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Malignant nerve sheath tumor

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https://www.readbyqxmd.com/read/28526004/hybrid-peripheral-nerve-sheath-tumors-report-of-five-cases-and-detailed-review-of-literature
#1
Nasir Ud Din, Zubair Ahmad, Jamshid Abdul-Ghafar, Rashida Ahmed
BACKGROUND: Hybrid peripheral nerve sheath tumors (PNSTs) have been recognized recently and were first included in the 4th edition of World Health Organization (WHO) Classification of Tumors of Soft tissue and Bone, published in 2013. These tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. The most common combinations are those of schwannoma/perineurioma followed by combinations of neurofibroma/schwannoma and neurofibroma/perineurioma...
May 19, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28524122/-a-plasminogen-regulation-system-in-brain-tumors
#2
O I Kit, E M Frantsiyants, L S Kozlova, E E Rostorguev, I V Balyazin-Parfenov, Yu A Pogorelova
INTRODUCTION: Tumor progression and neovascularization during malignant processes are believed to be associated with plasminogen activators and the PAI-1 inhibitor, but their role and interactions in various types of brain tumors have been studied insufficiently. AIM: To conduct a comparative study of plasminogen regulation in optic nerve sheath meningiomas, glioblastomas, and brain metastases of breast cancer, as well as in perifocal tissues surrounding the tumors...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28515751/malignant-triton-tumor-of-the-lung-infiltrating-the-left-atrium-and-left-ventricle-with-metastasis-to-the-small-intestine
#3
Dariusz Tomaszewski, Sebastian Beta, Jan A Rogowski
Malignant triton tumors are a rare subtype of malignant peripheral nerve sheath tumors showing rhabdomyosarcomatous differentiation. We report the case of a 33-year-old patient who underwent an excision of such a tumor by thoracic and cardiac surgery. He was reoperated on 1 month later because of a tumor of the small intestine and received adjuvant radiation.
March 2017: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/28508686/malignant-peripheral-nerve-sheath-tumor-of-the-femur-a-rare-diagnosis-supported-by-complete-immunohistochemical-loss-of-h3k27me3
#4
Masato Sugawara, Eisuke Kobayashi, Naofumi Asano, Akihiko Yoshida, Akira Kawai
The histological diagnosis of malignant peripheral nerve sheath tumor (MPNST) is challenging because of the wide morphological spectrum and suboptimal performance of conventional immunohistochemical markers. MPNST arising primarily in the bone is exceptional, and its definitive diagnosis, particularly out of the neurofibromatosis type 1 (NF1) context, is even more problematic. Recurrent inactivation of EED or SUZ12 in a majority of MPNSTs results in a complete loss of trimethylated histone H3 at lysine 27 (H3K27me3) immunoreactivity, making it a highly specific biomarker of MPNSTs...
May 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28505957/spindle-cell-type-malignant-peripheral-nerve-sheath-tumor-arising-in-benign-schwannoma-with-multiple-intraosseous-spinal-metastasis-a-case-report
#5
Jae Yoon Chung, Sung Sun Kim, Sung Kyu Kim
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) arising in benign schwannoma with multiple intraosseous spinal metastasis is extremely rare, having a highly aggressive progression and poor prognosis. In such cases, the malignant cells of MPNST usually have an epithelioid morphology. Here, the authors present a very rare case of spindle cell type MPNST arising in benign schwannoma. CASE: A 47-year-old woman had a history of wide marginal excision of right buttock spindle cell sarcoma previously...
May 5, 2017: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/28502561/acute-hydrocephalus-due-to-a-primary-malignant-peripheral-nerve-sheath-tumor-of-the-cervicothoracic-junction-a-case-report-and-review-of-the-literature
#6
Y Samanci, H S Togay, R Yakar, F Kabukcuoglu, S E Celik
BACKGROUND: The estimated incidence of malignant nerve sheath tumors is 0.001% per year, and only 2-3% of those tumors involve the spinal nerves. We present a rare case of acute hydrocephalus caused by primary malignant peripheral nerve sheath tumor of the cervicothoracic junction. CASE DESCRIPTION: A 29-year-old previously healthy male patient, except for a history of two previous surgeries for ulnar nerve entrapment and progressive left upper extremity weakness, presented with acute onset somnolence...
May 11, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28502478/the-cellular-retinoic-acid-binding-protein-2-promotes-survival-of-malignant-peripheral-nerve-sheath-tumor-cells
#7
Susan Fischer-Huchzermeyer, Anna Dombrowski, Christian Hagel, Victor Felix Mautner, Jens Schittenhelm, Anja Harder
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive neoplasms that commonly occur in patients with neurofibromatosis type 1 (NF1). Effective chemotherapy is not available. To characterize a therapeutic target for treatment, we investigated the role of cellular retinoic acid binding protein 2 (CRABP2) in MPNST in vitro. CRABP2 is a transcriptional co-activator of retinoic acid signaling. Although overexpression of CRABP2 is described in several cancers, it has not yet been studied in MPNSTs. We investigated CRABP2 expression in cultured Schwann cells and formalin-fixed, paraffin-embedded specimens of human peripheral nerve sheath tumors...
May 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28498283/synovial-sarcoma-of-peripheral-nerves-analysis-of-15-cases
#8
John S A Chrisinger, Usama I Salem, Lars-Gunnar Kindblom, Behrang Amini, Magnus Hansson, Jeanne M Meis
Synovial sarcoma of peripheral nerve (SSPN) is rare with only 26 cases reported in English. SSPN is often mistaken for a benign or malignant peripheral nerve sheath tumor (PNST) by clinicians and pathologists. Fifteen cases of SSPN were retrieved from the pathology files of 3 institutions. All tumors arose in a nerve based on imaging and/or operative findings and the diagnoses were histologically confirmed. Neuropathic symptoms predominated in 11 women and 4 men, 19- to 62-year-old (median, 39 years) with tumors involving the ulnar (5), median (3), peroneal (3) or sciatic (2) nerve, or the L4 or T1 nerve root (2)...
May 11, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28493803/soft-tissue-sarcomas-of-the-abdomen-and-pelvis-radiologic-pathologic-features-part-2-uncommon-sarcomas
#9
Angela D Levy, Maria A Manning, Markku M Miettinen
Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article...
May 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28475007/tim-3-plays-a-more-important-role-than-pd-1-in-the-functional-impairments-of-cytotoxic-t-cells-of-malignant-schwannomas
#10
Zhao Li, Xiaobing Liu, Rongbin Guo, Pengfei Wang
Cancer immunotherapy using cytotoxic T cells demonstrates dramatic survival benefits in lymphomas, but its efficacy in solid tumors is limited. Here, we investigated the possibility of using cytotoxic T cells to treat malignant Schwannoma, a rare but aggressive nerve sheath tumor, by examining the native T-cell immunity in the host. We found that compared to CD8(+) T cells from healthy controls or benign Schwannoma patients, the CD8(+) T cells from malignant Schwannoma patients were present at normal frequencies but were substantially enriched with PD-1(-)TIM-3(+) and PD-1(+)TIM-3(+) cells...
May 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/28469964/comprehensive-pharmacological-profiling-of-neurofibromatosis-cell-lines
#11
Jianman Guo, Michael R Grovola, Hong Xie, Grace E Coggins, Patrick Duggan, Rukhsana Hasan, Jiale Huang, Danny W Lin, Claire Song, Gabriela M Witek, Simon Berritt, David C Schultz, Jeffrey Field
Patients with Neurofibromatosis type 1 (NF1) and Neurofibromatosis type 2 (NF2) are predisposed to tumors of the nervous system. NF1 patients predominantly develop neurofibromas, and Malignant Peripheral Nerve Sheath Tumors (MPNST) while NF2 patients develop schwannomas and meningiomas. Here we quantified the drug sensitivities of NF1 and NF2 tumor cell lines in a high throughput platform. The platform contained a comprehensive collection of inhibitors of MEK, RAF, RAS, farnesyl transferase, PAK and ERK, representative drugs against many other cancer pathways including Wnt, Hedgehog, p53, EGF, HDAC, as well as classical cytotoxic agents recommended for treating MPNST, such as doxorubicin and etoposide...
2017: American Journal of Cancer Research
https://www.readbyqxmd.com/read/28468046/-primary-esophageal-malignant-peripheral-nerve-sheath-tumor-report-of-a-case
#12
Z Y Yue, X H Guo, M X Pang, Y G Dong, H Wang, W X Li
No abstract text is available yet for this article.
May 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28454108/telomere-erosion-in-nf1-tumorigenesis
#13
Rhiannon E Jones, Julia W Grimstead, Ashni Sedani, Duncan Baird, Meena Upadhyaya
Neurofibromatosis type 1 (NF1; MIM# 162200) is a familial cancer syndrome that affects 1 in 3,500 individuals worldwide and is inherited in an autosomal dominant fashion. Malignant Peripheral Nerve Sheath Tumors (MPNSTs) represent a significant cause of morbidity and mortality in NF1 and currently there is no treatment or definite prognostic biomarkers for these tumors. Telomere shortening has been documented in numerous tumor types. Short dysfunctional telomeres are capable of fusion and it is considered that the ensuing genomic instability may facilitate clonal evolution and the progression to malignancy...
April 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28452074/clinicopathologic-features-and-immunohistochemical-spectrum-of-11-cases-of-epithelioid-malignant-peripheral-nerve-sheath-tumors-including-ini1-smarcb1-results-and-braf-v600e-analysis
#14
Bharat Rekhi, Kemal Kosemehmetoglu, Gaye Guler Tezel, Sergulen Dervisoglu
Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare, relatively less chemosensitive sarcoma. We report clinicopathologic features of 11 epithelioid MPNSTs, including rare forms, along with INI1 immunostaining and BRAF V600E mutation results. BRAF V600E mutation was tested by Real-time polymerase chain reaction (PCR) technique. Eleven tumors occurred in six men and five women (M:F ratio = 0.85:1) within an age range of 5-73 years (average = 44), mostly in lower limbs (five), followed by upper limbs (four)...
April 27, 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28447903/low-grade-sinonasal-sarcoma-with-neural-and-myogenic-features-a-recently-discovered-entity-with-unique-features-and-diagnostic-challenge
#15
Meriam Triki, Lobna Ayadi
Low-grade sinonasal sarcoma with neural and myogenic features is an entity recently described in the literature. Little is known about its etiopathogenesis, natural history, or optimal treatment. In fact, it has relatively unique findings: it has a distinctive cytogenetic signature, and it expresses both smooth muscle actin and S100 protein. However, its diagnosis is challenging on biopsies showing negative staining for these 2 markers. The differential diagnoses include fibrosarcoma, malignant peripheral nerve sheath tumors, and other benign and malignant lesions...
May 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28443217/malignant-peripheral-nerve-sheath-tumor-of-the-c2-nerve-root-case-report
#16
Joshua D Burks, Robert G Briggs, Chad A Glenn, John C Greenert, Cordell M Baker, Keren Bartal, Greg A Krempl, Michael E Sughrue
Here we present the case of a 36-year-old man who was found to have a symptomatic malignant neural sheath tumor growing from the C2 nerve root following a period of progressively worsening headaches. The patient was successfully treated with surgical resection resulting in resolution of cranial nerve deficits. Though uncommon, malignant peripheral nerve sheath tumor must be considered in the differential diagnosis of tumors involving the cervical nerve roots and carotid space.
April 2017: Journal of Neurological Surgery Reports
https://www.readbyqxmd.com/read/28436992/corrigendum-kank1-inhibits-cell-growth-by-inducing-apoptosis-through-regulating-cxxc5-in-human-malignant-peripheral-nerve-sheath-tumors
#17
Zhibin Cui, Yingjia Shen, Kenny H Chen, Suresh K Mittal, Jer-Yen Yang, GuangJun Zhang
No abstract text is available yet for this article.
April 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28409885/long-term-results-of-the-transmanubrial-osteomuscular-sparing-approach-for-pediatric-tumors
#18
Aziz El Madi, Sabine Irtan, Frédérique Sauvat, Michel Zérah, Gudrun Schleiermacher, Louise Galmiche-Roland, Véronique Minard-Colin, Hervé Brisse, Sabine Sarnacki
BACKGROUND: The complete and safe resection of pediatric cervicothoracic tumors, mostly represented by neurogenic tumors, remains a surgical challenge because of the complex anatomy of this region. The transmanubrial osteomuscular-sparing approach (TOSA) is an alternative to isolated or combined cervical and thoracic approaches enabling the control of supra-aortic vessels and nerves through the thoracic inlet. METHODS: We retrospectively reviewed the tumor characteristics, completeness of resection, morbidity, and long-term outcome of patients with cervicothoracic tumors removed by TOSA between 2000 and 2012 in our institution...
April 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28403573/desmoplastic-melanoma-may-mimic-a-cutaneous-peripheral-nerve-sheath-tumor-report-of-3-challenging-cases
#19
Isidro Machado, Beatriz Llombart, Julia Cruz, Víctor Traves, Celia Requena, Eduardo Nagore, Antonina Parafioriti, Carlos Monteagudo, Antonio Llombart-Bosch
Desmoplastic melanoma (DM) and cutaneous malignant peripheral nerve sheath tumors (MPNST) reveal histological and immunohistochemical similarities, including S100 positivity and negative staining for conventional melanocytic markers. We present 3 cases of cutaneous S100-positive spindle cell tumors in elderly patients, in which first findings led to initial misdiagnoses as cutaneous MPNST and benign peripheral sheath nerve tumor (neurofibroma). The identification of adjacent atypical melanocytic hyperplasia in the overlying skin along with tumor cell proliferation, also in the superficial dermis, the neurotropic component and the absence of any relationship between the tumor and a major nerve, pre-existing neural benign tumor or the existence of stigmata suggestive of neurofibromatosis raised consideration of a DM...
April 12, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28387148/malignant-triton-tumor-malignant-peripheral-nerve-sheath-tumor-with-rhabdomyoblastic-differentiation-occurring-in-a-vascularized-free-flap-reconstruction-graft
#20
Roopa Ram, Jerad Gardner, Sindhura Alapati, Kedar Jambhekar, Tarun Pandey, Corey Montgomery, Richard Nicholas
Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT) is a subtype of MPNST with a component of malignant rhabdomyoblasts in addition to malignant Schwann cells. MPNST and MTT are both aggressive malignancies that most commonly arise from large deep neurofibromas in patients with neurofibromatosis type 1 (NF-1). However, sporadic non-NF-1 cases of MTT have also been reported in the literature...
April 1, 2017: International Journal of Surgical Pathology
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