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https://www.readbyqxmd.com/read/28213670/emerging-genotype-phenotype-relationships-in-patients-with-large-nf1-deletions
#1
REVIEW
Hildegard Kehrer-Sawatzki, Victor-Felix Mautner, David N Cooper
The most frequent recurring mutations in neurofibromatosis type 1 (NF1) are large deletions encompassing the NF1 gene and its flanking regions (NF1 microdeletions). The majority of these deletions encompass 1.4-Mb and are associated with the loss of 14 protein-coding genes and four microRNA genes. Patients with germline type-1 NF1 microdeletions frequently exhibit dysmorphic facial features, overgrowth/tall-for-age stature, significant delay in cognitive development, large hands and feet, hyperflexibility of joints and muscular hypotonia...
February 17, 2017: Human Genetics
https://www.readbyqxmd.com/read/28184331/verticillin-a-inhibits-leiomyosarcoma-and-malignant-peripheral-nerve-sheath-tumor-growth-via-induction-of-apoptosis
#2
A Zewdu, G Lopez, D Braggio, C Kenny, D Constantino, H K Bid, K Batte, O H Iwenofu, N H Oberlies, C J Pearce, A M Strohecker, D Lev, R E Pollock
OBJECTIVE: The heterogeneity of soft tissue sarcoma (STS) represents a major challenge for the development of effective therapeutics. Comprised of over 50 different histology subtypes of various etiologies, STS subsets are further characterized as either karyotypically simple or complex. Due to the number of genetic anomalies associated with genetically complex STS, development of therapies demonstrating potency against this STS cluster is especially challenging and yet greatly needed...
November 2016: Clinical & Experimental Pharmacology
https://www.readbyqxmd.com/read/28147331/aurora-a-kinase-inhibition-enhances-oncolytic-herpes-virotherapy-through-cytotoxic-synergy-and-innate-cellular-immune-modulation
#3
Mark A Currier, Les Sprague, Tilat A Rizvi, Brooke Nartker, Chun-Yu Chen, Pin-Yi Wang, Brian J Hutzen, Meghan R Franczek, Ami V Patel, Katherine E Chaney, Keri A Streby, Jeffrey A Ecsedy, Joe Conner, Nancy Ratner, Timothy P Cripe
Malignant peripheral nerve sheath tumor (MPNST) and neuroblastoma models respond to the investigational small molecule Aurora A kinase inhibitor, alisertib. We previously reported that MPNST and neuroblastomas are also susceptible to oncolytic herpes virus (oHSV) therapy. Herein, we show that combination of alisertib and HSV1716(HSV1716), a virus derived from HSV-1 and attenuated by deletion of RL1, exhibits significantly increased antitumor efficacy compared to either monotherapy. Alisertib and HSV1716 reduced tumor growth and increased survival in two xenograft models of MPNST and neuroblastoma...
January 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28124441/confirmation-of-mutation-landscape-of-nf1-associated-malignant-peripheral-nerve-sheath-tumors
#4
Pierre Sohier, Armelle Luscan, Angharad Lloyd, Kevin Ashelford, Ingrid Laurendeau, Audrey Briand-Suleau, Dominique Vidaud, Nicolas Ortonne, Eric Pasmant, Meena Upadhyaya
The commonest tumors associated with neurofibromatosis type 1 (NF1) are benign peripheral nerve sheath tumors, called neurofibromas. Malignant transformation of neurofibromas into aggressive MPNSTs may occur with a poor patient prognosis. A co-operative role of SUZ12 or EED inactivation, along with NF1, TP53, and CDKN2A loss-of-function, has been proposed to drive progression to MPNSTs. An exome sequencing analysis of eight MPNSTs, one plexiform neurofibroma, and seven cutaneous neurofibromas was undertaken...
January 25, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28103882/a-distal-ileum-malignant-peripheral-nerve-sheath-tumor-causing-intussusception-in-a-patient-in-china-a-case-report
#5
Lin-Bo Zhu, Peng-Fei Li, Wei-Hua Xiao, Peng-Bin Zhang, Jun-Qiang Li, Ming-Fei Sun
BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures. CASE PRESENTATION: Here, we report the first Chinese case of a malignant peripheral nerve sheath tumor of the distal ileum presenting as intussusception...
January 19, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28078568/rediagnosing-one-of-smith-s-patients-john-mccann-with-neuromas-tumours-1849
#6
Martino Ruggieri, Andrea D Praticò, Rosario Caltabiano, Agata Polizzi
In 1849, the Irish Professor of Surgery, Sir Robert William Smith, by publishing his "Treatise on the Pathology, Diagnosis and Treatment of Neuroma", collected six previous examples of "general development of neuromatous tumours" and reported three further cases (two personal and one referred) of what is nowadays known as neurofibromatosis. Among these latter cases, there was a 35-year-old cattle-driver, John McCann, who was first admitted at hospital in 1840 because of a large tumour on the right side of his neck thought to be malignant (and a second tumour sublingually) but not operated...
January 11, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28067315/kank1-inhibits-cell-growth-by-inducing-apoptosis-though-regulating-cxxc5-in-human-malignant-peripheral-nerve-sheath-tumors
#7
Zhibin Cui, Yingjia Shen, Kenny H Chen, Suresh K Mittal, Jer-Yen Yang, GuangJun Zhang
Malignant peripheral nerve sheath tumors (MPNSTs) are a type of rare sarcomas with a poor prognosis due to its highly invasive nature and limited treatment options. Currently there is no targeted-cancer therapy for this type of malignancy. Thus, it is important to identify more cancer driver genes that may serve as targets of cancer therapy. Through comparative oncogenomics, we have found that KANK1 was a candidate tumor suppressor gene (TSG) for human MPNSTs. Although KANK1 is known as a cytoskeleton regulator, its tumorigenic function in MPNSTs remains largely unknown...
January 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28065690/medium-throughput-biochemical-compound-screening-identifies-novel-agents-for-pharmacotherapy-of-neurofibromatosis-type-i
#8
Galina Semenova, Dina Stepanova, Sergey M Deyev, Jonathan Chernoff
The variable manifestation of phenotypes that occur in patients with neurofibromatosis type 1 (NF1) includes benign and malignant neurocutaneous tumors for which no adequate treatment exists. Cell-based screening of known bioactive compounds library identified the protein phosphatase 2A (PP2A) inhibitor Cantharidin and the L-type calcium channel blocker Nifedipine as potential candidates for NF1 pharmacotherapy. Validation of screening results using human NF1-associated malignant peripheral nerve sheath tumor (MPNST) cells showed that Cantharidin effectively impeded MPNST cell growth, while Nifedipine treatment significantly decreased local tumor growth in an MPNST xenograft animal model...
January 5, 2017: Biochimie
https://www.readbyqxmd.com/read/28055968/bh3-mimetics-suppress-cxcl12-expression-in-human-malignant-peripheral-nerve-sheath-tumor-cells
#9
Christopher D Graham, Niroop Kaza, Hawley C Pruitt, Lauren M Gibson, Barbara J Klocke, Lalita A Shevde, Steven L Carroll, Kevin A Roth
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, Schwann cell-derived neoplasms of the peripheral nervous system that have recently been shown to possess an autocrine CXCL12/CXCR4 signaling loop that promotes tumor cell proliferation and survival. Importantly, the CXCL12/CXCR4 signaling axis is driven by availability of the CXCL12 ligand rather than CXCR4 receptor levels alone. Therefore, pharmacological reduction of CXCL12 expression could be a potential chemotherapeutic target for patients with MPNSTs or other pathologies wherein the CXCL12/CXCR4 signaling axis is active...
January 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/28052660/outcomes-of-treatment-for-malignant-peripheral-nerve-sheath-tumors-different-clinical-features-associated-with-neurofibromatosis-type-1
#10
In Kyung Hwang, Seung Min Hahn, Hyo Sun Kim, Sang Kyum Kim, Hyo Song Kim, Kyoo-Ho Shin, Chang Ok Suh, Chuhl Joo Lyu, Jung Woo Han
Purpose: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of sarcoma that occur spontaneously or in association with neurofibromatosis type 1 (NF-1). This study aimed to clinically differentiate these types of MPNSTs. Materials and Methods: The study reviewed 95 patients diagnosed with and treated for MPNST at Yonsei University Health System, Seoul, Korea over a 27-year period. The clinical characteristics, prognostic factors, and treatment outcomes of sporadic (sMPNST) and NF-1 associated MPNST (NF-MPNST) cases were compared...
December 1, 2016: Cancer Research and Treatment: Official Journal of Korean Cancer Association
https://www.readbyqxmd.com/read/28002805/expression-of-psma-in-tumor-neovasculature-of-high-grade-sarcomas-including-synovial-sarcoma-rhabdomyosarcoma-undifferentiated-sarcoma-and-mpnst
#11
Birthe Heitkötter, Marcel Trautmann, Inga Grünewald, Martin Bögemann, Kambiz Rahbar, Heidrun Gevensleben, Eva Wardelmann, Wolfgang Hartmann, Konrad Steinestel, Sebastian Huss
AIMS: PSMA (prostate specific membrane antigen) is physiologically expressed in normal prostate tissue. It is overexpressed in prostate cancer cells and has been suggested as a target for antibody-based radioligand therapy. As PSMA expression so far has not been systematically analyzed in soft tissue tumors, the current study aims at investigating a large cohort of different subtypes. METHODS AND RESULTS: Immunohistochemistry was used to detect PSMA expression in 779 samples of soft tissue tumors and Ewing sarcoma as a primary bone malignancy...
December 16, 2016: Oncotarget
https://www.readbyqxmd.com/read/27904856/primary-hepatic-malignant-peripheral-nerve-sheath-tumor-successfully-treated-with-combination-therapy-a-case-report-and-literature-review
#12
Hae Il Jung, Hyoung Uk Lee, Tae Sung Ahn, Jong Eun Lee, Hyun Yong Lee, Hyon Doek Cho, Sang Cheol Lee, Sang Ho Bae
Primary malignant peripheral nerve sheath tumor (MPNST) in a young female patient, not associated with neurofibromatosis type-I is extremely rare in the liver. A 33-year-old female was admitted with a right flank pain for a weak. The CT scan showed 12.5-cm-sized mass located at the right hepatic lobe. At laparotomy, about 20.0-cm-sized mass was on the right hepatic lobe with attachment to right diaphragmatic pleura. Right hepatic lobe and adherent part of diaphragmatic pleura were resected. On histology and immunohistochemistry, it was diagnosed MPNST...
December 2016: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/27879444/histologic-and-immunohistochemical-analyses-of-soft-tissue-sarcomas-from-brca2-mutant-tp53-mutant-zebrafish-are-consistent-with-neural-crest-schwann-cell-origin
#13
L A White, J M Sexton, H R Shive
The zebrafish ( Danio rerio) provides a powerful model for analyzing genetic contributors to cancer. Multiple zebrafish lines with cancer-associated genetic mutations develop soft tissue sarcomas that are histologically consistent with malignant peripheral nerve sheath tumor (MPNST). The goal of this study was to determine the phenotype of soft tissue sarcomas in a brca2-mutant/ tp53-mutant zebrafish line using immunohistochemical markers that are commonly expressed in mammalian MPNST. We classified 70 soft tissue sarcomas from a brca2-mutant/ tp53-mutant zebrafish cohort as MPNST, undifferentiated sarcoma, or other tumor based on histologic features...
March 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/27875628/clinical-genomic-profiling-identifies-tyk2-mutation-and-overexpression-in-patients-with-neurofibromatosis-type-1-associated-malignant-peripheral-nerve-sheath-tumors
#14
Angela C Hirbe, Madhurima Kaushal, Mukesh Kumar Sharma, Sonika Dahiya, Melike Pekmezci, Arie Perry, David H Gutmann
BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that arise at an estimated frequency of 8% to 13% in individuals with neurofibromatosis type 1 (NF1). Compared with their sporadic counterparts, NF1-associated MPNSTs (NF1-MPNSTs) develop in young adults, frequently recur (approximately 50% of cases), and carry a dismal prognosis. As such, most individuals affected with NF1-MPNSTs die within 5 years of diagnosis, despite surgical resection combined with radiotherapy and chemotherapy...
November 22, 2016: Cancer
https://www.readbyqxmd.com/read/27872789/malignant-peripheral-nerve-sheath-tumor-of-prostate-a-rare-case-report-and-literature-review
#15
Kun-Lin Hsieh, Chih-Cheng Lu, Chien-Feng Li, Yin-Hsun Feng, Alex C Liao
A mid-aged male presented with progressive lower urinary tract symptoms (LUTS) for years. Huge prostate with low serum prostate-specific antigen (PSA) level was detected. The specimen from transurethral resection revealed surprising pathology finding as malignant peripheral nerve sheath tumor (MPNST). Considering its huge size (more than 300 gm) and location, we prescribed neoadjuvant chemotherapy firstly. The tumor became regressive and then radical surgical resection was achieved. Adjuvant multimodality treatment including concurrent chemoradiotherapy (CCRT) and target therapy was given...
2016: Case Reports in Urology
https://www.readbyqxmd.com/read/27872787/synchronous-malignant-peripheral-nerve-sheath-tumor-and-adenocarcinoma-of-the-prostate-case-report-and-literature-review
#16
Nikolaos Ferakis, Antonios Katsimantas, Konstantinos Bouropoulos, Antonios Farmakis
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) of the prostate are extremely rare. A very unusual case of simultaneous adenocarcinoma and MPNST of the prostate is reported. A 60-year-old Caucasian male presented for annual urologic examination. Digital rectal examination revealed a painless, toughish, and asymmetrically enlarged prostate. Serum prostate-specific antigen was 1 ng/mL. Radiologic examinations demonstrated a large mass, which was arising from the left peripheral lobe of the prostate. The patient underwent transrectal ultrasound-guided biopsy of the prostate which revealed a smooth muscle tumor of uncertain malignant potential...
2016: Case Reports in Urology
https://www.readbyqxmd.com/read/27834629/malignant-peripheral-nerve-sheath-tumors-of-the-spine-results-of-surgical-management-from-a-multicenter-study
#17
Dean Chou, Mark H Bilsky, Alessandro Luzzati, Charles G Fisher, Ziya L Gokaslan, Laurence D Rhines, Mark B Dekutoski, Michael G Fehlings, Ravi Ghag, Peter Varga, Stefano Boriani, Niccole M Germscheid, Jeremy J Reynolds
OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft-tissue sarcomas. Resection is the mainstay of treatment and the most important prognostic factor. However, complete resection of spinal MPNSTs with tumor-free margins is challenging due to the likelihood of residual tumor cells. The objective of this study was to describe whether the type of Enneking resection in the management of spinal MPNSTs had an effect on local recurrence and survival. METHODS The AOSpine Knowledge Forum Tumor developed a multicenter database that includes demographic, diagnostic, therapeutic, local recurrence, and survival data on patients with primary spinal column tumors...
November 11, 2016: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/27833160/photothermal-therapy-improves-the-efficacy-of-a-mek-inhibitor-in-neurofibromatosis-type-1-associated-malignant-peripheral-nerve-sheath-tumors
#18
Elizabeth E Sweeney, Rachel A Burga, Chaoyang Li, Yuan Zhu, Rohan Fernandes
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive tumors with low survival rates and the leading cause of death in neurofibromatosis type 1 (NF1) patients under 40 years old. Surgical resection is the standard of care for MPNSTs, but is often incomplete and can generate loss of function, necessitating the development of novel treatment methods for this patient population. Here, we describe a novel combination therapy comprising MEK inhibition and nanoparticle-based photothermal therapy (PTT) for MPNSTs...
November 11, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27816720/multivacuolated-mucin-filled-cells-a-unique-cell-characteristic-of-plexiform-neurofibroma-a-report-of-11-cases
#19
Michael Michal, Dmitry V Kazakov, Ladislav Hadravský, Květoslava Michalová, Boris Rychlý, Michal Michal
The authors present 11 cases of plexiform neurofibroma (PN) that featured a very characteristic type of cell appearing as multivacuolated mucin-filled cells (MMFC). The 11 cases were obtained after reviewing 109 cases of PN. Six out of 10 patients showed clinical features of neurofibromatosis type 1 (NF1). The size of PN ranged from 0.8 cm to 11.5 cm in the largest dimension. The lesions represented classical PN in all cases with myxoid, hypocellular stroma. The MMFC were found within the most myxoid tumorous nodules and were haphazardly located, typically featuring a variably sized, multivacuolated cytoplasm divided by a fine septa with a small polygonal nucleus on one side, which was often compressed or slightly indented by the cytoplasmic mucous substances...
November 2, 2016: Human Pathology
https://www.readbyqxmd.com/read/27761835/evaluating-the-effect-of-hdac8-inhibition-in-malignant-peripheral-nerve-sheath-tumors
#20
Gonzalo Lopez, Raphael E Pollock
Malignant peripheral nerve sheath tumor (MPNST) is a highly aggressive disease with a dismal prognosis. The disease can occur sporadically or in patients with inherited neurofibromatosis (NF-1). MPNST is typically resistant to therapeutic intervention. Hence, the need for improved therapies is warranted. Several broad spectrum histone deacetylase (HDAC) inhibitors have a high affinity for class I HDAC isoforms. Inhibition of multiple HDAC isoforms often results in undesirable side effects, while inhibiting a single isoform could possibly improve the therapeutic window and limit toxicity...
2017: Methods in Molecular Biology
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