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 …

Perspectives on 3D bioprinting of peripheral nerve conduits

SS Soman, S Vijayavenkataraman - International journal of molecular …, 2020 - mdpi.com
The peripheral nervous system controls the functions of sensation, movement and motor
coordination of the body. Peripheral nerves can get damaged easily by trauma or …

[HTML][HTML] Humanized neurofibroma model from induced pluripotent stem cells delineates tumor pathogenesis and developmental origins

J Mo, C Anastasaki, Z Chen, T Shipman… - The Journal of …, 2021 - Am Soc Clin Investig
Neurofibromatosis type 1 (NF1) is a common tumor predisposition syndrome caused by NF1
gene mutation, in which affected patients develop Schwann cell lineage peripheral nerve …

[HTML][HTML] Modeling iPSC-derived human neurofibroma-like tumors in mice uncovers the heterogeneity of Schwann cells within plexiform neurofibromas

H Mazuelas, M Magallón-Lorenz… - Cell Reports, 2022 - cell.com
Plexiform neurofibromas (pNFs) are developmental tumors that appear in neurofibromatosis
type 1 individuals, constituting a major source of morbidity and potentially transforming into a …

Development and in vitro differentiation of Schwann cells

SJ Hörner, N Couturier, DC Gueiber, M Hafner… - Cells, 2022 - mdpi.com
Schwann cells are glial cells of the peripheral nervous system. They exist in several
subtypes and perform a variety of functions in nerves. Their derivation and culture in vitro are …

Neurofibromatosis in the era of precision medicine: development of MEK inhibitors and recent successes with selumetinib

R Galvin, AL Watson, DA Largaespada, N Ratner… - Current oncology …, 2021 - Springer
Abstract Purpose of Review Patients with neurofibromatosis type 1 (NF1) are at increased
risk for benign and malignant neoplasms. Recently, targeted therapy with the MEK inhibitor …

Neuron-Schwann cell interactions in peripheral nervous system homeostasis, disease, and preclinical treatment

JT Oliveira, C Yanick, N Wein… - Frontiers in Cellular …, 2023 - frontiersin.org
Schwann cells (SCs) have a critical role in the peripheral nervous system. These cells are
able to support axons during homeostasis and after injury. However, mutations in genes …

Human organ‐specific 3D cancer models produced by the stromal self‐assembly method of tissue engineering for the study of solid tumors

V Roy, B Magne, M Vaillancourt-Audet… - BioMed Research …, 2020 - Wiley Online Library
Cancer research has considerably progressed with the improvement of in vitro study
models, helping to understand the key role of the tumor microenvironment in cancer …

Atypical neurofibromas reveal distinct epigenetic features with proximity to benign peripheral nerve sheath tumor entities

C Kresbach, M Dottermusch, A Eckhardt… - Neuro …, 2023 - academic.oup.com
Background Plexiform neurofibromas can transform into atypical neurofibromas (ANF) and
then further progress to aggressive malignant peripheral nerve sheath tumors (MPNST) …

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 …