Neurofibromatosis type 1

DH Gutmann, RE Ferner, RH Listernick… - Nature Reviews …, 2017 - nature.com
Neurofibromatosis type 1 is a complex autosomal dominant disorder caused by germline
mutations in the NF1 tumour suppressor gene. Nearly all individuals with neurofibromatosis …

Neurofibromatosis type 1: a multidisciplinary approach to care

AC Hirbe, DH Gutmann - The Lancet Neurology, 2014 - thelancet.com
Neurofibromatosis type 1 is a relatively common inherited disorder. Patients have a high
predisposition to develop both benign and malignant tumours. Although many …

A RASopathy gene commonly mutated in cancer: the neurofibromatosis type 1 tumour suppressor

N Ratner, SJ Miller - Nature Reviews Cancer, 2015 - nature.com
Abstract Neurofibromatosis type 1 (NF1) is a common genetic disorder that predisposes
affected individuals to tumours. The NF1 gene encodes a RAS GTPase-activating protein …

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 …

Neurofibromatosis 1 French national guidelines based on an extensive literature review since 1966

C Bergqvist, A Servy, L Valeyrie-Allanore… - Orphanet Journal of …, 2020 - Springer
Neurofibromatosis type 1 is a relatively common genetic disease, with a prevalence ranging
between 1/3000 and 1/6000 people worldwide. The disease affects multiple systems with …

Malignant peripheral nerve sheath tumors

M Farid, EG Demicco, R Garcia, L Ahn… - The …, 2014 - academic.oup.com
Learning Objectives Explain the characteristics and treatment of malignant peripheral nerve
sheath tumors, both in relation to neurofibromatosis type I and otherwise. Cite the unique …

Neurofibromatosis type 1 revisited

VC Williams, J Lucas, MA Babcock, DH Gutmann… - …, 2009 - publications.aap.org
Neurofibromatosis type 1 (NF1) is an autosomal dominant condition with a worldwide
incidence of∼ 1 per 2500 to 3000 individuals. Caused by a germ-line–inactivating mutation …

[HTML][HTML] MEK inhibition exhibits efficacy in human and mouse neurofibromatosis tumors

WJ Jessen, SJ Miller, E Jousma, J Wu… - The Journal of …, 2013 - Am Soc Clin Investig
Children with neurofibromatosis type 1 (NF1) are predisposed to juvenile myelomonocytic
leukemia (JMML), an aggressive myeloproliferative neoplasm (MPN) that is refractory to …

Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience

CCH Stucky, KN Johnson, RJ Gray, BA Pockaj… - Annals of surgical …, 2012 - Springer
Background Malignant peripheral nerve sheath tumors (MPNST) are a rare form of soft
tissue sarcoma with few studies reporting on patient outcomes and prognostic variables …

Soft tissue sarcoma across the age spectrum: a population‐based study from the surveillance epidemiology and end results database

A Ferrari, I Sultan, TT Huang… - Pediatric blood & …, 2011 - Wiley Online Library
Background Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal
malignancies that occur throughout the lifespan. The impact of age on disease features and …