Therapeutic advances for the tumors associated with neurofibromatosis type 1, type 2, and schwannomatosis

Ketotifen suppression of NF1 neurofibroma growth over 30 years

(2015) Vincent M. Riccardi   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Phase II study of mTORC1 inhibition by everolimus in neurofibromatosis type 2 patients with growing vestibular schwannomas

(2015) Stéphane Goutagny et al.   JOURNAL OF NEURO-ONCOLOGY

Gamma Knife radiosurgery for meningiomas in patients with neurofibromatosis Type 2

(2015) Ann Liu et al.   JOURNAL OF NEUROSURGERY

Neurofibromin 1 (NF1) Defects Are Common in Human Ovarian Serous Carcinomas and Co-occur with TP53 Mutations

(2015) Navneet Sangha et al.   NEOPLASIA

Relationship between whole-body tumor burden, clinical phenotype, and quality of life in patients with neurofibromatosis

(2014) Vanessa L. Merker et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Merlin/NF2 Loss-Driven Tumorigenesis Linked to CRL4DCAF1-Mediated Inhibition of the Hippo Pathway Kinases Lats1 and 2 in the Nucleus

(2014) Wei Li et al.   CANCER CELL

Combined MEK Inhibition and BMP2 Treatment Promotes Osteoblast Differentiation and Bone Healing inNf1Osx−/−Mice

(2014) Jean de la Croix Ndong et al.   JOURNAL OF BONE AND MINERAL RESEARCH

Prolonged survival in adult neurofibromatosis type I patients with recurrent high-grade gliomas treated with bevacizumab

(2014) Brett J. Theeler et al.   JOURNAL OF NEUROLOGY

Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2

(2014) Rosalie E. Ferner et al.   JOURNAL OF NEUROLOGY

Clinical course of vestibular schwannoma in pediatric neurofibromatosis Type 2

(2014) Jung Won Choi et al.   Journal of Neurosurgery-Pediatrics

Li-Fraumeni syndrome: cancer risk assessment and clinical management

(2014) Kate A. McBride et al.   Nature Reviews Clinical Oncology

Phase 2 randomized, flexible crossover, double-blinded, placebo-controlled trial of the farnesyltransferase inhibitor tipifarnib in children and young adults with neurofibromatosis type 1 and progressive plexiform neurofibromas

(2014) B. C. Widemann et al.   NEURO-ONCOLOGY

Akt- or MEK-mediated mTOR inhibition suppresses Nf1 optic glioma growth

(2014) Aparna Kaul et al.   NEURO-ONCOLOGY

CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2007-2011

(2014) Q. T. Ostrom et al.   NEURO-ONCOLOGY

Sirolimus for progressive neurofibromatosis type 1-associated plexiform neurofibromas: a Neurofibromatosis Clinical Trials Consortium phase II study

(2014) B. Weiss et al.   NEURO-ONCOLOGY

Mutations in LZTR1 add to the complex heterogeneity of schwannomatosis

(2014) M. J. Smith et al.   NEUROLOGY

Clinical response to bevacizumab in schwannomatosis

(2014) J. Blakeley et al.   NEUROLOGY

Phase II trial of pirfenidone in children and young adults with neurofibromatosis type 1 and progressive plexiform neurofibromas

(2014) Brigitte C. Widemann et al.   PEDIATRIC BLOOD & CANCER

Merlin Deficiency Predicts FAK Inhibitor Sensitivity: A Synthetic Lethal Relationship

(2014) I. M. Shapiro et al.   Science Translational Medicine

Defining Key Signaling Nodes and Therapeutic Biomarkers in NF1-Mutant Cancers

(2014) C. F. Malone et al.   Cancer Discovery

SMARCB1 mutations in schwannomatosis and genotype correlations with rhabdoid tumors

(2014) Miriam J. Smith et al.   Cancer Genetics

Quantitative analysis of mammalian GIRK2 channel regulation by G proteins, the signaling lipid PIP2 and Na+ in a reconstituted system

(2014) Weiwei Wang et al.   eLife

Handheld Optical Coherence Tomography During Sedation in Young Children With Optic Pathway Gliomas

(2014) Robert A. Avery et al.   JAMA Ophthalmology

Update from the 2011 International Schwannomatosis Workshop: From genetics to diagnostic criteria

(2013) Scott R. Plotkin et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Optimizing biologically targeted clinical trials for neurofibromatosis

(2013) David H Gutmann et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Approaches to Treating NF1 Tibial Pseudarthrosis

(2013) David A. Stevenson et al.   JOURNAL OF PEDIATRIC ORTHOPAEDICS

Genomic sequencing of meningiomas identifies oncogenic SMO and AKT1 mutations

(2013) Priscilla K Brastianos et al.   NATURE GENETICS

Proteomic and bioinformatic analysis of mammalian SWI/SNF complexes identifies extensive roles in human malignancy

(2013) Cigall Kadoch et al.   NATURE GENETICS

Phase II study of everolimus in children and adults with neurofibromatosis type 2 and progressive vestibular schwannomas

(2013) Matthias A. Karajannis et al.   NEURO-ONCOLOGY

Hearing and facial function outcomes for neurofibromatosis 2 clinical trials

(2013) S. R. Plotkin et al.   NEUROLOGY

Conclusions and future directions for the REiNS International Collaboration

(2013) B. C. Widemann et al.   NEUROLOGY

Recommendations for imaging tumor response in neurofibromatosis clinical trials

(2013) E. Dombi et al.   NEUROLOGY

Functional outcome measures for NF1-associated optic pathway glioma clinical trials

(2013) M. J. Fisher et al.   NEUROLOGY

Conservative Management of Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2 Patients

(2013) Matthieu Peyre et al.   NEUROSURGERY

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

(2013) Scott R. Plotkin et al.   OTOLOGY & NEUROTOLOGY

Marked Recovery of Vision in Children With Optic Pathway Gliomas Treated With Bevacizumab

(2013) Robert A. Avery et al.   JAMA Ophthalmology

Abstract 4686: Requirements of Gαi1 and Gαi3 for brain-derived neurotrophic factor (BDNF) and nerve growth factor (NGF) induced PI3K/AKT and MAPK signaling as well as neuronal survival and migration

(2012) Cong Cao et al.   CANCER RESEARCH

Internal tumor burden in neurofibromatosis Type I patients with large NF1 deletions

(2012) Lan Kluwe et al.   GENES CHROMOSOMES & CANCER

Comparative Oncogenomics Implicates the Neurofibromin 1 Gene (NF1) as a Breast Cancer Driver

(2012) M. D. Wallace et al.   GENETICS

Spectrum and Prevalence of Vasculopathy in Pediatric Neurofibromatosis Type 1

(2012) Bonnie Kaas et al.   JOURNAL OF CHILD NEUROLOGY

Life expectancy in hereditary cancer predisposing diseases: an observational study

(2012) Anna Wilding et al.   JOURNAL OF MEDICAL GENETICS

Long-term natural history of neurofibromatosis Type 2–associated intracranial tumors

(2012) Michael S. Dirks et al.   JOURNAL OF NEUROSURGERY

Imatinib mesylate for plexiform neurofibromas in patients with neurofibromatosis type 1: a phase 2 trial

(2012) Kent A Robertson et al.   LANCET ONCOLOGY

Regulation of mTOR Complex 2 Signaling in Neurofibromatosis 2-Deficient Target Cell Types

(2012) M. F. James et al.   MOLECULAR CANCER RESEARCH

Phase II trial of lapatinib in adult and pediatric patients with neurofibromatosis type 2 and progressive vestibular schwannomas

(2012) Matthias A. Karajannis et al.   NEURO-ONCOLOGY

Visual outcomes in children with neurofibromatosis type 1-associated optic pathway glioma following chemotherapy: a multicenter retrospective analysis

(2012) M. J. Fisher et al.   NEURO-ONCOLOGY

Frequency of SMARCB1 mutations in familial and sporadic schwannomatosis

(2012) Miriam J. Smith et al.   NEUROGENETICS

The NF2 tumor suppressor regulates microtubule-based vesicle trafficking via a novel Rac, MLK and p38SAPK pathway

(2012) R F Hennigan et al.   ONCOGENE

Clinical Features of Schwannomatosis: A Retrospective Analysis of 87 Patients

(2012) V. L. Merker et al.   ONCOLOGIST

Bevacizumab for Progressive Vestibular Schwannoma in Neurofibromatosis Type 2

(2012) Scott R. Plotkin et al.   OTOLOGY & NEUROTOLOGY

Phase I trial and pharmacokinetic study of sorafenib in children with neurofibromatosis type I and plexiform neurofibromas

(2012) AeRang Kim et al.   PEDIATRIC BLOOD & CANCER

Vestibular schwannomas occur in schwannomatosis and should not be considered an exclusion criterion for clinical diagnosis

(2011) Miriam J. Smith et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Pathogenesis of Plexiform Neurofibroma: Tumor-Stromal/Hematopoietic Interactions in Tumor Progression

(2011) Karl Staser et al.   Annual Review of Pathology-Mechanisms of Disease

SMARCB1/INI1 germline mutations contribute to 10% of sporadic schwannomatosis

(2011) Guillaume Rousseau et al.   BMC Neurology

A phase 2 study of pegylated interferon α-2b (PEG-Intron®) in children with diffuse intrinsic pontine glioma

(2011) Katherine Warren et al.   CANCER

Dose-Dependent Effects of Focal Fractionated Irradiation on Secondary Malignant Neoplasms in Nf1 Mutant Mice

(2011) J. L. Nakamura et al.   CANCER RESEARCH

Genetic testing and screening of individuals at risk of NF2

(2011) DG Evans et al.   CLINICAL GENETICS

Pediatric Plexiform Neurofibromas: Impact on Morbidity and Mortality in Neurofibromatosis Type 1

(2011) Carlos E. Prada et al.   JOURNAL OF PEDIATRICS

Pulmonary Hypertension in Patients With Neurofibromatosis Type I

(2011) David Montani et al.   MEDICINE

Nilotinib alone or in combination with selumetinib is a drug candidate for neurofibromatosis type 2

(2011) S. Ammoun et al.   NEURO-ONCOLOGY

AR42, a novel histone deacetylase inhibitor, as a potential therapy for vestibular schwannomas and meningiomas

(2011) M. L. Bush et al.   NEURO-ONCOLOGY

Germline SMARCB1 mutation predisposes to multiple meningiomas and schwannomas with preferential location of cranial meningiomas at the falx cerebri

(2011) Pepijn van den Munckhof et al.   NEUROGENETICS

Phase I trial of pegylated interferon- -2b in young patients with plexiform neurofibromas

(2011) R. I. Jakacki et al.   NEUROLOGY

Merlin/NF2 Functions Upstream of the Nuclear E3 Ubiquitin Ligase CRL4DCAF1 to Suppress Oncogenic Gene Expression

(2011) J. Cooper et al.   Science Signaling

Birth incidence and prevalence of tumor-prone syndromes: Estimates from a UK family genetic register service

(2010) D.G. Evans et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

A mutant allele of the Swi/Snf member BAF250a determines the pool size of fetal liver hemopoietic stem cell populations

(2010) J. Krosl et al.   BLOOD

Anti–Vascular Endothelial Growth Factor Therapies as a Novel Therapeutic Approach to Treating Neurofibromatosis-Related Tumors

(2010) Hon Kit Wong et al.   CANCER RESEARCH

Merlin, a “Magic” Linker Between the Extracellular Cues and Intracellular Signaling Pathways that Regulate Cell Motility, Proliferation, and Survival

(2010) Ivan Stamenkovic et al.   CURRENT PROTEIN & PEPTIDE SCIENCE

SWI/SNF complexes, chromatin remodeling and skeletal myogenesis: It's time to exchange!

(2010) Sonia Albini et al.   EXPERIMENTAL CELL RESEARCH

SMARCB1 mutations are not a common cause of multiple meningiomas

(2010) K. D. Hadfield et al.   JOURNAL OF MEDICAL GENETICS

Meningiomas and neurofibromatosis

(2010) Stéphane Goutagny et al.   JOURNAL OF NEURO-ONCOLOGY

CXCL12 alone is insufficient for gliomagenesis in Nf1 mutant mice

(2010) Tao Sun et al.   JOURNAL OF NEUROIMMUNOLOGY

The molecular pathology of cancer

(2010) Timothy J. R. Harris et al.   Nature Reviews Clinical Oncology

The Diagnostic and Clinical Significance of Café-au-lait Macules

(2010) Kara N. Shah   PEDIATRIC CLINICS OF NORTH AMERICA

Consensus Recommendations to Accelerate Clinical Trials for Neurofibromatosis Type 2

(2009) D. G. Evans et al.   CLINICAL CANCER RESEARCH

Alterations in the SMARCB1 (INI1) tumor suppressor gene in familial schwannomatosis

(2009) C Boyd et al.   CLINICAL GENETICS

ATP-dependent chromatin remodeling in neural development

(2009) Andrew S Yoo et al.   CURRENT OPINION IN NEUROBIOLOGY

Gliomas in patients with neurofibromatosis type 1

(2009) Anne C Albers et al.   Expert Review of Neurotherapeutics

Schwannomatosis associated with multiple meningiomas due to a familial SMARCB1 mutation

(2009) Costanza Bacci et al.   NEUROGENETICS

THREE-DIMENSIONAL VOLUMETRICS FOR TRACKING VESTIBULAR SCHWANNOMA GROWTH IN NEUROFIBROMATOSIS TYPE II

(2009) Gordon J. Harris et al.   NEUROSURGERY

Hearing Improvement after Bevacizumab in Patients with Neurofibromatosis Type 2

(2009) Scott R. Plotkin et al.   NEW ENGLAND JOURNAL OF MEDICINE

Concordance of Bilateral Vestibular Schwannoma Growth and Hearing Changes in Neurofibromatosis 2

(2009) Laurel M. Fisher et al.   OTOLOGY & NEUROTOLOGY

Three new datasets supporting use of the Numerical Rating Scale (NRS-11) for children’s self-reports of pain intensity

(2009) Carl L. von Baeyer et al.   PAIN

Clinical Experience With Radiation Therapy in the Management of Neurofibromatosis-Associated Central Nervous System Tumors

(2008) Stacy Wentworth et al.   INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS

Comprehensive genomic characterization defines human glioblastoma genes and core pathways

(2008) Roger McLendon et al.   NATURE

Magic but treatable? Tumours due to loss of Merlin

(2007) C. O. Hanemann   BRAIN

Evidence of a four-hit mechanism involvingSMARCB1andNF2in schwannomatosis-associated schwannomas

(2007) Roberta Sestini et al.   HUMAN MUTATION