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Malignant nerve sheath tumor

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https://www.readbyqxmd.com/read/29616298/intracranial-malignant-peripheral-nerve-sheath-tumor-variant-an-unusual-neurovascular-phenotype-sarcoma-case-invading-through-the-petrous-bone
#1
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/29606723/retrobulbar-malignant-peripheral-nerve-sheath-tumor-in-a-golden-retriever-dog-a-challenging-diagnosis
#2
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/29596596/%C3%AE-iii-spectrin-immunohistochemistry-as-a-potential-diagnostic-tool-with-high-sensitivity-for-malignant-peripheral-nerve-sheath-tumors
#3
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
#4
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
#5
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/29534899/single-and-multi-fraction-stereotactic-radiosurgery-dose-tolerances-of-the-optic-pathways
#6
Michael T Milano, Jimm Grimm, Scott G Soltys, Ellen Yorke, Vitali Moiseenko, Wolfgang A Tomé, Arjun Sahgal, Jinyu Xue, Lijun Ma, Timothy D Solberg, John P Kirkpatrick, Louis S Constine, John C Flickinger, Lawrence B Marks, Issam El Naqa
PURPOSE: Dosimetric and clinical predictors of radiation-induced optic nerve/chiasm neuropathy (RION) after single-fraction stereotactic radiosurgery (SRS) or hypofractionated (2-5 fractions) radiosurgery (fSRS) were analyzed from pooled data that were extracted from published reports (PubMed indexed from 1990 to June 2015). This study was undertaken as part of the American Association of Physicists in Medicine Working Group on Stereotactic Body Radiotherapy, investigating normal tissue complication probability (NTCP) after hypofractionated radiation...
January 31, 2018: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/29518229/case-report-metastasis-of-a-trigeminal-malignant-peripheral-nerve-sheath-tumor-to-the-corpus-callosum
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/29489027/extraskeletal-osteosarcoma-mdm2-and-h3k27me3-analysis-of-19-cases-suggest-disease-heterogeneity
#11
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/29487221/a-phase-ii-study-of-tumor-ablation-in-patients-with-metastatic-sarcoma-stable-on-chemotherapy
#12
Angela C Hirbe, Jack Jennings, Nael Saad, Joseph D Giardina, Yu Tao, Jingqin Luo, Shellie Berry, Jacqui Toeniskoetter, Brian A Van Tine
LESSONS LEARNED: Ablation therapy appears to be a reasonably safe and effective approach to obtain a significant treatment-free interval for a subset of patients with limited sites of metastatic disease for which systemic control can be obtained with six cycles of chemotherapy. BACKGROUND: Metastatic sarcoma often becomes resistant to treatment by chemotherapy. There is sometimes prolonged stable disease from active chemotherapy that provides a window of opportunity for an intervention to prolong disease-free survival...
February 27, 2018: Oncologist
https://www.readbyqxmd.com/read/29485367/orbital-malignant-meningioma-a-unique-presentation-of-a-rare-entity
#13
Dane H Slentz, Sunil Bellur, M Reza Taheri, M Isabel Almira-Suarez, Jonathan H Sherman, Tamer N Mansour
An elderly female with progressive proptosis was found to have an aggressive retrobulbar solid orbital mass. The mass was distinct from the optic nerve sheath and intracranial meninges, and produced concave erosion of the sphenoid wing. Operative findings demonstrated an orbital mass adherent to the dura of the superior orbital fissure. The mass did not demonstrate meningeal violation, infiltrate the superior orbital fissure, or display intracranial spread. The dura remained intact after gross total resection...
February 27, 2018: Orbit
https://www.readbyqxmd.com/read/29482987/immunohistochemical-evaluation-of-h3k27-trimethylation-in-malignant-peripheral-nerve-sheath-tumors
#14
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/29474318/fibroblastic-malignant-peripheral-nerve-sheath-tumour-of-the-uterine-cervix-report-of-a-case-and-literature-review-with-emphasis-on-possible-differential-diagnosis
#15
Valentina Sangiorgio, Vanna Zanagnolo, Giovanni Aletti, Luca Bocciolone, Simone Bruni, Fabio Landoni, Nicoletta Colombo, Angelo Maggioni, Enzo Ricciardi
Cervical sarcomas are rare neoplasms, accounting for <1% of all cervical malignancies and characterized by an aggressive course despite radical excision. We report the clinical and microscopic features of a spindle cell sarcoma arising as a polypoid endocervical mass in a 45-yr-old woman. The neoplasm was characterized by a monotonous, mildly atypical proliferation of spindle cells, displaying a fibrosarcoma-like parallel pattern of highly dense fascicles, growing under the cervical epithelium. Mitotic activity was conspicuous, with up to 40 mitoses per 10 HPF...
February 22, 2018: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/29473773/mln8237-treatment-in-an-orthoxenograft-murine-model-for-malignant-peripheral-nerve-sheath-tumors
#16
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
#17
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/29455369/intradural-spinal-tumors-in-adults-update-on-management-and-outcome
#18
REVIEW
Malte Ottenhausen, Georgios Ntoulias, Imithri Bodhinayake, Finn-Hannes Ruppert, Stefan Schreiber, Annette Förschler, John A Boockvar, Andreas Jödicke
Among spinal tumors that occur intradurally, meningiomas, nerve sheath tumors, ependymomas, and astrocytomas are the most common. While a spinal MRI is the state of the art to diagnose intradural spinal tumors, in some cases CT scans, angiography, CSF analyses, and neurophysiological examination can be valuable. The management of these lesions depends not only on the histopathological diagnosis but also on the clinical presentation and the anatomical location, allowing either radical resection as with most extramedullary lesions or less invasive strategies as with intramedullary lesions...
February 17, 2018: Neurosurgical Review
https://www.readbyqxmd.com/read/29449010/a-brief-report-of-plexiform-neurofibroma
#19
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/29446979/synovial-sarcoma-mimicking-a-thoracic-dumbell-schwannoma-a-case-report
#20
Susanth Subramanian, Gandham Edmond Jonathan, Bimal Patel, Krishna Prabhu
INTRODUCTION: Synovial sarcoma is a rare mesenchymal malignant neoplasm that accounts for less than 10% of soft tissue sarcomas. About 95% of the sarcomas occur in the extremities. Primary synovial sarcomas of the spine are a rare tumor arising from the paravertebral regions, paraspinal muscles or epidural spaces. PURPOSE: To report an atypical radiological presentation of synovial sarcoma of the thoracic spine mimicking a nerve sheath tumor in an elderly adult and describe the management with review of the literature...
February 15, 2018: British Journal of Neurosurgery
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