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Peripheral nerve sheath tumor

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https://www.readbyqxmd.com/read/29683959/malignant-peripheral-nerve-sheath-tumors-in-neurofibromatosis-impact-of-family-history
#1
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
#2
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/29675081/intra-fourth-ventricular-schwannoma-in-pediatric-age-group-report-of-second-case-in-the-western-literature-with-review-of-literature
#3
Luis Rafael Moscote-Salazar, Guru Dutta Satyarthee, Hector Farid-Escorcia, Willem Guillermo Calderon-Miranda, Huber Said Padilla-Zambrano, Angel Lee, Alfonso Pacheco-Hernandez, Amit Agrawal
Schwannoma originates from the myelin sheath of peripheral nerves. It accounts for about 8% of all intracranial tumors. Commonly, schwannoma is located in the extra-axial locations; intra-axial schwannomas are extremely uncommon. The schwannoma arising from fourth ventricle is extremely uncommon and authors in a detailed PubMed and MEDLINE search could find only seven cases reported in the literature in the form of isolated cases report, who were managed surgically, with only one being a pediatric case. Authors report second case of intra-fourth ventricular schwannoma occurring in the pediatric age group...
October 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29673820/extra-osseous-ewing-s-sarcoma-of-sciatic-nerve-masquerading-as-benign-nerve-sheath-tumor-and-presented-as-lumbar-radiculopathy
#4
Jung Soo Bang, Nitin Adsul, Jae-Hyeon Lim, Il-Tae Jang
BACKGROUND: Extraosseous Ewing Sarcoma (EES) arising from peripheral nerves is extremely rare with only 11 cases, mentioned in the literature. It poses a diagnostic challenge to the clinicians due to its nonspecific presentation and rare occurrence. CASE DESCRIPTION: A 56-year-old woman came with a history of low back pain and intractable right leg pain. Physical examination revealed the normal motor power of both lower limbs with decreased sensation along the lateral aspect of the foot with decreased ankle reflex...
April 16, 2018: World Neurosurgery
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
#5
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/29662612/exploiting-mitochondrial-and-metabolic-homeostasis-as-a-vulnerability-in-nf1-deficient-cells
#6
Robert J Allaway, Matthew D Wood, Sondra L Downey, Stephanie J Bouley, Nicole A Traphagen, Jason D Wells, Jaya Batra, Sir Norman Melancon, Carol Ringelberg, William Seibel, Nancy Ratner, Yolanda Sanchez
Neurofibromatosis type 1 is a disease caused by mutation of neurofibromin 1 ( NF1 ), loss of which results in hyperactive Ras signaling and a concomitant increase in cell proliferation and survival. Patients with neurofibromatosis type 1 frequently develop tumors such as plexiform neurofibromas and malignant peripheral nerve sheath tumors. Mutation of NF1 or loss of the NF1 protein is also observed in glioblastoma, lung adenocarcinoma, and ovarian cancer among other sporadic cancers. A therapy that selectively targets NF1 deficient tumors would substantially advance our ability to treat these malignancies...
March 23, 2018: Oncotarget
https://www.readbyqxmd.com/read/29661713/review-with-novel-markers-facilitates-precise-categorization-of-41-cases-of-diagnostically-challenging-undifferentiated-small-round-cell-tumors-a-clinicopathologic-immunophenotypic-and-molecular-analysis
#7
Isidro Machado, Akihiko Yoshida, María Gema Nieto Morales, Lucas Faria Abrahão-Machado, Samuel Navarro, Julia Cruz, Javier Lavernia, Antonina Parafioriti, Piero Picci, Antonio Llombart-Bosch
BACKGROUND: Despite extensive immunohistochemical (IHC) and molecular studies combined with morphologic findings, a group of round/ovoid cell tumors histologically similar to Ewing sarcomas (ES) but lacking EWSR1-rearrangements may remain unclassifiable. DESIGN: We retrospectively analyzed 41 Ewing-like tumors (formalin-fixed, paraffin-embedded) previously determined as negative or non-informative for EWSR1-rearrangements by FISH and/or RT-PCR. A new histopathology revision and additional IHC and molecular analyses were carried out in order to investigate whether additional IHC and/or molecular testing in combination with the morphological findings may help in reaching a definitive diagnosis...
November 29, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29656234/intramasseteric-schwanoma-mimicking-an-isolated-cheek-mass-case-report-and-review-of-literature
#8
Nazir El Khatib, Antoine Nehme, Selim Nasser, Nabil Moukarzel, Houssam Khodor Abtar
INTRODUCTION: Schwannoma is a benign well circumscribed tumor of the nerve sheath and it is mostly localized in the head and neck. Intramasseteric schwannoma represents a very rare entity and a few cases have been described in the literature. PRESENTATION OF CASE: We present a case of an isolated, asymptomatic and slowly progressive right cheek tumor in a middle aged man. Although multiple investigations, including neck scanner and fine needle aspiration, were done, the diagnosis was obscure and difficult before definite surgical resection...
April 7, 2018: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29622111/imaging-of-benign-neoplastic-and-nonneoplastic-salivary-gland-tumors
#9
REVIEW
Daniel Thomas Ginat
This article reviews the multimodality diagnostic imaging features of benign neoplastic and nonneoplastic tumors associated with the major salivary glands. Examples of neoplastic conditions that are depicted and discussed include pleomorphic adenoma, Warthin tumor, oncocytoma, peripheral nerve sheath tumors, lipoma, and hemangiomas or hemangioendotheliomas. Examples of nonneoplastic conditions that are depicted and discussed include ranulas, benign lymphoepithelial lesions, Kimura disease, and vascular malformations...
May 2018: Neuroimaging Clinics of North America
https://www.readbyqxmd.com/read/29616298/intracranial-malignant-peripheral-nerve-sheath-tumor-variant-an-unusual-neurovascular-phenotype-sarcoma-case-invading-through-the-petrous-bone
#10
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/29606945/a-case-of-vulvar-schwannoma-mimicking-epidermoid-cyst
#11
Jingwen Tan, Jia Chen, Lianjuan Yang
Schwannoma is a generally benign tumor of the peripheral nerve sheath arising from Schwann cells. Most schwannomas are found on the head and neck. Here, we describe a rare case of benign vulvar schwannoma located at the labium majus which was similar to an epidermoid cyst in clinical appearance.
January 2018: Case Reports in Dermatology
https://www.readbyqxmd.com/read/29606723/retrobulbar-malignant-peripheral-nerve-sheath-tumor-in-a-golden-retriever-dog-a-challenging-diagnosis
#12
Cécile Briffod, Pierre Hélie, Julie De Lasalle, Louis-Philippe de Lorimier, Alicia R Moreau, Derron A Alves, Maria Vanore
A 9-year-old golden retriever dog was diagnosed with a left retrobulbar mass. Fine-needle aspirations and incisional biopsies resulted in discordant diagnoses: myxosarcoma/myxoma or rhadomyosarcoma, respectively. Immunohistochemistry following exenteration allowed definitive diagnosis of malignant peripheral nerve sheath tumor with fibromyxomatous differentiation. Fifteen weeks after surgery, an aggressive recurrence resulted in euthanasia.
April 2018: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/29603044/genipin-cross-linked-chitosan-nerve-conduits-containing-tnf-%C3%AE-inhibitors-for-peripheral-nerve-repair
#13
Li Zhang, Weijia Zhao, Changmei Niu, Yujie Zhou, Haiyan Shi, Yalin Wang, Yumin Yang, Xin Tang
Tissue engineered nerve grafts (TENGs) are considered a promising alternative to autologous nerve grafting, which is considered the "gold standard" clinical strategy for peripheral nerve repair. Here, we immobilized tumor necrosis factor-α (TNF-α) inhibitors onto a nerve conduit, which was introduced into a chitosan (CS) matrix scaffold utilizing genipin (GP) as the crosslinking agent, to fabricate CS-GP-TNF-α inhibitor nerve conduits. The in vitro release kinetics of TNF-α inhibitors from the CS-GP-TNF-α inhibitor nerve conduits were investigated using high-performance liquid chromatography...
March 30, 2018: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/29596596/%C3%AE-iii-spectrin-immunohistochemistry-as-a-potential-diagnostic-tool-with-high-sensitivity-for-malignant-peripheral-nerve-sheath-tumors
#14
Angela C Hirbe, Xiaochun Zhang, Sonika Dahiya, Abigail Godec, John Chrisinger, Yu Tao, Jingqin Luo, David H Gutmann
No abstract text is available yet for this article.
March 27, 2018: Neuro-oncology
https://www.readbyqxmd.com/read/29543881/correction-hypoxia-inducible-factor-1-alpha-is-a-poor-prognostic-factor-and-potential-therapeutic-target-in-malignant-peripheral-nerve-sheath-tumor
#15
Suguru Fukushima, Makoto Endo, Yoshihiro Matsumoto, Jun-Ichi Fukushi, Tomoya Matsunobu, Ken-Ichi Kawaguchi, Nokitaka Setsu, Keiichiro IIda, Nobuhiko Yokoyama, Makoto Nakagawa, Kenichiro Yahiro, Yoshinao Oda, Yukihide Iwamoto, Yasuharu Nakashima
[This corrects the article DOI: 10.1371/journal.pone.0178064.].
2018: PloS One
https://www.readbyqxmd.com/read/29543677/fluorescent-in-situ-hybridization-for-tp53-in-the-diagnosis-of-pediatric-osteogenic-sarcoma
#16
Paula Marrano, Mary Shago, Gino R Somers, Paul S Thorner
Osteogenic sarcoma (OS) is the most common malignant bone tumor in children and adolescents. Despite advances in molecular genetic characterization of pediatric and adult tumors, the diagnosis of OS still depends almost entirely on light microscopy. The lack of consistent genetic changes in OS has greatly hindered the development of any diagnostic molecular test. Recently, whole-genome sequencing has shown that ~50% of cases of OS have a translocation involving the TP53 gene with breakpoints confined to the first intron...
March 14, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29518229/case-report-metastasis-of-a-trigeminal-malignant-peripheral-nerve-sheath-tumor-to-the-corpus-callosum
#17
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
#18
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/29512865/malignant-progression-of-a-peripheral-nerve-sheath-tumor-in-the-setting-of-rhabdoid-tumor-predisposition-syndrome
#19
Santhosh A Upadhyaya, Rose B McGee, Breelyn A Wilky, Alberto Broniscer
Malignant progression of a benign or low-grade tumor in individuals with germline alteration of SMARCB1 gene is not well characterized. In a family in which two carrier children had germline SMARCB1 mutations and atypical teratoid rhabdoid tumor, we report malignant progression of a nerve sheath tumor over a 7-year period in an affected adult family member. Prompt identification of the germline SMARCB1 alteration and the resultant rhabdoid tumor predisposition syndrome can help guide genetic counseling and surveillance in affected family members...
March 7, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29492148/case-of-an-intracranial-malignant-peripheral-nerve-sheath-tumor-in-the-setting-of-pacer-dependent-heart-block
#20
Jonathan Chica, Isaac Yepes, S Shelby Burks, Ricardo Komotar, Roger Carrillo
Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are an extremely rare entity with only a handful of cases reported in the literature. MPNSTs typically occur in the extremities and the trunk. The treatment algorithm includes, when possible, gross-total resection as these tumors are extremely aggressive. When these tumors occur intracranially, they are termed malignant intracerebral nerve sheath tumors. The diagnosis hinges on immunohistochemistry and pathological features and often the diagnosis can be delayed for this reason...
January 2018: Asian Journal of Neurosurgery
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