Malignant peripheral nerve sheath tumors in neurofibromatosis: Impact of family history

Fatema Malbari, Menachem Spira, Pamela B. Knight, Chong Zhu, Michael Roth, Jonathan Gill, Ira Richmond Abbott, III, Adam S. Levy

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Objective: The main objective of this study was to determine if family history of malignant peripheral nerve sheath tumor (MPNST) increases risk of developing an MPNST in patients with neurofibromatosis-1 (NF-1). Materials and Methods: Individuals with NF-1 registered with the Children's Tumor Foundation's Neurofibromatosis Registry were emailed an anonymous 15-minute survey with regard to personal and family history of NF-1,MPNST, ages of onset, and symptomatology. Participation was voluntary and information was self-reported. Results: The survey was sent to 4801 registrants, 878 responded. Presence of a family history of MPNST was found to be a risk factor for the development of MPNST; 19.4% of respondents confirming a family history of MPNST developed MPNST compared with 7.5% of respondents with no family history (odds ratio, 2.975; 95% confidence interval, 1.232-7.187; P=0.021). NF-1 patients with a positive family history developed MPNST at a younger age than those with no family history (8.3% vs. 0.5% P=0.003 and 13.9% vs. 2.4% P=0.003, for onset before 10 and 20, respectively). In the MPNST population with a known family history, onset prior to age 10 was significantly more prevalent (42.9% vs. 7% P=0.029). Conclusions: These results suggest a positive family history of MPNST represents a risk factor for the development and early onset of MPNST in individuals with NF-1.

Original languageEnglish (US)
Pages (from-to)e359-e363
JournalJournal of Pediatric Hematology/Oncology
Volume40
Issue number6
DOIs
StatePublished - Aug 1 2018

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Neurofibromatoses
Neurilemmoma
Neurofibromatosis 1
Age of Onset
Registries
Odds Ratio
Confidence Intervals

Keywords

  • family history
  • malignant peripheral nerve sheath tumor
  • neurofibromatosis-1

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Malignant peripheral nerve sheath tumors in neurofibromatosis : Impact of family history. / Malbari, Fatema; Spira, Menachem; Knight, Pamela B.; Zhu, Chong; Roth, Michael; Gill, Jonathan; Abbott, III, Ira Richmond; Levy, Adam S.

In: Journal of Pediatric Hematology/Oncology, Vol. 40, No. 6, 01.08.2018, p. e359-e363.

Research output: Contribution to journalArticle

Malbari, Fatema ; Spira, Menachem ; Knight, Pamela B. ; Zhu, Chong ; Roth, Michael ; Gill, Jonathan ; Abbott, III, Ira Richmond ; Levy, Adam S. / Malignant peripheral nerve sheath tumors in neurofibromatosis : Impact of family history. In: Journal of Pediatric Hematology/Oncology. 2018 ; Vol. 40, No. 6. pp. e359-e363.
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abstract = "Objective: The main objective of this study was to determine if family history of malignant peripheral nerve sheath tumor (MPNST) increases risk of developing an MPNST in patients with neurofibromatosis-1 (NF-1). Materials and Methods: Individuals with NF-1 registered with the Children's Tumor Foundation's Neurofibromatosis Registry were emailed an anonymous 15-minute survey with regard to personal and family history of NF-1,MPNST, ages of onset, and symptomatology. Participation was voluntary and information was self-reported. Results: The survey was sent to 4801 registrants, 878 responded. Presence of a family history of MPNST was found to be a risk factor for the development of MPNST; 19.4{\%} of respondents confirming a family history of MPNST developed MPNST compared with 7.5{\%} of respondents with no family history (odds ratio, 2.975; 95{\%} confidence interval, 1.232-7.187; P=0.021). NF-1 patients with a positive family history developed MPNST at a younger age than those with no family history (8.3{\%} vs. 0.5{\%} P=0.003 and 13.9{\%} vs. 2.4{\%} P=0.003, for onset before 10 and 20, respectively). In the MPNST population with a known family history, onset prior to age 10 was significantly more prevalent (42.9{\%} vs. 7{\%} P=0.029). Conclusions: These results suggest a positive family history of MPNST represents a risk factor for the development and early onset of MPNST in individuals with NF-1.",
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AU - Gill, Jonathan

AU - Abbott, III, Ira Richmond

AU - Levy, Adam S.

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