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https://www.readbyqxmd.com/read/29200699/metastatic-epithelioid-malignant-peripheral-nerve-sheath-tumor-in-a-known-case-of-neurofibromatosis-1-cytomorphological-appearance-and-critical-analysis-of-immunohistochemistry
#1
Rakesh Kumar Gupta, Ravindra Kumar Saran, Deepak Ghuliani, Lalit Garg, Abhijit Das
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue tumors commonly arising from nerve roots in the extremities with a predilection in neurofibromatosis (NF) patients. MPNSTs are known to behave aggressively, with worse prognosis in nonresectable cases, and a 5-year survival of about 52% and 15% in resectable and unresectable cases, respectively. Although cytological diagnosis in a known case of NF-1 is possible, however, in the absence of any associated syndrome in a sporadic case, it is very challenging to accurately diagnose this tumor...
July 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29179472/bcrp-expression-in-schwannoma-plexiform-neurofibroma-and-mpnst
#2
Maurits de Vries, Olaf van Tellingen, Andel G L van der Mey, Antonius M G Bunt, Inge Briaire-de Bruijn, Pancras C W Hogendoorn
Background: peripheral nerve sheath tumors comprise a broad spectrum of neoplasms. Vestibular schwannomas and plexiform neurofibromas are symptomatic albeit benign, but a subset of the latter pre-malignant lesions will transform to malignant peripheral nerve sheath tumors (MPNST). Surgery and radiotherapy are the primary strategies to treat these tumors. Intrinsic resistance to drug therapy characterizes all three tumor subtypes. The breast cancer resistance protein BCRP is a transmembrane efflux transporter considered to play a key role in various biological barriers such as the blood brain barrier...
October 24, 2017: Oncotarget
https://www.readbyqxmd.com/read/29165710/risk-of-soft-tissue-sarcoma-among-69-460-five-year-survivors-of-childhood-cancer-in-europe
#3
Chloe J Bright, Mike M Hawkins, David L Winter, Daniela Alessi, Rodrigue S Allodji, Francesca Bagnasco, Edit Bárdi, Andrea Bautz, Julianne Byrne, Elizabeth A M Feijen, Miranda M Fidler, Stanislaw Garwicz, Desiree Grabow, Thorgerdur Gudmundsdottir, Joyeeta Guha, Nadia Haddy, Momcilo Jankovic, Peter Kaatsch, Melanie Kaiser, Claudia E Kuehni, Helena Linge, Hilde Øfstaas, Cecile M Ronckers, Roderick Skinner, Jop C Teepen, Monica Terenziani, Giao Vu-Bezin, Finn Wesenberg, Thomas Wiebe, Carlotta Sacerdote, Zsuzsanna Jakab, Riccardo Haupt, Päivi Lähteenmäki, Lorna Zadravec Zaletel, Rahel Kuonen, Jeanette F Winther, Florent de Vathaire, Leontien C Kremer, Lars Hjorth, Raoul C Reulen
Background: Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer. Methods: We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated. Results: Overall, 301 STS developed compared with 19 expected (SIR = 15...
November 20, 2017: Journal of the National Cancer Institute
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
#4
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
#5
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
#6
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
#7
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
#8
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/29127250/malignant-peripheral-nerve-sheath-tumours-of-the-pericardium-in-a-patient-with-neurofibromatosis-type-1-the-diagnostic-value-of-18f-fdg-pet-ct-and-i-123-mibg-spect-ct
#9
Mathieu Charest, Josephine Pressacco, Jaramie Thomas-Gittens
A 25 year old female with known neurofibromatosis type 1 with a large anterior mediastinal mass was investigated. F18-FDG PET-CT revealed a radiotracer avid anterior mediastinal mass with SUVmax of 4.3 and demonstrating a hypoactive center. The Iodine-123 MIBG SPECT-CT study performed subsequently did not demonstrate any uptake, thereby excluding for the most part the diagnoses of paraganglioma or neuroblastoma. At final pathology, a malignant peripheral nerve sheath tumour (MPNST) of the pericardium with areas of chondrosarcomatous and angiosarcomatous differentiation was diagnosed...
November 10, 2017: Journal of Nuclear Medicine Technology
https://www.readbyqxmd.com/read/29118384/the-genomic-landscape-of-malignant-peripheral-nerve-sheath-tumors-diverse-drivers-of-ras-pathway-activation
#10
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
#11
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/29110923/malignant-triton-tumor-of-the-gluteal-region-in-a-patient-unaffected-by-neurofibromatosis-a-case-report
#12
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/29076075/proximal-type-epithelioid-sarcoma-in-skull-base-a-pathological-diagnosis-challenge-with-other-intracranial-tumors
#13
Duan Zejun, Yao Kun, Lu Dehong, Qi Xueling
Proximal-type ES (PES) is a rare and aggressive sarcoma originated from soft tissues with uncertain differentiation. It mainly affects middle-aged patients and often locates in proximal extremity and deep-seated tissues. Only one case of PES located in the skull base has been reported to date. Herein, we report two cases of PES occurred in the middle cranial fossa in two middle-aged Chinese women. Microscopically, the tumors were consisted of epithelial-like cells with or without rhabdoid cells. And frequent mitotic activity and coagulation necrosis were present...
October 26, 2017: Pathology Oncology Research: POR
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
#14
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
#15
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
#16
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/28973692/malignant-peripheral-nerve-sheath-tumors-are-not-a-feature-of-neurofibromatosis-type-2-in-the-unirradiated-patient
#17
Andrew T King, Scott A Rutherford, Charlotte Hammerbeck-Ward, Simon K Lloyd, Simon R Freeman, Omar N Pathmanaban, Mark Kellett, Rupert Obholzer, Shazia Afridi, Patrick Axon, Dorothy Halliday, Allyson Parry, Owen M Thomas, Roger D Laitt, Martin G McCabe, Stavros Stivaros, Sara Erridge, D Gareth Evans
BACKGROUND: The published literature suggests that malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in neurofibromatosis type 2 (NF2). A recent review based on incidence data in North America showed that 1 per 1000 cerebellopontine angle nerve sheath tumors were malignant. OBJECTIVE: To determine whether MPNST occurred spontaneously in NF2 by reviewing our NF2 database. METHODS: The prospective database consists of 1253 patients with NF2...
July 20, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28926683/salvage-rates-and-prognostic-factors-after-relapse-in-children-and-adolescents-with-malignant-peripheral-nerve-sheath-tumors
#18
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/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
#19
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
#20
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
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