TY - JOUR
T1 - Malignant peripheral nerve sheath tumors in neurofibromatosis
T2 - Impact of family history
AU - Malbari, Fatema
AU - Spira, Menachem
AU - Knight, Pamela B.
AU - Zhu, Chong
AU - Roth, Michael
AU - Gill, Jonathan
AU - Abbott, Rick
AU - Levy, Adam S.
N1 - Publisher Copyright:
© 2018 Wolters Kluwer Health, Inc. All rights reserved.
PY - 2018/8/1
Y1 - 2018/8/1
N2 - 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.
AB - 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.
KW - family history
KW - malignant peripheral nerve sheath tumor
KW - neurofibromatosis-1
UR - http://www.scopus.com/inward/record.url?scp=85050970181&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85050970181&partnerID=8YFLogxK
U2 - 10.1097/MPH.0000000000001156
DO - 10.1097/MPH.0000000000001156
M3 - Article
C2 - 29683959
AN - SCOPUS:85050970181
SN - 1077-4114
VL - 40
SP - e359-e363
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
IS - 6
ER -