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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
#1
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
#2
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
#3
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
#4
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...
March 7, 2018: Urologia Internationalis
https://www.readbyqxmd.com/read/29489027/extraskeletal-osteosarcoma-mdm2-and-h3k27me3-analysis-of-19-cases-suggest-disease-heterogeneity
#5
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. Dedifferentiated liposarcoma (DDLPS) and malignant peripheral nerve sheath tumor (MPNST) are the two most common types of sarcoma that can harbor 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, 2 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
#6
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...
January 31, 2018: Pathology, Research and Practice
https://www.readbyqxmd.com/read/29473773/mln8237-treatment-in-an-orthoxenograft-murine-model-for-malignant-peripheral-nerve-sheath-tumors
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/29409029/the-characteristics-of-76-atypical-neurofibromas-as-precursors-to-neurofibromatosis-1-associated-malignant-peripheral-nerve-sheath-tumors
#11
Christine S Higham, Eva Dombi, Aljosja Rogiers, Sucharita Bhaumik, Steven Pans, Steve E J Connor, Markku Miettinen, Raf Sciot, Roberto Tirabosco, Hilde Brems, Andrea Baldwin, Eric Legius, Brigitte C Widemann, Rosalie E Ferner
Background: Neurofibromatosis 1 (NF1) leads to the development of benign and malignant peripheral nerve sheath tumors (MPNST). MPNST have been described to develop in pre-existing benign plexiform neurofibromas (PN) and have a poor prognosis. Atypical neurofibromas (ANF) were recently described as precursor lesions for MPNST, making early detection and management of ANF a possible strategy to prevent MPNST. We aimed to clinically characterize ANF and identify management approaches. Methods: We analyzed clinical, imaging, and pathology findings of all patients with NF1 and ANF at three institutions...
February 2, 2018: Neuro-oncology
https://www.readbyqxmd.com/read/29408426/malignant-peripheral-nerve-sheath-tumor-of-the-trigeminal-nerve-involving-the-middle-and-posterior-cranial-fossa
#12
Aijun Liang, Bin Xi, Chaoyang Zhou, Yu Yang, Jianzhong Zhang, Shaogao Gui, Fanghua Xu, Dengfeng Wan
Although benign trigeminal schwannomas are uncommon, malignant peripheral nerve sheath tumors (MPNSTs) of the trigeminal nerve are extraordinarily rare. This study aimed to describe an exceedingly rare case of MPNST of the trigeminal nerve involving the middle and posterior cranial fossa. Only 20 similar cases of MPNSTs of the trigeminal nerve have been reported so far. The clinical characteristics and management of these extremely rare tumors have also been explored in this study.
February 2, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29369179/targeted-next-generation-sequencing-of-malignant-peripheral-nerve-sheath-tumor-of-the-pterygopalatine-fossa-with-intracranial-metastatic-recurrence
#13
Xinjie Bao, Xiangyi Kong, Chengxian Yang, Huanwen Wu, Wenbin Ma, Renzhi Wang
Malignant peripheral nerve sheath tumor (MPNST) is an uncommon neoplasm that rarely involves the head and neck region. Intracranial MPNSTs unrelated to cranial nerves are highly malignant tumors with poor overall survival, probably because of infiltrating growth into surrounding brain tissue. The pathogenesis of MPNST remains unclear. There are no conclusive explanations for the mechanisms underlying the initiation, progression, and metastasis of MPNST. In this paper, we describe a case of MPNST in the pterygopalatine fossa with intracranial metastatic recurrence and review related literatures...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29351992/involvement-of-aryl-hydrocarbon-receptor-in-myelination-and-in-human-nerve-sheath-tumorigenesis
#14
Ghjuvan'Ghjacumu Shackleford, Nirmal Kumar Sampathkumar, Mehdi Hichor, Laure Weill, Delphine Meffre, Ludmila Juricek, Ingrid Laurendeau, Aline Chevallier, Nicolas Ortonne, Frédérique Larousserie, Marc Herbin, Ivan Bièche, Xavier Coumoul, Mathieu Beraneck, Etienne-Emile Baulieu, Frédéric Charbonnier, Eric Pasmant, Charbel Massaad
Aryl hydrocarbon receptor (AHR) is a ligand-activated transcription factor involved in xenobiotic metabolism. Plexiform neurofibromas (PNFs) can transform into malignant peripheral nerve sheath tumors (MPNSTs) that are resistant to existing therapies. These tumors are primarily composed of Schwann cells. In addition to neurofibromatosis type 1 ( NF1 ) gene inactivation, further genetic lesions are required for malignant transformation. We have quantified the mRNA expression levels of AHR and its associated genes in 38 human samples...
February 6, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29332262/tumor-expression-of-survivin-p53-cyclin-d1-osteopontin-and-fibronectin-in-predicting-the-response-to-neo-adjuvant-chemotherapy-in-children-with-advanced-malignant-peripheral-nerve-sheath-tumor
#15
Gabrielle Karpinsky, Malgorzata A Krawczyk, Ewa Izycka-Swieszewska, Aleksandra Fatyga, Agnieszka Budka, Walentyna Balwierz, Grazyna Sobol, Beata Zalewska-Szewczyk, Magdalena Rychlowska-Pruszynska, Teresa Klepacka, Bozenna Dembowska-Baginska, Bernarda Kazanowska, Anna Gabrych, Ewa Bien
PURPOSE: Selected cell-cycle regulators and extracellular matrix proteins were found to play roles in malignant peripheral nerve sheath tumor (MPNST) biology. We aimed to analyze whether initial tumor tissue expressions of survivin, p53, cyclin D1, osteopontin (OPN) and fibronectin (FN) correlate with the response to neo-adjuvant CHT (naCHT) in children with advanced inoperable MPNST. METHODS: The study included 26 children with MPNST (M/F 14/12, median age 130 months) treated in Polish centers of pediatric oncology between 1992 and 2013...
March 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29304038/tumor-targeted-delivery-of-doxorubicin-in-malignant-peripheral-nerve-sheath-tumors
#16
A B Madhankumar, Oliver D Mrowczynski, Becky Slagle-Webb, Vagisha Ravi, Alexandre J Bourcier, Russell Payne, Kimberly S Harbaugh, Elias Rizk, James R Connor
Peripheral nerve sheath tumors are benign tumors that have the potential to transform into malignant peripheral nerve sheath tumors (MPNSTs). Interleukin-13 receptor alpha 2 (IL13Rα2) is a cancer associated receptor expressed in glioblastoma and other invasive cancers. We analyzed IL13Rα2 expression in several MPNST cell lines including the STS26T cell line, as well as in several peripheral nerve sheath tumors to utilize the IL13Rα2 receptor as a target for therapy. In our studies, we demonstrated the selective expression of IL13Rα2 in several peripheral nerve sheath tumors by immunohistochemistry (IHC) and immunoblots...
2018: PloS One
https://www.readbyqxmd.com/read/29285213/clinicopathological-features-and-prognosis-of-malignant-peripheral-nerve-sheath-tumor-a-retrospective-study-of-159-cases-from-1999-to-2016
#17
Zhennan Yuan, Libin Xu, Zhenguo Zhao, Songfeng Xu, Xinxin Zhang, Ting Liu, Shuguang Zhang, Shengji Yu
Objective: To investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors (MPNST). Results: A total of 159 patients with MPNST were enrolled in the study. The ratio of male to female was 1.04 to 1. The median age was 40 (range: 5-76) years at the time of diagnosis. The 3- and 5-year overall survival rates were 50.0% and 43.0%, respectively. The median follow-up period was 31.0 (range: 2.0-199.0) months. Multivariate analysis showed that AJCC stage and S-100 were independent factors affecting overall survival ( p < 0...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29200699/metastatic-epithelioid-malignant-peripheral-nerve-sheath-tumor-in-a-known-case-of-neurofibromatosis-1-cytomorphological-appearance-and-critical-analysis-of-immunohistochemistry
#18
Rakesh Kumar Gupta, Ravindra Kumar Saran, Deepak Ghuliani, Lalit Garg, Abhijit Das
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue tumors commonly arising from nerve roots in the extremities with a predilection in neurofibromatosis (NF) patients. MPNSTs are known to behave aggressively, with worse prognosis in nonresectable cases, and a 5-year survival of about 52% and 15% in resectable and unresectable cases, respectively. Although cytological diagnosis in a known case of NF-1 is possible, however, in the absence of any associated syndrome in a sporadic case, it is very challenging to accurately diagnose this tumor...
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29179472/bcrp-expression-in-schwannoma-plexiform-neurofibroma-and-mpnst
#19
Maurits de Vries, Olaf van Tellingen, Andel G L van der Mey, Antonius M G Bunt, Inge Briaire-de Bruijn, Pancras C W Hogendoorn
Background: peripheral nerve sheath tumors comprise a broad spectrum of neoplasms. Vestibular schwannomas and plexiform neurofibromas are symptomatic albeit benign, but a subset of the latter pre-malignant lesions will transform to malignant peripheral nerve sheath tumors (MPNST). Surgery and radiotherapy are the primary strategies to treat these tumors. Intrinsic resistance to drug therapy characterizes all three tumor subtypes. The breast cancer resistance protein BCRP is a transmembrane efflux transporter considered to play a key role in various biological barriers such as the blood brain barrier...
October 24, 2017: Oncotarget
https://www.readbyqxmd.com/read/29165710/risk-of-soft-tissue-sarcoma-among-69-460-five-year-survivors-of-childhood-cancer-in-europe
#20
Chloe J Bright, Mike M Hawkins, David L Winter, Daniela Alessi, Rodrigue S Allodji, Francesca Bagnasco, Edit Bárdi, Andrea Bautz, Julianne Byrne, Elizabeth A M Feijen, Miranda M Fidler, Stanislaw Garwicz, Desiree Grabow, Thorgerdur Gudmundsdottir, Joyeeta Guha, Nadia Haddy, Momcilo Jankovic, Peter Kaatsch, Melanie Kaiser, Claudia E Kuehni, Helena Linge, Hilde Øfstaas, Cecile M Ronckers, Roderick Skinner, Jop C Teepen, Monica Terenziani, Giao Vu-Bezin, Finn Wesenberg, Thomas Wiebe, Carlotta Sacerdote, Zsuzsanna Jakab, Riccardo Haupt, Päivi Lähteenmäki, Lorna Zadravec Zaletel, Rahel Kuonen, Jeanette F Winther, Florent de Vathaire, Leontien C Kremer, Lars Hjorth, Raoul C Reulen
Background: Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer. Methods: We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated. Results: Overall, 301 STS developed compared with 19 expected (SIR = 15...
November 20, 2017: Journal of the National Cancer Institute
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