Malignant peripheral nerve sheath tumor: models, biology, and translation

BN Somatilaka, A Sadek, RM McKay, LQ Le - Oncogene, 2022 - nature.com
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, invasive cancer that
comprise around 10% of all soft tissue sarcomas and develop in about 8–13% of patients …

Malignant peripheral nerve sheath tumors state of the science: leveraging clinical and biological insights into effective therapies

AR Kim, DR Stewart, KM Reilly, D Viskochil… - Sarcoma, 2017 - Wiley Online Library
Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in
patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed …

Genetically engineered minipigs model the major clinical features of human neurofibromatosis type 1

SH Isakson, AE Rizzardi, AW Coutts… - Communications …, 2018 - nature.com
Abstract Neurofibromatosis Type 1 (NF1) is a genetic disease caused by mutations in
Neurofibromin 1 (NF1). NF1 patients present with a variety of clinical manifestations and are …

Canonical Wnt/β-catenin signaling drives human schwann cell transformation, progression, and tumor maintenance

AL Watson, EP Rahrmann, BS Moriarity, K Choi… - Cancer discovery, 2013 - AACR
Genetic changes required for the formation and progression of human Schwann cell tumors
remain elusive. Using a Sleeping Beauty forward genetic screen, we identified several …

[HTML][HTML] Immortalization of human normal and NF1 neurofibroma Schwann cells

H Li, LJ Chang, DR Neubauer, DF Muir… - Laboratory …, 2016 - Elsevier
Neurofibromas, which are benign Schwann cell tumors, are the hallmark feature in the
autosomal dominant condition neurofibromatosis 1 (NF1) and are associated with biallelic …

STAT3 and HIF1α signaling drives oncogenic cellular phenotypes in malignant peripheral nerve sheath tumors

E Rad, K Dodd, L Thomas, M Upadhyaya… - Molecular Cancer …, 2015 - AACR
Therapeutic options are limited for neurofibromatosis type 1 (NF1)-associated malignant
peripheral nerve sheath tumors (MPNST) and clinical trials using drug agents have so far …

CDKs in sarcoma: mediators of disease and emerging therapeutic targets

JL Kohlmeyer, DJ Gordon, MR Tanas, V Monga… - International journal of …, 2020 - mdpi.com
Sarcomas represent one of the most challenging tumor types to treat due to their diverse
nature and our incomplete understanding of their underlying biology. Recent work suggests …

[HTML][HTML] Co-targeting the MAPK and PI3K/AKT/mTOR pathways in two genetically engineered mouse models of schwann cell tumors reduces tumor grade and …

AL Watson, LK Anderson, AD Greeley, VW Keng… - Oncotarget, 2014 - ncbi.nlm.nih.gov
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that occur
spontaneously, or from benign plexiform neurofibromas, in the context of the genetic …

New model systems and the development of targeted therapies for the treatment of neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors

KB Williams, DA Largaespada - Genes, 2020 - mdpi.com
Neurofibromatosis Type 1 (NF1) is a common genetic disorder and cancer predisposition
syndrome (1: 3000 births) caused by mutations in the tumor suppressor gene NF1. NF1 …

The challenge of cancer genomics in rare nervous system neoplasms: malignant peripheral nerve sheath tumors as a paradigm for cross-species comparative …

SL Carroll - The American journal of pathology, 2016 - Elsevier
Comprehensive genomic analyses of common nervous system cancers provide new
insights into their pathogenesis, diagnosis, and treatment. Although analogous studies of …