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Malignant Peripheral Nerve Sheath Tumor

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https://www.readbyqxmd.com/read/29138703/malignant-peripheral-nerve-sheath-tumor-of-the-inguinum-and-angiosarcoma-of-the-scalp-in-a-child-with-neurofibromatosis-type-1
#1
Marija Milković Periša, Tihana Džombeta, Jasminka Stepan Giljević, Božo Krušlin
Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma are rare tumors in children and adolescents and mostly occur in young patients in relation to NF1. Both histological types can be present in the same tumor mass in patients with NF1. We present a case of 12.5-year-old girl with NF1 who first presented with MPNST of the right inguinal region and 1.5 years later with unrelated angiosarcoma of the scalp.
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/29138631/sarc006-phase-ii-trial-of-chemotherapy-in-sporadic-and-neurofibromatosis-type-1-associated-chemotherapy-naive-malignant-peripheral-nerve-sheath-tumors
#2
Christine S Higham, Seth M Steinberg, Eva Dombi, Arie Perry, Lee J Helman, Scott M Schuetze, Joseph A Ludwig, Arthur Staddon, Mohammed M Milhem, Daniel Rushing, Robin L Jones, Michael Livingston, Stewart Goldman, Christopher Moertel, Lars Wagner, David Janhofer, Christina M Annunziata, Denise Reinke, Lauren Long, David Viskochil, Larry Baker, Brigitte C Widemann
Background: Worse chemotherapy response for neurofibromatosis type 1- (NF1-) associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST) has been reported. Methods: We evaluated the objective response (OR) rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%)...
2017: Sarcoma
https://www.readbyqxmd.com/read/29137242/immune-profiling-of-nf1-associated-tumors-reveals-histologic-subtype-distinctions-and-heterogeneity-implications-for-immunotherapy
#3
Kellie B Haworth, Michael A Arnold, Christopher R Pierson, Kwangmin Choi, Nicholas D Yeager, Nancy Ratner, Ryan D Roberts, Jonathan L Finlay, Timothy P Cripe
Successful treatment of neurofibromatosis type 1 (NF1)-associated tumors poses a significant clinical challenge. While the primary underlying genetic defect driving RAS signaling is well described, recent evidence suggests immune dysfunction contributes to tumor pathogenesis and malignant transformation. As immunologic characterizations, prognostic and predictive of immunotherapeutic clinical response in other cancers, are not fully described for benign and malignant NF1-related tumors, we sought to define their immunologic profiles...
October 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29131833/mek-inhibitors-enhance-therapeutic-response-towards-atra-in-nf1-associated-malignant-peripheral-nerve-sheath-tumors-mpnst-in-vitro
#4
Susan Fischer-Huchzermeyer, Anna Dombrowski, Gordon Wilke, Verena Stahn, Anna Streubel, Victor Felix Mautner, Anja Harder
OBJECTIVE: Neurofibromatosis type 1 (NF1) is a hereditary tumor syndrome characterized by an increased risk of malignant peripheral nerve sheath tumors (MPNST). Chemotherapy of MPNST is still insufficient. In this study, we investigated whether human tumor Schwann cells derived from NF1 associated MPNST respond to all-trans retinoic acid (ATRA). We analyzed effects of ATRA and MEK inhibitor (MEKi) combination therapy. METHODS: MPNST cell lines S462, T265, NSF1 were treated with ATRA and MEKi U0126 and PD0325901...
2017: PloS One
https://www.readbyqxmd.com/read/29127695/management-of-the-other-retroperitoneal-sarcomas
#5
REVIEW
Piotr L Rutkowski, John T Mullen
The focus of this review is on the management of the less common sarcomas occurring in the retroperitoneal space, including solitary fibrous tumor (SFT), malignant peripheral nerve sheath tumor (MPNST), perivascular epithelioid cell tumor (PEComa), and undifferentiated pleomorphic sarcoma (UPS) of the psoas muscle. As for other retroperitoneal sarcomas, surgical resection is the mainstay of curative therapy, and multidisciplinary preoperative assessment, including percutaneous needle biopsy for histologic confirmation, is the basis for personalized management, as the surgical management, and the integration of systemic therapy and radiation therapy is unique to each histologic subtype...
November 11, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29118384/the-genomic-landscape-of-malignant-peripheral-nerve-sheath-tumors-diverse-drivers-of-ras-pathway-activation
#6
Andrew S Brohl, Elliot Kahen, Sean J Yoder, Jamie K Teer, Damon R Reed
Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma. To more fully characterize the genomic landscape of this tumor type, we performed next generation sequencing studies for mutational and copy number analysis. We analyzed whole exome sequencing data from 12 MPNST and SNP arrays for a subset of these. We additionally conducted a literature review of prior next generation sequencing studies in this disease and compared to the current study. We report recurrent mutations in NF1, SUZ12, EED, TP53 and CDKN2A in our study cohort...
November 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29117388/neurofibromatosis-type-1-associated-mpnst-state-of-the-science-outlining-a-research-agenda-for-the-future
#7
Karlyne M Reilly, AeRang Kim, Jaishri Blakely, Rosalie E Ferner, David H Gutmann, Eric Legius, Markku M Miettinen, R Lor Randall, Nancy Ratner, N L Jumbé, Annette Bakker, David Viskochil, Brigitte C Widemann, Douglas R Stewart
Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma for which the only effective therapy is surgery. In 2016, an international meeting entitled "MPNST State of the Science: Outlining a Research Agenda for the Future" was convened to establish short- and long-term research priorities. Key recommendations included the: 1) development of standardized, cost-efficient fluorodeoxyglucose positron emission tomography and whole-body magnetic resonance imaging guidelines to evaluate masses concerning for MPNST; 2) development of better understanding and histologic criteria for the transformation of a plexiform neurofibroma to MPNST; 3) establishment of a centralized database to collect genetic, genomic, histologic, immunohistochemical, molecular, radiographic, treatment, and related clinical data from MPNST subspecialty centers in a standardized manner; 4) creation of accurate mouse models to study the plexiform neurofibroma-to-MPNST transition, MPNST metastasis, and drug resistance; 5) use of trial designs that minimize regulatory requirements, maximize availability to patients, consider novel secondary end points, and study patients with newly diagnosed disease...
August 1, 2017: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/29115724/malignant-peripheral-nerve-sheath-tumor-the-need-to-get-it-right-first-time-around
#8
Bernadette Brennan
No abstract text is available yet for this article.
November 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29114437/magnetic-resonance-imaging-appearance-of-schwannomas-from-head-to-toe-a-pictorial-review
#9
Jamie Crist, Jacob R Hodge, Matthew Frick, Fiona P Leung, Eugene Hsu, Ming Tye Gi, Sudhakar K Venkatesh
Schwannomas are benign soft-tissue tumors that arise from peripheral nerve sheaths throughout the body and are commonly encountered in patients with neurofibromatosis Type 2. The vast majority of schwannomas are benign, with rare cases of malignant transformation reported. In this pictorial review, we discuss the magnetic resonance imaging (MRI) appearance of schwannomas by demonstrating a collection of tumors from different parts of the body that exhibit similar MRI characteristics. We review strategies to distinguish schwannomas from malignant soft-tissue tumors while exploring the anatomic and histologic origins of these tumors to discuss how this correlates with their imaging findings...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/29114282/multicompartmental-primary-spinal-extramedullary-tumors-value-of-an-interdisciplinary-approach
#10
Guruprasad Bettaswamy, Paurush Ambesh, Raj Kumar, Rabi Narayan Sahu, Kuntal Kanti Das, Awadhesh Kumar Jaiswal, Arun Kumar Srivastava, Sanjay Behari
Background: Primary extramedullary tumors involving multiple compartments around the spine are a technically demanding group of tumors whose extent traverses beyond the normal confines of those anatomical regions which fall in the common domain of neurosurgeons. In the following series, we present 12 patients who were diagnosed with primary spinal extramedullary tumors with multicompartmental extension, and whose surgical management was facilitated by a combined multidisciplinary approach involving surgeons of other superspecialties...
October 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29110923/malignant-triton-tumor-of-the-gluteal-region-in-a-patient-unaffected-by-neurofibromatosis-a-case-report
#11
Abdullah Merter, Kerem Başarır, Yusuf Yıldız, Yener Sağlık
Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) made up of both malignant schwannoma cells and malignant rhabdomyoblasts. A 26-years-old male patient was admitted with an asymptomatic gluteal mass. Magnetic resonance imaging showed heterogeneous soft tissue mass and he underwent open biopsy. Malignant peripheral nerve sheath tumor was diagnosed. He was given adjuvant chemotherapy following the removal of the tumor with hip disarticulation. The tumor was diagnosed as "malignant triton tumor" based on pathological examination including immunohistochemical studies...
October 27, 2017: Acta Orthopaedica et Traumatologica Turcica
https://www.readbyqxmd.com/read/29104109/aberrant-expression-of-thyroid-transcription-factor-1-in-schwannomas
#12
Dai-Zhong Wang, Ping Liu, Li Yao, Ying-Hua Hao, Rui-Juan Zhu, Tao Zhang, Xian-Bin Tang
Aberrant expression of thyroid transcription factor-1 (TTF-1) has been observed in tumors arising in locations other than thyroid gland, lung and ventral forebrain. However, TTF-1 expression in schwannomas has not yet been studied. Meanwhile, a few inconsistent changes in protein expression have been identified between schwannomas and other peripheral nerve sheath tumors. We evaluated TTF-1 expression in 161 schwannomas and 43 other peripheral nervous system lesions, including ganglioneuromas (n=8), malignant peripheral nerve sheath tumors (MPNSTs) (n=11), neurofibromas (n=24), and traumatic neuromas (n=9), using immunohistochemistry and verified it using quantitative real-time reverse transcription polymerase chain reaction (qPCR) to explore TTF-1 expression in peripheral nervous system lesions...
November 2, 2017: Human Pathology
https://www.readbyqxmd.com/read/29089742/a-case-of-retroperitoneal-malignant-triton-tumor-in-a-nigerian-boy
#13
Jideofor Okechukwu Ugwu, Michael Emeka Onwukamuche, Hyginus O Ekwunife, Jude Kennedy C Emejulu, Victor Modekwe, Osuigwe An Osuigwe
Malignant peripheral nerve sheath tumor is a rare tumor occurring in 5%-10% of all malignant soft tissues sarcomas and triton tumor arising from neurofibromatosis type 1 (NF-1) is even rarer with associated high rate of mortality. No case of triton tumor has been reported in Nigeria to the best of our knowledge. We seek to report a case of lately detected retroperitoneal triton tumor presenting in a 12-year-old Nigerian child who was brought with bilateral lower limb weaknesses, weight loss, and a right lumbar mass...
July 2017: Nigerian Journal of Surgery: Official Publication of the Nigerian Surgical Research Society
https://www.readbyqxmd.com/read/29061454/malignant-peripheral-nerve-sheath-tumor-in-a-patient-with-bap1-tumor-predisposition-syndrome-case-report
#14
Megan C Kaszuba, Jose S Pulido, Andrew L Folpe, Pavel N Pichurin, McKinsey L Goodenberger, Robert J Spinner
BACKGROUND: Germline pathogenic variants in BRCA1-associated protein-1 (BAP1), a nuclear ubiquitin carboxy-terminal hydrolase with evidence suggestive of independent tumor suppressor function, predispose affected families to uveal melanoma, cutaneous melanoma, renal cell carcinoma, malignant mesothelioma, and possibly a range of other tumors and malignancies as part of the BAP1 tumor predisposition syndrome (BAP1-TPDS), a recently recognized hereditary cancer syndrome. CASE DESCRIPTION: A 50 year old female patient presented with a malignant peripheral nerve sheath tumor of the left fifth metatarsal head...
October 20, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29047224/ral-signaling-pathway-in-health-and-cancer
#15
REVIEW
Adel Rezaei Moghadam, Elham Patrad, Elham Tafsiri, Warner Peng, Benjamin Fangman, Timothy J Pluard, Anthony Accurso, Michael Salacz, Kushal Shah, Brandon Ricke, Danse Bi, Kyle Kimura, Leland Graves, Marzieh Khajoie Najad, Roya Dolatkhah, Zohreh Sanaat, Mina Yazdi, Naeimeh Tavakolinia, Mohammad Mazani, Mojtaba Amani, Saeid Ghavami, Robyn Gartell, Colleen Reilly, Zaid Naima, Tuba Esfandyari, Faris Farassati
The Ral (Ras-Like) signaling pathway plays an important role in the biology of cells. A plethora of effects is regulated by this signaling pathway and its prooncogenic effectors. Our team has demonstrated the overactivation of the RalA signaling pathway in a number of human malignancies including cancers of the liver, ovary, lung, brain, and malignant peripheral nerve sheath tumors. Additionally, we have shown that the activation of RalA in cancer stem cells is higher in comparison with differentiated cancer cells...
October 18, 2017: Cancer Medicine
https://www.readbyqxmd.com/read/29033684/fluorine-18-2-fluoro-2-deoxy-d-glucose-positron-emission-tomography-computed-tomography-masquerading-as-a-case-of-sporadic-malignant-peripheral-nerve-sheath-tumor-of-lower-extremity-presenting-as-massive-lower-limb-edema
#16
Deepa Singh, Rajender Kumar, Ashim Das, Subhash C Varma, Bhagwant R Mittal
Malignant peripheral nerve sheath tumors (MPNSTs) are rare neuroectodermal tumors resulting from the malignant transformation of benign plexiform neurofibromas. The sporadic form of these tumors is rare than familial variants (seen in neurofibromatosis Type 1) and making the diagnosis difficult. We are presenting a case of 40--year-old female with the complaint of progressive swelling of lower limb with initial suspicion of lymphedema and underwent lymphoscintigraphy, magnetic resonance imaging, and finally fluorine-18-2-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography scans were done to rule out mitotic etiology and extent of the disease...
October 2017: World Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29020982/the-prognostic-value-of-c-x-c-motif-chemokine-receptor-4-in-patients-with-sporadic-malignant-peripheral-nerve-sheath-tumors
#17
Chao Zhang, Fang-Yuan Chang, Wen-Ya Zhou, Ji-Long Yang
BACKGROUND: Recent studies indicate that C-X-C motif chemokine receptor 4 (CXCR4) and its ligand, C-X-C motif chemokine ligand 12 (CXCL12), stimulate expression of the cell cycle regulatory protein Cyclin D1 in neurofibromatosis 1-associated malignant peripheral nerve sheath tumor (MPNST) cells and promote their proliferation. In this study, we measured the expression of CXCR4, CXCL12, and Cyclin D1 proteins in sporadic MPNST tissues from Chinese patients and investigated their prognostic values...
October 11, 2017: Chinese Journal of Cancer
https://www.readbyqxmd.com/read/28991373/novel-enriched-pathways-in-superficial-malignant-peripheral-nerve-sheath-tumors-mpnst-and-spindle-desmoplastic-melanomas-sdm
#18
George Jour, Nicole K Andeen, Rami Al -Rohil, Phyu P Aung, Meenakshi Mehrotra, Dzifa Duose, Benjamin Hoch, Zolt Argenyi, Rajyalakshmi Luthra, Ignacio I Wistuba, Victor G Prieto
Superficial malignant peripheral nerve sheath tumor (MPNST) is a rare, soft tissue neoplasm that shares morphological features and some molecular events with spindle and desmoplastic melanoma (SDM). Herein we sought to identify molecular targets for therapy using targeted RNA/DNA sequencing and gene expression of key immunological players. DNA and RNA from formalin-fixed, paraffin-embedded (FFPE) tissue were extracted and processed. Massive high-throughput deep parallel sequencing was performed using the Oncomine comprehensive panel enabling detection of relevant SNVs, CNVs, gene fusions, and indels from 143 unique genes on the Ion torrent sequencer for clinical trial research programs...
October 9, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28991098/five-year-old-boy-with-behavioral-changes-and-papilledema
#19
Radha Ram, Jeremy Y Jones, Carrie A Mohila, Veeral S Shah
A 5-year-old boy had initial symptoms of behavioral changes, nausea, vomiting, headache, weight loss, and progressive vision failure. Brain MRI revealed abnormal signal intensity in both optic nerves, the optic chiasm, the right medial temporal lobe, and tissues surrounding the right supraclinoid internal carotid artery with associated leptomeningeal and spinal cord enhancement. After nondiagnostic dural and spinal arachnoid biopsies, a temporal lobe biopsy was diagnostic for a rare malignant peripheral nerve sheath tumor...
October 5, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28976138/leiomyosarcoma-of-maxilla-a-case-report-with-review-of-literature
#20
Renuka Gupta, Madhusudan Astekar, Ramakant Dandriyal, Manjunath Bs
One challenging feature of head and neck pathology is that a dizzying array of spindle cell lesions occurs here which ranges all the way from reactive, very aggressive forms to malignant lesions. Leiomyosarcoma is one such malignant tumour of mesenchymal origin exhibiting smooth muscle differentiation; presenting generally nonspecific signs and symptoms. Here we present a case of leiomyosarcoma in a 21 year old female patient associated with single reddish pink swelling present in the posterior right maxillary tuberosity region with moderate facial asymmetry...
September 2017: Journal of Experimental Therapeutics & Oncology
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