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Peripheral nerve sheath tumor

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https://www.readbyqxmd.com/read/29449010/a-brief-report-of-plexiform-neurofibroma
#1
Mahdi Khajavi Md, Shahrokh Khoshsirat Md, Lida Ahangarnazari Md, Niloofar Majdinasab Md
Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF). It develops as a result of proliferation in all parts of peripheral nervous system and can cause the functional damage, deformities, pain, considerable mortality, and morbidity and even the increasing risk of malignant transformation in some critical cases. Currently, the surgical intervention is the treatment of choice for PNF patients, which due to the tumor invasion, massive growth, and the chance of postoperative regrowth is not possible...
January 10, 2018: Current Problems in Cancer
https://www.readbyqxmd.com/read/29440792/ancient-schwannoma-of-gingiva-a-rare-case-report
#2
Manas Bajpai, Nilesh Pardhe
Oral schwannomas (OSs) are rare benign tumors of oral cavity derived from peripheral nerve sheath, composed of proliferation of schwann cells in a characteristic pattern. AS are long-standing lesions which exhibit degenerative changes and atypia due to which they could be mistaken for malignancy. The mean age of occurrence of AS in oral cavity is 43 years, and no case of oral AS has been reported in a pediatric patient so far. Here, we present a rare case of AS of oral cavity in a 10-year-old male child.
May 2017: Journal of Indian Society of Periodontology
https://www.readbyqxmd.com/read/29438698/programming-of-schwann-cells-by-lats1-2-taz-yap-signaling-drives-malignant-peripheral-nerve-sheath-tumorigenesis
#3
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
#4
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/29420386/neurofibroma-of-the-nasal-ala-pediatric-nasal-reconstruction
#5
Oswaldo J Gómez, Mónica Bernal, Diana Carolina Martínez
Benign tumors of the nasal cavity originating from a peripheral nerve sheath are rare. The authors present a case of a 3-month-old boy with a mass in the left nasal vestibule. The surgical resection was complicated by full-thickness necrosis of more than 50% of the nasal ala. The tumor was diagnosed histopathologically as a neurofibroma with glial heterotopia. The details of the nasal reconstruction with a paramedian forehead flap in 3 stages and postoperative results are provided with satisfactory cosmetic and functional results...
February 7, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29410763/oral-peripheral-nerve-sheath-tumors-a-clinicopathological-and-immunohistochemical-study-of-32-cases-in-a-brazilian-population
#6
Talita Franco, Silas-Antonio-Juvencio de Freitas Filho, Laís-Borges Muniz, Paulo-Rogério de Faria, Adriano-Mota Loyola, Sérgio-Vitorino Cardoso
Background: Oral peripheral nerve sheath tumors (OPNSTs) are reactive or neoplastic diseases that develop from proliferation of the nerve itself or their limiting sheaths. Here we describe the clinicopathologic data of OPNSTs observed in a sample of the Brazilian population and evaluate the expression of molecules associated with neural biology to determine their usefulness in the diagnosis. Material and Methods: Descriptive study of cases diagnosed as OPNSTs, from the Pathology Laboratory at the School of Dentistry/ Federal University of Uberlandia, followed by an immunohistochemical study of S-100, CD57, neurofilament protein (NFP) and epithelial membrane antigen (EMA)...
December 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/29409029/the-characteristics-of-76-atypical-neurofibromas-as-precursors-to-neurofibromatosis-1-associated-malignant-peripheral-nerve-sheath-tumors
#7
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
#8
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/29402239/primary-extra-uterine-and-extra-ovarian-mullerian-adenosarcoma-case-report-and-literature-review
#9
Vincenzo Dario Mandato, Federica Torricelli, Valentina Mastrofilippo, Riccardo Valli, Lorenzo Aguzzoli, Giovanni Battista La Sala
BACKGROUND: Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes...
February 5, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29398191/spinal-hemangioma-mimicking-a-dumbbell-shaped-schwannoma-case-report-and-review-of-the-literature
#10
Kevin T Huang, Malia McAvoy, Jeffrey Helgager, Viren Vasudeva, John H Chi
Spinal hemangiomas are common, benign vascular lesions that involve the bony portion of vertebral bodies and are generally asymptomatic. Rarely, they can become aggressive and present with predominantly epidural extension, mimicking other neoplasms. We present the case of a fifty-one year old woman who presented with myelopathy and was discovered to have a large mass causing epidural spinal cord compression, thought to be due to a peripheral nerve sheath tumor. She underwent surgery for tumor debulking. Intraoperatively, the mass was found to be mostly epidural with minimal bone involvement...
February 1, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29396607/-sinonasal-tumors-news-from-the-who-with-special-reference-to-mesenchymal-entities
#11
REVIEW
A Agaimy, F Haller, A Hartmann
The last two decades have seen significant advances in the pathology of sinonasal tract neoplasms. This was the consequence of the availability of several innovative diagnostic tools, which resulted in a dynamic evolution of entities and splitting of newly defined or conceptualized entities and subtypes that have been included in the spectrum of old heterogeneous diseases. Most of these new tumor subtypes have distinctive demographic, clinicopathologic, and biological characteristics with prognostic and therapeutic implications for individual patients...
February 2, 2018: Der Pathologe
https://www.readbyqxmd.com/read/29394776/-a-rare-case-of-abdominal-malignant-triton-tumor
#12
Toshihiro Ogawa, Masao Harano, Toshiaki Morito, Kouichi Ichimura, Yasuhiro Choda, Hiroyoshi Matsukawa, Yasutomo Ojima, Hitoshi Idani, Shigehiro Shiozaki, Masazumi Okajima
A 60-year-old woman presented at our hospital with abdominal pain and vomiting.Three abdominal tumors were detected, and she was referred to our department for surgery.She underwent an enterectomy including 2 of the tumors.The third tumor could not be resected because it had invaded the superior mesenteric artery(SMA)and superior mesenteric vein(SMV). Based on positive immunohistochemistry results for S-100 protein and desmin, nerve sheath differentiation with rhabdomyoblastic differentiation was confirmed, and she was diagnosed with a malignant triton tumor(MTT)...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29381644/schwannoma-of-the-membranous-nasal-septum-a-clinical-report-with-aesthetic-approach
#13
Yong-Ha Kim, Jin Ho Lee, Youngsoo Park, Kyu Jin Chung
Schwannoma is an uncommon tumor of nerve sheath that arises from any peripheral, cranial, or autonomic nerve. Only 4% of head and neck schwannomas originate from the sinonasal tract, and a finding of a schwannoma in the nasal septum is exceedingly rare. The authors experienced nasal septal schwannoma with the functional and aesthetic consideration of nasal contour. The authors present an open rhinoplasty approach for nasal septal schwannoma which has not been reported in the previous literature yet.
January 19, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29369179/targeted-next-generation-sequencing-of-malignant-peripheral-nerve-sheath-tumor-of-the-pterygopalatine-fossa-with-intracranial-metastatic-recurrence
#14
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
#15
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...
January 19, 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
#16
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...
January 13, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29318408/atypical-presentation-of-a-primary-cardiac-malignant-peripheral-nerve-sheath-tumor
#17
J A Eindhoven, E E G Loonstra, C Kik, E J van den Bos, M J M Kofflard
No abstract text is available yet for this article.
January 10, 2018: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29314300/low-grade-fibromyxoid-sarcoma-arising-within-the-median-nerve
#18
Amy A Swanson, Caterina Giannini, Andrew L Folpe, Daniel L Van Dyke, Kimberly K Amrami, William A Michalak, Rachael A Vaubel
We report a case of low-grade fibromyxoid sarcoma arising within the median nerve. A 31-year-old woman presented with symptoms of carpal tunnel syndrome and an enlarging mass in her right palm over 1 year. MRI demonstrated a mass associated with the right median nerve with solid and cystic components. At surgery, the mass was located within the epineurium, could be bluntly dissected from the nerve fascicles, and was suspected to be a schwannoma. A 3.4 cm, tan-pink, glistening, smooth, homogenous mass was submitted to pathology...
January 3, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29304038/tumor-targeted-delivery-of-doxorubicin-in-malignant-peripheral-nerve-sheath-tumors
#19
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/29291290/minimally-invasive-approach-to-resection-of-paraspinal-schwannoma
#20
Patrick Paullus, Taylor A Wilson, Paul Lee, Arunprasad Gunasekaran, Noojan Kazemi
In this video, the authors demonstrate a minimally invasive approach and resection of a paraspinal schwannoma. Using an expandable retractor, the authors were able to identify important adjacent bony landmarks and hence visualize and remove this peripheral nerve sheath tumor. While a tubular retractor is commonly used for interbody fusion procedures, the location of the tumor allowed this minimally invasive approach resulting in excellent access, minimal soft-tissue injury, and a short hospital stay. The authors present this approach as a less invasive and yet effective technique for resection of otherwise difficult-to-access nerve lesions...
January 2018: Neurosurgical Focus
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