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Peripheral nerve sheath tumor

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https://www.readbyqxmd.com/read/29220302/biology-and-management-of-undifferentiated-pleomorphic-sarcoma-myxofibrosarcoma-and-malignant-peripheral-nerve-sheath-tumors-state-of-the-art-and-perspectives
#1
Brigitte C Widemann, Antoine Italiano
Undifferentiated pleomorphic sarcomas, myxofibrosarcomas, and malignant peripheral nerve sheath tumors are characterized by complex genomic characteristics and aggressive clinical behavior. Recent advances in the understanding of the pathogenesis of these tumors may allow for the development of more-effective innovative therapeutic strategies, including immunotherapies. This review describes the current knowledge of the epidemiology, clinical presentation, treatment, and pathogenesis of these tumors and highlights ongoing and future research...
December 8, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29214122/peripheral-nerve-sheath-tumors-of-the-upper-extremity-and-hand-in-patients-with-neurofibromatosis-type-1-topography-of-tumors-and-evaluation-of-surgical-treatment-in-62-patients
#2
Reinhard E Friedrich, Caroline Diekmeier
Objective: Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome with a tendency to develop peripheral nerve sheath tumors (PNST). Plexiform neurofibromas (PNF) are detected in a high proportion of affected patients. The tumors can lead to severe disfigurement and are classified as precancerous. This study examines the surgical procedures that have been performed on large PNST of the upper limb and hand, and investigates whether a specific distribution pattern of the tumors can be detected in surgically treated cases...
2017: GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW
https://www.readbyqxmd.com/read/29206885/does-elevated-glucose-metabolism-correlate-with-higher-cell-density-in-neurofibromatosis-type-1-associated-peripheral-nerve-sheath-tumors
#3
Dominik Berzaczy, Marius E Mayerhoefer, Amedeo A Azizi, Alexander R Haug, Daniela Senn, Dietrich Beitzke, Michael Weber, Tatjana Traub-Weidinger
PURPOSE: To investigate whether elevated glucose metabolism in neurofibroma, determined by [F18]-FDG-PET, is correlated with cell density in MRI, as expressed through the apparent diffusion coefficient. MATERIALS AND METHODS: Patients diagnosed with neurofibromatosis type 1 and peripheral nerve sheath tumors (PNST) were enrolled in this prospective, IRB-approved study. After a single [F18]-FDG injection, patients consecutively underwent [F18]-FDG-PET/CT and [F18]-FDG-PET/MRI on the same day...
2017: PloS One
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
#4
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/29187472/pilomatrixoma-of-the-neck-shoulder-region-mimicking-a-rapidly-growing-neoplasm-of-peripheral-nerve-sheath-origin-in-neurofibromatosis-type-1
#5
Reinhard E Friedrich, Ulrich Schüller, Christian Hagel
Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary disorder. Neurofibroma is the most common neoplasm of this disease. This lesion is characterized by circumscribed soft or knotty skin tumors derived from peripheral nerve sheath cells. Numerous other neoplasms have been described for this tumor predisposition syndrome. This case report adds the diagnostic and therapeutic procedures in the case of an NF1 patient in whom the rapidly growing, nodular, subcutaneous tumor initially led to the suspicion of a malignant neoplasm...
December 2017: Anticancer Research
https://www.readbyqxmd.com/read/29179472/bcrp-expression-in-schwannoma-plexiform-neurofibroma-and-mpnst
#6
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/29174469/boundary-cap-cells-in-development-and-disease
#7
REVIEW
Katarzyna J Radomska, Piotr Topilko
Broad plasticity of the peripheral glia is an emerging concept during development of the peripheral nervous system (PNS). Recent studies have identified the neural crest-derived boundary caps (BCs), as a multitask stem cell population of the developing PNS. BC progeny migrate along the nerves to provide the major glial component of nerve roots and nerve terminals in the skin. Strikingly, those two locations constitute the privileged sites for development of benign peripheral nerve sheath tumors called neurofibromas in patients with neurofibromatosis type 1 (NF1), making BCs attractive candidates for the 'cell of origin' of this disease...
November 22, 2017: Current Opinion in Neurobiology
https://www.readbyqxmd.com/read/29165710/risk-of-soft-tissue-sarcoma-among-69-460-five-year-survivors-of-childhood-cancer-in-europe
#8
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/29162506/expression-of-the-braf-l597q-mutation-in-sporadic-neurofibromas-of-the-upper-extremity
#9
Ashkaun Shaterian, Daniela Bota, Amber Leis
The pathogenesis of neurofibromas is poorly understood outside of neurofibromatosis (NF). The BRAF proto-oncogene has been implicated in malignant peripheral nerve sheath tumors (PNSTs), however its role in neurofibromas has not been described. In this study, we identify a BRAF mutation in a 61-year old non-NF patient with a history of sporadic and recurrent neurofibromas localized to the right upper extremity. Despite repeat resections to establish local control, he developed median nerve neuropathy secondary to a 13×4cm plexiform neurofibroma...
November 18, 2017: Experimental and Molecular Pathology
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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