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Malignant Peripheral Nerve Sheath Tumor

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https://www.readbyqxmd.com/read/28926683/salvage-rates-and-prognostic-factors-after-relapse-in-children-and-adolescents-with-malignant-peripheral-nerve-sheath-tumors
#1
Luca Bergamaschi, Gianni Bisogno, Carla Manzitti, Paolo D'Angelo, Giuseppe Maria Milano, Angela Scagnellato, Mirko Cappelletti, Stefano Chiaravalli, Patrizia Dall'Igna, Rita Alaggio, Antonio Ruggiero, Martina Di Martino, Maria Carmen Affinita, Marta Pierobon, Alberto Garaventa, Michela Casanova, Andrea Ferrari
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is one of the most common nonrhabdomyosarcoma soft tissue sarcomas encountered in pediatric age, and it is generally characterized by poor outcome, particularly for relapsing patients. MATERIALS AND METHODS: This study considered 73 patients <21 years of age with relapsing MPNST observed among 120 patients enrolled in Italian pediatric protocols from 1979 to 2004. With the aim of possibly establishing a risk-adapted stratification, patients' outcome was examined using univariate and multivariate analysis based on clinical features at onset, first-line treatments, clinical findings at the time of first relapse, and second-line treatments...
September 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28924675/malignant-triton-tumor-grand-round-presentation-of-a-rare-aggressive-case-thoracolumbar-spine-tumor
#2
Soufiane Ghailane, Sandra Fauquier, Sébastien Lepreux, Jean-Charles Le Huec
INTRODUCTION: We report a rare and aggressive case of malignant triton tumor (MTT) at the thoracolumbar junction with foraminal extension mistreated as schwannoma. MATERIALS AND METHODS: A 70-year-old man with a 2-year history of lower back pain and left L4 radiculopathy with no history of neurofibromatosis. RESULTS: Pre-operative MRI suggested a typical schwannoma. Upon complete marginal resection, histological findings revealed a MTT. The patient presented with a local and regional recurrence and died 10 months after surgery...
September 18, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28914875/-a-malignant-peripheral-nerve-sheath-tumor-developed-from-the-auditory-nerve-a-case-report-and-a-literature-review
#3
V N Shimanskiy, K V Shevchenko, M V Ryzhova, S V Tanyashin, D A Odamanov, V K Poshataev
We present a rare clinical case of a patient with a malignant peripheral nerve sheath tumor developed from the auditory nerve as well as a literature review, including 30 reported cases of this disease.
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28903419/development-of-zebrafish-medulloblastoma-like-pnet-model-by-talen-mediated-somatic-gene-inactivation
#4
Jaegal Shim, Jung-Hwa Choi, Moon-Hak Park, Hyena Kim, Jong Hwan Kim, Seon-Young Kim, Dongwan Hong, Sunshin Kim, Ji Eun Lee, Cheol-Hee Kim, Jeong-Soo Lee, Young-Ki Bae
Genetically engineered animal tumor models have traditionally been generated by the gain of single or multiple oncogenes or the loss of tumor suppressor genes; however, the development of live animal models has been difficult given that cancer phenotypes are generally induced by somatic mutation rather than by germline genetic inactivation. In this study, we developed somatically mutated tumor models using TALEN-mediated somatic gene inactivation of cdkn2a/b or rb1 tumor suppressor genes in zebrafish. One-cell stage injection of cdkn2a/b-TALEN mRNA resulted in malignant peripheral nerve sheath tumors with high frequency (about 39%) and early onset (about 35 weeks of age) in F0 tp53(e7/e7) mutant zebrafish...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28900340/primary-intraosseous-malignant-peripheral-nerve-sheath-tumor-of-metacarpal-bones-of-the-hand-in-a-patient-without-neurofibromatosis-1-report-of-a-rare-case
#5
Bharti Devnani, Ahitagni Biswas, Sameer Bakhshi, Shah Alam Khan, Asit Ranjan Mridha, Shipra Agarwal
Malignant peripheral nerve sheath tumor (MPNST) usually arises in peripheral nerve sheath cells. The intraosseous location of MPNST is rare. Mandible is the most common site of bony involvement. Involvement of bones of the hand is quite unusual. We report a case of MPNST involving metacarpal bones of the left hand treated with surgery followed by adjuvant radiation and chemotherapy and review the pertinent literature.
April 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28898989/role-of-histone-h3k27-trimethylation-loss-as-a-marker-for-malignant-peripheral-nerve-sheath-tumor-in-fine-needle-aspiration-and-small-biopsy-specimens
#6
Jeffrey K Mito, Xiaohua Qian, Leona A Doyle, Jason L Hornick, Vickie Y Jo
Objectives: Accurate diagnosis of malignant peripheral nerve sheath tumor (MPNST) is often challenging on fine-needle aspiration (FNA) or core needle biopsy. Recurrent mutations in EED and SUZ12, which encode subunits of polycomb repressive complex 2 (PRC2), have been identified in 70% to 92% of MPNSTs; PRC2 inactivation leads to loss of trimethylation of lysine 27 of histone H3 (H3K27me3). We evaluated the utility of H3K27me3 immunohistochemistry for distinguishing MPNST from its cytomorphologic mimics...
August 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28885122/clinicopathological-variables-of-sporadic-schwannomas-of-peripheral-nerve-in-291-patients-and-expression-of-biologically-relevant-markers
#7
Eric D Young, Davis Ingram, William Metcalf-Doetsch, Dilshad Khan, Ghadah Al Sannaa, Francois Le Loarer, Alexander J F Lazar, John Slopis, Keila E Torres, Dina Lev, Raphael E Pollock, Ian E McCutcheon
OBJECTIVE While sporadic peripheral schwannomas (SPSs) are generally well treated with surgery, their biology is not well understood. Consequently, treatment options are limited. The aim of this study was to provide a comprehensive description of SPS. The authors describe clinicopathological features and treatment outcomes of patients harboring these tumors, and they assess expression of biomarkers using a clinically annotated tissue microarray. Together, these data give new insight into the biology and management of SPS...
September 8, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28884110/malignant-triton-tumor-a-rare-cause-of-sciatic-pain-and-foot-drop
#8
Maribel R Gomes, Alexandre M P Sousa, Roberto J A Couto, Marco M B Oliveira, João L M Moura, Carlos A Vilela
Malignant peripheral nerve sheath tumors (MPNST) are very rare and are frequently localized in the buttocks, thigh, arm, or paraspinal region; one variant is the malignant Triton tumor, with rhabdomyosarcomatous differentiation. The authors present a challenging differential diagnosis of a sciatic pain and foot drop in a woman with history of lumbar disk herniation, which was found to be caused by a Triton tumor of the sciatic nerve. She underwent surgical excision, followed by radiation and chemotherapy. Malignant Triton tumor cases have rarely been described and reported in the literature...
June 2017: Revista Brasileira de Ortopedia
https://www.readbyqxmd.com/read/28876532/successful-treatment-with-doxorubicin-and-ifosfamide-for-mediastinal-malignant-peripheral-nerve-sheath-tumor-with-loss-of-h3k27me3-expression
#9
Noriko Seno, Toshirou Fukushima, Daisuke Gomi, Takashi Kobayashi, Nodoka Sekiguchi, Hidehiro Matsushita, Takesumi Ozawa, Yoshiko Tsukahara, Keiko Mamiya, Tomonobu Koizumi, Kenji Sano
Malignant peripheral nerve sheath tumor (MPNST) in the thorax is an extremely rare disease, and half of all cases of MPNST are associated with neurofibromatosis type I. Sporadic intrathoracic MPNST is difficult to diagnose and treat. Because of the rarity of intrathoracic MPNST, the optimal method of diagnosis and the efficacy of chemotherapy are unknown. Herein, we present a case of inoperable mediastinal MPNST, in which the diagnosis was immunohistochemically made by the loss of H3K27me3 expression in a transbronchial needle biopsy specimen...
September 6, 2017: Thoracic Cancer
https://www.readbyqxmd.com/read/28870819/-type-1-neurofibromatosis-onset-of-two-tumors-before-the-age-of-5years
#10
M Remillieux, C Durand, H Sartelet, C Piolat, E Bourgeois, P Pommier, F Hameury, K Dieterich, D Vidaud, C Perret
Neurofibromatosis type 1 (NF1) is a frequent autosomal dominant genetic disorder that predisposes to the development of benign and malignant tumors. Mutation of the NF1 gene affects the RAS-MAPK signaling pathway and leads to a dysfunction in cell proliferation and induces tumor development. Epidemiology of cancer in children with NF1 is very different from the general pediatric population, which requires regular and specific monitoring. Neurofibroma is the most frequent benign tumor. It can be very invalidating depending on the size and location of the tumor...
September 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28859862/the-promise-of-signal-transduction-in-genetically-driven-sarcomas-of-the-nerve
#11
REVIEW
AeRang Kim, Christine A Pratilas
Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome. Malignant peripheral nerve sheath tumors (MPNST) are aggressive soft tissue sarcomas arising from peripheral nerve sheaths, and the most commonly lethal feature associated with NF1. The hallmark of NF1 and NF1-related MPNST is the loss of neurofibromin expression. Loss of neurofibromin is considered a tumor-promoting event, and leads to constitutive activation of RAS and its downstream effectors. However, RAS activation alone is not sufficient for MPNST formation, and additional tumor suppressors and signaling pathways have been implicated in tumorigenesis of MPNST...
August 28, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28855982/peritoneal-metastases-of-rare-carcinomas-treated-with-cytoreductive-surgery-and-hipec-a-single-center-case-series
#12
Andreas Brandl, Christina Barbara Zielinski, Wieland Raue, Johann Pratschke, Beate Rau
INTRODUCTION: In selected cases, cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is an established treatment for patients suffering from peritoneal metastases from colorectal, ovarian, gastric or appendiceal origin. The effectiveness of this extensive has not been elucidated within other rare diseases by now. METHODS: We conducted a retrospective analysis of patients treated with CRS for peritoneal carcinomatosis during the period between July 2010 and September 2015...
October 2017: Annals of Medicine and Surgery
https://www.readbyqxmd.com/read/28846462/immunohistochemical-markers-for-prospective-studies-in-neurofibromatosis-1-porcine-models
#13
David K Meyerholz, Georgina K Ofori-Amanfo, Mariah R Leidinger, J Adam Goeken, Rajesh Khanna, Jessica C Sieren, Benjamin W Darbro, Dawn E Quelle, Jill M Weimer
Neurofibromatosis type 1 (NF1) is a common, cancer-predisposing disease caused by mutations in the NF1 tumor gene. Patients with NF1 have an increased risk for benign and malignant tumors of the nervous system (e.g., neurofibromas, malignant peripheral nerve sheath tumors, gliomas) and other tissues (e.g., leukemias, rhabdomyosarcoma, etc.) as well as increased susceptibility to learning disabilities, chronic pain/migraines, hypertension, pigmentary changes, and developmental lesions (e.g., tibial pseudoarthrosis)...
August 1, 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/28832074/programmed-death-ligand-1-pd-l1-expression-in-malignant-mesenchymal-tumors
#14
Kemal Kösemehmetoğlu, Ece Özoğul, Berrin Babaoğlu, Gaye Güler Tezel, Gökhan Gedikoğlu
OBJECTIVE: Programmed death ligand 1 (PD-L1) found on tumor cells has recently been reported to have a key role in the development and dissemination of many tumors, such as lung and breast carcinomas. In this study, we retrospectively analyzed PD-L1 expression among different types of sarcomas. MATERIAL AND METHOD: Tissue microarrays of 3-4 mm diameter were composed from paraffin blocks of 222 various sarcomas. Slides prepared from microarrays were stained for PD-L1 antibody (Cell Signaling, E1L3N®) using Leica Bond Autostainer...
2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28828137/radiologic-manifestation-of-the-malignant-peripheral-nerve-sheet-tumor-involving-the-brachial-plexus
#15
Shima Aran, Gloria Suarez Duran, Valeria Potigailo, Andrew E Kim
A 63-year-old African American female with history of bilateral breast cancer status after lumpectomy and radiation therapy presented with right hand, wrist, and arm pain. She was found to have a right axillary mass and a large lesion in the right brachial plexus. A biopsy of the brachial plexus mass came back as a malignant peripheral nerve sheath tumor. This case report illustrates the critical value of multiple imaging modalities in definitive diagnosis of this rare pathologic entity.
September 2017: Radiology case reports
https://www.readbyqxmd.com/read/28821978/assessment-of-paraspinal-neurogenic-tumors-with-diffusion-weighted-mr-imaging
#16
Ahmed Abdel Khalek Abdel Razek, Germeen Albair Ashmalla
PURPOSE: To assess paraspinal neurogenic tumors with diffusion-weighted MR imaging. METHODS: Retrospective analysis was done upon 34 patients with paraspinal neurogenic tumors that underwent diffusion-weighted MR imaging. The ADC values of the mediastinal neurogenic tumors were calculated and correlated with biopsy results. RESULTS: The ADC of benign paraspinal neurogenic tumors (1.5 ± 0.28 × 10(-3) mm(2)/s) was significantly higher (P = 0...
August 18, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28813519/expression-and-inhibition-of-brd4-ezh2-and-top2a-in-neurofibromas-and-malignant-peripheral-nerve-sheath-tumors
#17
Azadeh Amirnasr, Rob M Verdijk, Patricia F van Kuijk, Walter Taal, Stefan Sleijfer, Erik A C Wiemer
Malignant peripheral nerve sheath tumors (MPNST) are rare, highly aggressive sarcomas that can occur spontaneously or from pre-existing plexiform neurofibromas in neurofibromatosis type1 (NF1) patients. MPNSTs have high local recurrence rates, metastasize easily, are generally resistant to therapeutic intervention and frequently fatal for the patient. Novel targeted therapeutic strategies are urgently needed. Standard treatment for patients presenting with advanced disease is doxorubicin based chemotherapy which inhibits the actions of the enzyme topoisomerase IIα (TOP2A)...
2017: PloS One
https://www.readbyqxmd.com/read/28795017/malignant-renal-schwannoma-in-a-cat
#18
Monier Sharif, Adel Mohamed, Manfred Reinacher
A nine-year-old male European shorthair cat with rapidly enlarging mass at the left kidney doubted to be malignant was presented. The purpose of this study is to present the clinical, radiological and pathological findings of a primary renal tumor in the cat. Grossly, the mass mostly encapsulated the kidney. Histologically, excisional biopsy showed worrying histological features. A sarcoma-like tumor composed mainly of neoplastic spindle-shaped cells. Neoplastic nodules of aggregations of fusiform cells arranged in multidirectional bundles...
2017: Open veterinary journal
https://www.readbyqxmd.com/read/28776579/significance-of-h3k27me3-loss-in-the-diagnosis-of-malignant-peripheral-nerve-sheath-tumors
#19
Melike Pekmezci, Areli K Cuevas-Ocampo, Arie Perry, Andrew E Horvai
The diagnosis of malignant peripheral nerve sheath tumors can be challenging and other spindle cell sarcomas commonly enter in the differential diagnosis. Loss of trimethylation at lysine 27 of histone-H3 (H3K27me3) by immunohistochemistry was recently described in malignant peripheral nerve sheath tumors. However, its specificity remains controversial. We therefore studied 82 synovial sarcomas, 39 malignant peripheral nerve sheath tumors, and 10 fibrosarcomatous dermatofibrosarcoma protuberans for H3K27me3 loss by immunohistochemistry...
August 4, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28762137/a-clinicopathologic-study-of-head-and-neck-malignant-peripheral-nerve-sheath-tumors
#20
Adepitan A Owosho, Cherry L Estilo, Joseph M Huryn, Ping Chi, Cristina R Antonescu
Head and neck high grade malignant peripheral nerve sheath tumors (HN-MPNSTs) are rare highly aggressive soft tissue sarcomas that show overlapping morphologic and immunophenotypic features with melanoma and other high grade sarcomas, resulting in diagnostic challenges, particularly in sporadic settings. Recent discoveries have implicated loss of function mutations in the polycomb repressive complex 2 (PRC2) components, including EED or SUZ12 genes, as one of the leading pathogenetic mechanisms in high grade MPNST...
July 31, 2017: Head and Neck Pathology
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