TY - JOUR
T1 - Diagnosis and management of malignant peripheral nerve sheath tumors
T2 - Current practice and future perspectives
AU - Prudner, Bethany C.
AU - Ball, Tyler
AU - Rathore, Richa
AU - Hirbe, Angela C.
N1 - Publisher Copyright:
© 2019 The Author(s) 2019. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.
PY - 2020/7/1
Y1 - 2020/7/1
N2 - One of the most common malignancies affecting adults with the neurofibromatosis type 1 (NF1) cancer predisposition syndrome is the malignant peripheral nerve sheath tumor (MPNST), a highly aggressive sarcoma that typically develops from benign plexiform neurofibromas. Approximately 8-13% of individuals with NF1 will develop MPNST during young adulthood. There are few therapeutic options, and the vast majority of people with these cancers will die within 5 years of diagnosis. Despite efforts to understand the pathogenesis of these aggressive tumors, the overall prognosis remains dismal. This manuscript will review the current understanding of the cellular and molecular progression of MPNST, diagnostic workup of patients with these tumors, current treatment paradigms, and investigational treatment options. Additionally, we highlight novel areas of preclinical research, which may lead to future clinical trials. In summary, MPNST remains a diagnostic and therapeutic challenge, and future work is needed to develop novel and rational combinational therapy for these tumors.
AB - One of the most common malignancies affecting adults with the neurofibromatosis type 1 (NF1) cancer predisposition syndrome is the malignant peripheral nerve sheath tumor (MPNST), a highly aggressive sarcoma that typically develops from benign plexiform neurofibromas. Approximately 8-13% of individuals with NF1 will develop MPNST during young adulthood. There are few therapeutic options, and the vast majority of people with these cancers will die within 5 years of diagnosis. Despite efforts to understand the pathogenesis of these aggressive tumors, the overall prognosis remains dismal. This manuscript will review the current understanding of the cellular and molecular progression of MPNST, diagnostic workup of patients with these tumors, current treatment paradigms, and investigational treatment options. Additionally, we highlight novel areas of preclinical research, which may lead to future clinical trials. In summary, MPNST remains a diagnostic and therapeutic challenge, and future work is needed to develop novel and rational combinational therapy for these tumors.
KW - MPNST
KW - diagnosis
KW - neurofibromatosis
KW - treatment
UR - http://www.scopus.com/inward/record.url?scp=85126615700&partnerID=8YFLogxK
U2 - 10.1093/noajnl/vdz047
DO - 10.1093/noajnl/vdz047
M3 - Article
AN - SCOPUS:85126615700
SN - 2632-2498
VL - 2
SP - I40-I49
JO - Neuro-Oncology Advances
JF - Neuro-Oncology Advances
ER -