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https://www.readbyqxmd.com/read/29909207/low-grade-malignant-peripheral-nerve-sheath-tumor-mimicking-a-schwannoma-the-role-and-importance-of-trimethylated-h3k27m-staining
#1
Hannah Gilder, Ross C Puffer, Robert J Spinner, Aditya Raghunathan, Mohamad Bydon
It is important to differentiate low grade malignant peripheral nerve sheath tumors (MPNSTs) from benign nerve sheath tumors as MPNSTs may require a more aggressive treatment strategy during and after initial resection. Loss of expression of the trimethyl Histone H3 at the Lys27 position (H3K27-me3) has recently been described in MPNSTs, and may help distinguish this tumor from pathologic mimics. A 43-year-old woman presented with symptoms of radiculopathy and a history of pelvic radiation for cervical cancer 7 years prior...
June 14, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29900032/malignant-peripheral-nerve-sheath-tumor-of-the-scalp-two-rare-case-reports
#2
Muhammad Firdaus, Arwinder Singh Gill, Dewi Aisiyah Mukarramah, Rini Andriani, Lenny Sari, Dian Cahyanti, Ahmad Faried
Background: Malignant peripheral nerve sheath tumors (MPNSTs) constitute a group of soft tissue neoplasm with neuroectodermal origin. Most cases are small at presentation and only some have been described reaching giant dimensions. Case Description: We report two cases that were diagnosed and treated as giant MPNST of the scalp. Both patients had extensive lesion on the head with intracranial infiltration. Microsurgical resection was indicated and a vascularized free flap was used to cover the defect...
2018: Surgical Neurology International
https://www.readbyqxmd.com/read/29883816/case-series-malignant-peripheral-nerve-sheath-tumor-in-the-course-of-the-mandibular-nerve
#3
Probst Monika, Koerdt Steffen, Ritschl Lucas Maximilian, Bissinger Oliver, Liesche Friederike, Gempt Jens, Meyer Bernhard, Burian Egon, Lummel Nina, Kolk Andreas
OBJECTIVE: Malignant peripheral nerve sheath tumors (MPNST) are infiltrating, aggressive tumors belonging to the group of soft tissue sarcomas. This report refers to three patients with a tumorous swelling in the entire inferior alveolar nerve (IAN) with similar disease courses suspect for a MPNST, which is particularly rare in the trigeminal nerve. METHODS: Diagnostic tools, surgical proceedings and reconstructive procedures were highlighted. Three male patients (58-68 years), who suffered from numbness, pain and mild swelling in the sensation area served by the mental nerve presented at the department of oral and maxillofacial surgery and underwent diagnostic workup including CT, MRI, F18-PET-CT, as well as a biopsy of the clinical visible tumor mass with histopathological and molecular pathological analysis...
June 5, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29805750/identification-of-reck-as-an-evolutionarily-conserved-tumor-suppressor-gene-for-zebrafish-malignant-peripheral-nerve-sheath-tumors
#4
Rashmi Kumari, Martin R Silic, Yava L Jones-Hall, Alexandra Nin-Velez, Jer-Yen Yang, Suresh K Mittal, GuangJun Zhang
Malignant peripheral nerve sheath tumors (MPNSTs) are a type of sarcoma with poor prognosis due to their complex genetic changes, invasive growth, and insensitivity to chemo- and radiotherapies. One of the most frequently lost chromosome arms in human MPNSTs is chromosome 9p. However, the cancer driver genes located on it remain largely unknown, except the tumor suppressor gene, p16 (INK4)/CDKN2A . Previously, we identified RECK as a tumor suppressor gene candidate on chromosome 9p using zebrafish-human comparative oncogenomics...
May 4, 2018: Oncotarget
https://www.readbyqxmd.com/read/29796169/aberrant-atrx-protein-expression-is-associated-with-poor-overall-survival-in-nf1-mpnst
#5
Hsiang-Chih Lu, Vanessa Eulo, Anthony J Apicelli, Melike Pekmezci, Yu Tao, Jingqin Luo, Angela C Hirbe, Sonika Dahiya
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are aggressive soft tissue sarcomas that can occur sporadically or in the setting of the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome. These tumors carry a dismal overall survival. Previous work in our lab had identified ATRX chromatin remodeler ( ATRX ), previously termed, Alpha Thalassemia/Mental Retardation Syndrome X Linked as a gene mutated in a subset of MPNSTs. Given the great need for novel biomarkers and therapeutic targets for MPNSTs, we sought to determine the expression of ATRX in a larger subset of sporadic and NF1 associated MPNSTs (NF1-MPNSTs)...
May 1, 2018: Oncotarget
https://www.readbyqxmd.com/read/29762158/recent-advances-in-the-diagnosis-and-pathogenesis-of-neurofibromatosis-type-1-nf1-associated-peripheral-nervous-system-neoplasms
#6
Jody F Longo, Shannon M Weber, Brittany P Turner-Ivey, Steven L Carroll
The diagnosis of a neurofibroma or a malignant peripheral nerve sheath tumor (MPNST) often raises the question of whether the patient has the genetic disorder neurofibromatosis type 1 (NF1) as well as how this will impact the patient's outcome, what their risk is for developing additional neoplasms and whether treatment options differ for NF1-associated and sporadic peripheral nerve sheath tumors. Establishing a diagnosis of NF1 is challenging as this disorder has numerous neoplastic and non-neoplastic manifestations which are variably present in individual patients...
May 4, 2018: Advances in Anatomic Pathology
https://www.readbyqxmd.com/read/29720369/genomic-status-of-met-potentiates-sensitivity-to-met-and-mek-inhibition-in-nf1-related-malignant-peripheral-nerve-sheath-tumors
#7
Jacqueline D Peacock, Matthew G Pridgeon, Elizabeth A Tovar, Curt J Essenburg, Megan J Bowman, Zachary B Madaj, Julie Koeman, Elissa A Boguslawski, Jamie Grit, Rebecca D Dodd, Vadim Khachaturov, Diana M Cardona, Mark Chen, David G Kirsch, Flavio Maina, Rosanna Dono, Mary E Winn, Carrie R Graveel, Matthew R Steensma
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are highly resistant sarcomas that occur in up to 13% of individuals with Neurofibromatosis Type 1 (NF1). Genomic analysis of longitudinally collected tumor samples in a case of MPNST disease progression revealed early hemizygous microdeletions in NF1 and TP53, with progressive amplifications of MET, HGF, and EGFR. To examine the role of MET in MPNST progression, we developed mice with enhanced MET expression and Nf1 ablation (Nf1fl/KO;lox-stop-loxMETtg/+;Plp-creERTtg/+; referred to as NF1 MET)...
May 2, 2018: Cancer Research
https://www.readbyqxmd.com/read/29685535/imaging-cellularity-in-benign-and-malignant-peripheral-nerve-sheath-tumors-utility-of-the-target-sign-by-diffusion-weighted-imaging
#8
Shivani Ahlawat, Laura M Fayad
OBJECTIVE: To determine the utility of "target sign" on diffusion weighted imaging (DWI) and apparent diffusion coefficient (ADC) mapping for peripheral nerve sheath tumor (PNST) characterization. MATERIALS AND METHODS: This IRB-approved, HIPAA-compliant study retrospectively reviewed the MR imaging (comprised of T2- FS, DWI (b-values 50, 400, 800 s/mm2 and ADC mapping), and static contrast-enhanced (CE) T1-W imaging) of 42 patients (mean age: 40 years (range 8-68 years), 48% (20/42) females) with 15 malignant PNSTs (MPNSTs) and 33 benign PNSTs (BPNSTs)...
May 2018: European Journal of Radiology
https://www.readbyqxmd.com/read/29683959/malignant-peripheral-nerve-sheath-tumors-in-neurofibromatosis-impact-of-family-history
#9
Fatema Malbari, Menachem Spira, Pamela B Knight, Chong Zhu, Michael Roth, Jonathan Gill, Rick Abbott, Adam S Levy
OBJECTIVE: The main objective of this study was to determine if family history of malignant peripheral nerve sheath tumor (MPNST) increases risk of developing an MPNST in patients with neurofibromatosis-1 (NF-1). MATERIALS AND METHODS: Individuals with NF-1 registered with the Children's Tumor Foundation's Neurofibromatosis Registry were emailed an anonymous 15-minute survey with regard to personal and family history of NF-1, MPNST, ages of onset, and symptomatology...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29681099/2016-children-s-tumor-foundation-conference-on-neurofibromatosis-type-1-neurofibromatosis-type-2-and-schwannomatosis
#10
Michael J Fisher, Allan J Belzberg, Peter de Blank, Thomas De Raedt, Florent Elefteriou, Rosalie E Ferner, Marco Giovannini, Gordon J Harris, Michel Kalamarides, Matthias A Karajannis, AeRang Kim, Conxi Lázaro, Lu Q Le, Wei Li, Robert Listernick, Staci Martin, Helen Morrison, Eric Pasmant, Nancy Ratner, Elisabeth Schorry, Nicole J Ullrich, David Viskochil, Brian Weiss, Brigitte C Widemann, Yuan Zhu, Annette Bakker, Eduard Serra
Organized and hosted by the Children's Tumor Foundation (CTF), the Neurofibromatosis (NF) conference is the premier annual gathering for clinicians and researchers interested in neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN). The 2016 edition constituted a blend of clinical and basic aspects of NF research that helped in clarifying different advances in the field. The incorporation of next generation sequencing is changing the way genetic diagnostics is performed for NF and related disorders, providing solutions to problems like genetic heterogeneity, overlapping clinical manifestations, or the presence of mosaicism...
May 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29663170/racial-ethnic-disparities-and-incidence-of-malignant-peripheral-nerve-sheath-tumors-results-from-the-surveillance-epidemiology-and-end-results-program-2000-2014
#11
Erin C Peckham-Gregory, Roberto E Montenegro, David A Stevenson, David H Viskochil, Michael E Scheurer, Philip J Lupo, Joshua D Schiffman
BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors, generally high-grade, and comprise ~ 5-10% of soft tissue sarcomas. Over two-thirds of MPNSTs metastasize, and upwards of 40% clinically recur. Etiologic risk factors for MPNSTs are historically understudied. There is evidence to suggest MPNST incidence differs across racial/ethnic groups in pediatric populations. Therefore, we sought to estimate differences in MPNST incidence by race/ethnicity among all ages in the United States...
April 16, 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29616298/intracranial-malignant-peripheral-nerve-sheath-tumor-variant-an-unusual-neurovascular-phenotype-sarcoma-case-invading-through-the-petrous-bone
#12
Oliver D Mrowczynski, Robert J Greiner, Malika Kapadia, Julie C Fanburg-Smith, Mark R Iantosca, Elias B Rizk
INTRODUCTION: Intracranial malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. Previously reported cases of intracranial MPNST have been associated with development within a prominent cranial nerve. METHODS: This is the first report of an MPNST with both nerve sheath and vascular phenotype that follows the neurovascular bundle, without arising in a major cranial nerve or in the setting of neurofibromatosis type 1 (NF1). RESULTS: The patient is a 14-year-old boy with a history of worsening headaches for the past several months, left-sided hearing loss, nausea, vomiting, and vertigo...
April 3, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29518229/case-report-metastasis-of-a-trigeminal-malignant-peripheral-nerve-sheath-tumor-to-the-corpus-callosum
#13
Max Shutran, David Mosbach, Zachary Tataryn, Knarik Arkun, Julian K Wu
BACKGROUND AND IMPORTANCE: Malignant peripheral nerve sheath tumors (MPNST) are relatively rare tumors of peripheral nerves that are notable for their locally aggressive nature, ability to metastasize, poor prognosis, and association with Neurofibromatosis type I. We present the case of a patient with a trigeminal nerve MPNST who developed an unusual metastasis to the corpus callosum, in the absence of any other central nervous system or systemic metastatic disease. We review the pathology and presentation of MPNST...
March 5, 2018: Neurosurgery
https://www.readbyqxmd.com/read/29514154/bladder-dysfunction-in-children-with-neurofibromatosis-type-i-report-of-four-cases-and-review-of-the-literature
#14
Aurore Bouty, Eric Dobremez, Luke Harper, Jérôme Harambat, Cécile Bouteiller, Brigitte Zaghet, Pierre Wolkenstein, Stéphane Ducassou, Yan Lefevre
AIM: Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disorder. Malignant transformation into malignant peripheral nerve sheath tumors (MPNST) can occur. However, urinary tract involvement is rare. We report 4 cases of NF1 with bladder dysfunction. METHODS: A retrospective single center analysis of 4 patients was conducted over a 17-year period, focusing on urinary tract involvement. RESULTS: NF1 was diagnosed at a median of 16...
2018: Urologia Internationalis
https://www.readbyqxmd.com/read/29489027/extraskeletal-osteosarcoma-mdm2-and-h3k27me3-analysis-of-19-cases-suggest-disease-heterogeneity
#15
Naohiro Makise, Masaya Sekimizu, Takashi Kubo, Susumu Wakai, Shun-Ichi Watanabe, Tomoyasu Kato, Takayuki Kinoshita, Nobuyoshi Hiraoka, Masashi Fukayama, Akira Kawai, Hitoshi Ichikawa, Akihiko Yoshida
AIMS: Extraskeletal osteosarcoma (ESOS) is a sarcoma in the non-skeletal tissue that directly produces neoplastic osteoid or bone. De-differentiated liposarcoma (DDLPS) and malignant peripheral nerve sheath tumour (MPNST) are the two most common types of sarcoma that can harbour heterologous osteosarcomatous differentiation. We aimed to determine the potential relationship of ESOS to DDLPS and MPNST. METHODS AND RESULTS: We investigated MDM2 and H3K27me3 status in 19 cases of ESOS, two of which contained a low-grade component...
February 28, 2018: Histopathology
https://www.readbyqxmd.com/read/29482987/immunohistochemical-evaluation-of-h3k27-trimethylation-in-malignant-peripheral-nerve-sheath-tumors
#16
Hiroshi Otsuka, Kenichi Kohashi, Masato Yoshimoto, Shin Ishihara, Yu Toda, Yuichi Yamada, Hidetaka Yamamoto, Yasuharu Nakashima, Yoshinao Oda
The histological definitive diagnosis of malignant peripheral nerve sheath tumor (MPNST) is quite difficult because the morphological features are not specific and no useful immunohistochemical marker has been identified. Loss-of-function mutations in EED or SUZ12, which encode the core subunit of polycomb repressive complex 2 (PRC2), were reported in MPNSTs, and the mutations were shown to cause inactivation of PRC2, leading to loss of trimethylation of histone H3 at lysine 27 (H3K27me3). Immunohistochemistry of H3K27me3 is expected to be a specific marker for MPNSTs...
March 2018: Pathology, Research and Practice
https://www.readbyqxmd.com/read/29473773/mln8237-treatment-in-an-orthoxenograft-murine-model-for-malignant-peripheral-nerve-sheath-tumors
#17
Russell Payne, Oliver D Mrowczynski, Becky Slagle-Webb, Alexandre Bourcier, Christine Mau, Dawit Aregawi, Achuthamangalam B Madhankumar, Sang Y Lee, Kimberly Harbaugh, James Connor, Elias B Rizk
OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas arising from peripheral nerves. MPNSTs have increased expression of the oncogene aurora kinase A, leading to enhanced cellular proliferation. This makes them extremely aggressive with high potential for metastasis and a devastating prognosis; 5-year survival estimates range from a dismal 15% to 60%. MPNSTs are currently treated with resection (sometimes requiring limb amputation) in combination with chemoradiation, both of which demonstrate limited effectiveness...
February 23, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29472158/-dumbbell-malignant-dorsal-schwannoma-embolized-and-operated-by-single-posterior-approach
#18
Gorka Zabalo, Daniel de Frutos, Juan Carlos García, Rodrigo Ortega, Juan José Guelbenzu, Idoya Zazpe
We report a case of a 41 years old patient complaining of chronic dorsalgia. MRI showed a well defined intradural extramedular dumbbell-shaped lesion, associated to a left paravertebral tumor at D5-D6 level. The tumor was embolizated prior to surgery. Following she underwent a D4-D6 laminotomy, left D5-D6 costotransversectomy and resection of the intracanal extradural part of the lesion with section of the left D5 nerve. Posteriorly, complete resection of the extracanal portion of the tumor was performed by a postero-lateral approach...
February 19, 2018: Neurocirugía
https://www.readbyqxmd.com/read/29438698/programming-of-schwann-cells-by-lats1-2-taz-yap-signaling-drives-malignant-peripheral-nerve-sheath-tumorigenesis
#19
Lai Man Natalie Wu, Yaqi Deng, Jincheng Wang, Chuntao Zhao, Jiajia Wang, Rohit Rao, Lingli Xu, Wenhao Zhou, Kwangmin Choi, Tilat A Rizvi, Marc Remke, Joshua B Rubin, Randy L Johnson, Thomas J Carroll, Anat O Stemmer-Rachamimov, Jianqiang Wu, Yi Zheng, Mei Xin, Nancy Ratner, Q Richard Lu
Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive Schwann cell (SC)-lineage-derived sarcomas. Molecular events driving SC-to-MPNST transformation are incompletely understood. Here, we show that human MPNSTs exhibit elevated HIPPO-TAZ/YAP expression, and that TAZ/YAP hyperactivity in SCs caused by Lats1/2 loss potently induces high-grade nerve-associated tumors with full penetrance. Lats1/2 deficiency reprograms SCs to a cancerous, progenitor-like phenotype and promotes hyperproliferation...
February 12, 2018: Cancer Cell
https://www.readbyqxmd.com/read/29438691/hippo-stampede-in-nerve-sheath-tumors
#20
M Laura Feltri, Yannick Poitelon
Current therapies for malignant peripheral nerve sheath tumors (MPNSTs) are ineffective. The study by Wu et al. in this issue of Cancer Cell provides evidence that the HIPPO pathway is overactive in human MPNSTs and that combined modulation of LATS1/2-YAP/TAZ and PDGFR signaling in Schwann cells reduces MPNST growth.
February 12, 2018: Cancer Cell
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