Results 11 to 20 of about 5,276 (179)

Chromosome 8 gain is associated with high-grade transformation in MPNST [PDF]

JCI Insight, 2021
One of the most common malignancies affecting adults with Neurofibromatosis type 1 (NF1) is the malignant peripheral nerve sheath tumor (MPNST), an aggressive and often fatal sarcoma that commonly arises from benign plexiform neurofibromas.
Carina Dehner, Chang In Moon, Xiyuan Zhang, Zhaohe Zhou, Chris Miller, Hua Xu, Xiaodan Wan, Kuangying Yang, Jay Mashl, Sara J.C. Gosline, Yuxi Wang, Xiaochun Zhang, Abigail Godec, Paul A. Jones, Sonika Dahiya, Himanshi Bhatia, Tina Primeau, Shunqiang Li, Kai Pollard, Fausto J. Rodriguez, Li Ding, Christine A. Pratilas, Jack F. Shern, Angela C. Hirbe   +23 more
doaj   +4 more sources

Metabolic Alterations in Macrophage Subtypes Propel Immune and Stromal Remodeling in Neurofibroma's Malignant Progression. [PDF]

MedComm (2020)
Illustration of the microenvironment factors driving malignant progression in MPNSTs. ABSTRACT Neurofibromatosis type 1 (NF1) is characterized by the development of benign plexiform neurofibromas (PNFs). In 10%–15% of patients, these tumors undergo malignant transformation into aggressive malignant peripheral nerve sheath tumors (MPNSTs).
Ge LL, Li YH, Yu X, Xing MY, Gu YH, Wang W, Huang JX, Liu J, Zhang HB, Wei CJ, Wang ZC, Li QF.   +11 more
europepmc   +2 more sources

Combination therapies for MPNSTs targeting RABL6A-RB1 signaling [PDF]

Oncotarget, 2021
Precision medicine relies on a detailed molecular understanding of disease pathogenesis. Here, we consider urgently needed therapeutic options for malignant peripheral nerve sheath tumors (MPNSTs) based on emerging insights into druggable pathway alterations found to drive this deadly cancer.
Kohlmeyer, Jordan L., Gordon, David J., Tanas, Munir R., Dodd, Rebecca D., Monga, Varun, Darbro, Benjamin W., Quelle, Dawn E.   +6 more
openaire   +2 more sources

“MPNST Epigenetics”—Response [PDF]

Molecular Cancer Research, 2019
We thank the authors for their letter ([1][1]) and for making these points about our July 2019 Molecular Cancer Research review article ([2][2]). We acknowledge that they have provided important novel insights, with their new article ([3][3]), into a topic discussed in our review regarding ...
Justin Korfhage, David B. Lombard
openaire   +2 more sources

Combined SHPments: An Effective Therapeutic Strategy for MPNST

Cancer Research, 2021
Abstract Targeted therapies have provided the foundation for many advances in the treatment options for patients with late-stage cancer, however, adaptive and compensatory responses frequently limit their efficacy. Rational combinations of targeted inhibitors are being actively tested in preclinical models to form the basis for more ...
Andrew E. Aplin, Claudia Capparelli
openaire   +3 more sources

Elevated Risk for MPNST in NF1 Microdeletion Patients [PDF]

The American Journal of Human Genetics, 2003
An NF1 microdeletion is the single most commonly reported mutation in individuals with neurofibromatosis type 1 (NF1). Individuals with an NF1 microdeletion have, as a group, more neurofibromas at a younger age than the group of all individuals with NF1. We report that NF1 microdeletion individuals additionally have a substantially higher lifetime risk
De Raedt, T., Brems, H., Wolkenstein, P., Vidaud, D., Pilotti, S., Perrone, F., Mautner, V., Frahm, S., Sciot, R., Legius, E.   +9 more
openaire   +2 more sources

MPNST after Radiosurgery: A Report and Review of the Literature [PDF]

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques, 2014
Objective:To report an unusual case of malignant degeneration in a ganglioneuroma of the jugular foramen post stereotactic radiosurgery as well as to systematically review the pertinent literature involving radiosurgery associated malignancies.Background:Ganglioneuromas are uncommon lesions of the peripheral nervous system and infrequently reported in ...
Albert, Tu, Roy, Ma, John, Maguire, Ryojo, Akagami   +3 more
openaire   +2 more sources

PRC2 loss drives MPNST metastasis and matrix remodeling

JCI Insight, 2022
The histone methyltransferase PRC2 plays a complex role in cancer. Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas with frequent loss-of-function mutations in PRC2 that are associated with poor outcome. Here, we identify a critical role for PRC2 loss in driving MPNST metastasis.
Qierra R. Brockman, Amanda Scherer, Gavin R. McGivney, Wade R. Gutierrez, Andrew P. Voigt, Alexandra L. Isaacson, Emily A. Laverty, Grace Roughton, Vickie Knepper-Adrian, Benjamin Darbro, Munir R. Tanas, Christopher S. Stipp, Rebecca D. Dodd   +12 more
openaire   +3 more sources

Analysis of H3k27me3 Expression in Malignant Peripheral Nerve Sheath Tumor (MPNST) and Other Spindle Cell Sarcoma Mimicking MPNST

Majalah Patologi Indonesia, 2023
Malignant Peripheral Nerve Sheath Tumor (MPNST) is a type of spindle cell sarcoma with approximately 5% of all sarcomas. Its diagnosis is challenging due to the absence of specific immunohistochemical markers. Recently, H3K27me3 was discovered as a potential specific immunohistochemical marker to differentiate MPNST from other sarcomas and distinguish ...
William Yordana, Evelina Evelina, Ria Kodariah   +2 more
openaire   +1 more source

Primary MPNST in Childhood- A Rare Case Report

JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH, 2014
Malignant peripheral nerve sheath tumour usually occurs between 20-50 years of age, comprising about 5-10% of soft tissue sarcomas. Only 1.7% of them have been reported to occur in children < 5 months of age according to the literature. Here, we are describing 18 mnth old male child presented with a swelling in the lower back.
Sandip Kudesia, Aparna Bhardwaj, Brijesh Thakur, Sanjeev Kishore, Neelima Bahal   +4 more
openaire   +3 more sources