelines may have some value in predicting OS in head and neck tumors, they do not reliably risk stratify patients with trunk and extremity MPNST. Ultimately, MPNST is associated with high mortality with few modifiable risk factors, and survival may not be adequately predicted by current staging systems.

KW - Head and neck neoplasms

KW - Neoplasm staging

KW - Sarcoma

KW - Survival analysis

UR - http://www.scopus.com/inward/record.url?scp=85072208936&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85072208936&partnerID=8YFLogxK

U2 - 10.1016/j.oraloncology.2019.09.010

DO - 10.1016/j.oraloncology.2019.09.010

M3 - Article

AN - SCOPUS:85072208936

VL - 98

SP - 13

EP - 19

JO - Oral Oncology

JF - Oral Oncology

SN - 1368-8375

ER -