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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
O Kalita, K Cwiertka, D Vrána, M Vaverka, L Tučková, M Megová
BACKGROUND: Malignant peripheral nerve sheath tumor schwannoma (MPNST), also known as malignant schwannoma, is a very rare tumor accounting for only 2% of all sarcomas. The prognosis is relatively poor, with a 5-year survival rate of 46-69%. The treatment of MPNST has not been standardized yet. Mainstay treatment is radical resection. Oncological adjuvant or neoadjuvant treatment has equivocal indications with unclear effects. CASE: The case report presents a 55-year-old patient who showed resistance in the medial-ventral area of the left lower limb...
2016: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
Kunihiro Ikuta, Takehiro Ota, Lisheng Zhuo, Hiroshi Urakawa, Eiji Kozawa, Shunsuke Hamada, Koji Kimata, Naoki Ishiguro, Yoshihiro Nishida
Hyaluronan (HA) has been shown to play important roles in the growth, invasion and metastasis of malignant tumors. Our previous study showing that high HA expression in malignant peripheral nerve sheath tumors (MPNST) is predictive of poor patient prognosis, prompted us to speculate that inhibition of HA synthesis in MPNST might suppress the tumorigenicity. The aim of our study was to investigate the antitumor effects of 4-methylumbelliferone (MU), an HA synthesis inhibitor, on human MPNST cells and tissues...
October 5, 2016: International Journal of Cancer. Journal International du Cancer
Weibo Pan, Bin Feng, Zhan Wang, Nong Lin, Zhaoming Ye
PURPOSE: Malignant peripheral nerve sheath tumors are extremely rare in the general population and display a predilection for metastasis to the lungs. Here, we present a rare case of a malignant peripheral nerve sheath tumor located in the paraspinal region and highlight the importance of preoperative biopsy in diagnosis of spinal epidural peripheral nerve sheath tumors. METHODS: We describe the clinical course of the patient as well as the radiological and pathological findings of the tumor...
September 27, 2016: European Spine Journal
Erika F Rodriguez, Jaishri Blakeley, Shannon Langmead, Alessandro Olivi, Anthony Tufaro, Abeer Tabbarah, Gail Berkenblit, Justin M Sacks, Scott D Newsome, Elizabeth Montgomery, Fausto J Rodriguez
Leptomeningeal dissemination of low grade Schwann cell neoplasms is an exceptionally rare occurrence, and has not been well documented in the literature. We encountered two cases of leptomeningeal dissemination of low grade Schwann cell neoplasms. Patient 1 was a 63-year-old woman with NF1 and a progressive low grade MPNST developing from a diffuse/plexiform orbital neurofibroma that arose in childhood. The neoplasm demonstrated local and leptomeningeal dissemination intracranially leading to the patient's death...
September 22, 2016: Human Pathology
Ushio Hanai, Tadashi Akamatsu, Megumi Kobayashi, Yotaro Tsunoda, Kenichi Hirabayashi, Tanehumi Baba, Hideki Atsumi, Mitsunori Matsumae
INTRODUCTION: The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely. We experienced a case of MPNST in the occipital region with NF-1. CASE REPORT: A 59-year-old woman presented with NF-1 and an MPNST of the occipital region. We performed wide excision involving the occipital bone, and reconstructed with a titanium plate and a free latissimus dorsi muscle flap...
2016: Tokai Journal of Experimental and Clinical Medicine
Humain Baharvahdat, Babak Ganjeifar, Nema Mohamadian Roshan, Aslan Baradaran
Spinal Intradural primary malignant peripheral nerve sheath tumors (MPNST) are rare in patients without neurofibromatosis. Here we represent a 3- year-old girl of primary intradural spinal malignant peripheral nerve sheath tumor. The tumor was removed partially and MPNST was diagnosed in the histopathological exam. Her condition deteriorated due to acute hydrocephalus in the following days. In this article we discuss the clinical presentation, imaging, treatment, and prognosis of our patient and the other 22 patients of primary intradural MPNST, found in the literature...
March 4, 2016: Turkish Neurosurgery
Aaron W James, Elizabeth Shurell, Arun Singh, Sarah M Dry, Fritz C Eilber
Malignant peripheral nerve sheath tumor (MPNST) is the sixth most common type of soft tissue sarcoma. Most MPNSTs arise in association with a peripheral nerve or preexisting neurofibroma. Neurofibromatosis type is the most important risk factor for MPNST. Tumor size and fludeoxyglucose F 18 avidity are among the most helpful parameters to distinguish MPNST from a benign peripheral nerve sheath tumor. The histopathologic diagnosis is predominantly a diagnosis of light microscopy. Immunohistochemical stains are most helpful to distinguish high-grade MPNST from its histologic mimics...
October 2016: Surgical Oncology Clinics of North America
Elizabeth Shurell, Arun S Singh, Joseph G Crompton, Sarah Jensen, Yunfeng Li, Sarah Dry, Scott Nelson, Bartosz Chmielowski, Nicholas Bernthal, Noah Federman, Paul Tumeh, Fritz C Eilber
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma with few treatment options. Tumor immune state has not been characterized in MPNST, and is important in determining response to immune checkpoint blockade. Our aim was to evaluate the expression of programmed death-ligand 1 (PD-L1), programmed cell death protein 1 (PD-1), and presence of CD8+ tumor infiltrating lymphocytes (TILs) in MPNST, and correlate these findings with clinical behavior and outcome...
August 31, 2016: Oncotarget
Tomohito Hagi, Tomoki Nakamura, Ayumu Yokoji, Akihiko Matsumine, Akihiro Sudo
The present study reports a case of medullary metastasis without lung metastasis that occurred as a result of a malignant peripheral nerve sheath tumor (MPNST). An 81-year-old woman presented with a MPNST in the left brachial plexus, arising from the cervical nerve root. The patient underwent carbon ion radiotherapy; however, tumor recurrence was identified in the left shoulder. Subsequently, the patient underwent wide excision. Three weeks subsequent to surgery, imbalance and dysarthria developed suddenly...
September 2016: Oncology Letters
Naofumi Asano, Akihiko Yoshida, Hitoshi Ichikawa, Taisuke Mori, Masaya Nakamura, Akira Kawai, Nobuyoshi Hiraoka
AIMS: The diagnosis of a malignant peripheral nerve sheath tumor (MPNST) can be challenging, as the morphological criteria and existing immunohistochemical markers are not entirely specific. The recent discovery of frequent inactivation of polycomb repressive complex 2 in MPNSTs suggests that immunohistochemical detection of trimethylated lysine 27 of histone 3 (H3K27me3) could be of diagnostic aid. METHODS AND RESULTS: To clarify the utility of this marker, we performed immunostaining studies, using monoclonal (C36B11) and polyclonal antibodies in parallel...
August 31, 2016: Histopathology
Christopher D Graham, Niroop Kaza, Barbara J Klocke, G Yancey Gillespie, Lalita A Shevde, Steven L Carroll, Kevin A Roth
Glioblastomas (GBMs) are the most common and aggressive primary human malignant brain tumors. 4-Hydroxy tamoxifen (OHT) is an active metabolite of the tamoxifen (TMX) prodrug and a well-established estrogen receptor (ER) and estrogen-related receptor antagonist. A recent study from our laboratory demonstrated that OHT induced ER-independent malignant peripheral nerve sheath tumor (MPNST) cell death by autophagic degradation of the prosurvival protein Kirsten rat sarcoma viral oncogene homolog. Because both MPNST and GBM are glial in cell origin, we hypothesized that OHT could mediate similar effects in GBM...
August 11, 2016: Journal of Neuropathology and Experimental Neurology
Tatsuya Maegawa, Motohiro Hirasawa, Atsushi Sasahara, Shigeru Tani, Shinji Hagiwara, Hirokazu Koseki, Chika Yoshimura, Yuichi Takahashi, Asami Kikuchi, Hidetoshi Kasuya
UNLABELLED: <i> CASE: A</i> 30-year-old woman presented with posterior cervical pain and left-sided omalgia. The patient had a history of non-Hodgkin's lymphoma for which she had received prophylactic whole-brain irradiation(including at the upper cervical level)17 years previously. A magnetic resonance imaging(MRI)scan obtained 1 month previously showed an intradural extramedullary mass lesion at the left C1/2 level. We initially considered the tumor to be a benign schwannoma, but the patient subsequently developed left hemiparesis and was consequently admitted 2 days after her first visit...
August 2016: No Shinkei Geka. Neurological Surgery
Sumit Majumdar, Sreekanth Kotina, Nirujogi Mahesh, Divya Uppala, Singam Praveen Kumar
Malignant Peripheral Nerve Sheath Tumor (MPNST) is biologically an aggressive tumor that is usually found in the extremities, trunk and infrequently found in the head and neck area particularly in the jaws, arising from the cells allied with nerve sheath. Mandibular MPNST may either arise from a preexisting neurofibroma or develop de novo. Because of the greater variability from case to case in overall appearance both clinically and histologically, a case of MPNST of the mandible in a 25-year-old female patient is reported...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
M A Dickson, M R Mahoney, W D Tap, S P D'Angelo, M L Keohan, B A Van Tine, M Agulnik, L E Horvath, J S Nair, G K Schwartz
BACKGROUND: Aurora kinase A (AURKA) is commonly overexpressed in sarcoma. The inhibition of AURKA by shRNA or by a specific AURKA inhibitor blocks in vitro proliferation of multiple sarcoma subtypes. MLN8237 (alisertib) is a novel oral adenosine triphosphate-competitive AURKA inhibitor. PATIENTS AND METHODS: This Cancer Therapy Evaluation Program-sponsored phase II study of alisertib was conducted through the Alliance for Clinical Trials in Oncology (A091102). Patients were enrolled into histology-defined cohorts: (i) liposarcoma, (ii) leiomyosarcoma, (iii) undifferentiated sarcoma, (iv) malignant peripheral nerve sheath tumor, or (v) other...
October 2016: Annals of Oncology: Official Journal of the European Society for Medical Oncology
Sidra Ahsan, Yubin Ge, Michael A Tainsky
The clinical management of malignant peripheral nerve sheath tumors (MPNSTs) is challenging not only due to its aggressive and invasive nature, but also limited therapeutic options. Using gene expression profiling, our lab identified BMP2-SMAD1/5/8 pathway as a potential therapeutic target for treating MPNSTs. In this study, we explored the therapeutic impact of targeting BMP2-SMAD1/5/8 pathway in conjunction with RAS-MEK-ERK signaling, which is constitutively activated in MPNSTs. Our results indicated that single agent treatment with LDN-193189, a BMP2 Type I receptor inhibitor, did not affect the growth and survival of MPNST cells at biochemically relevant inhibitory concentrations...
August 3, 2016: Oncotarget
D H Ki, S He, S Rodig, A T Look
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, frequently metastatic sarcomas that are associated with neurofibromatosis type 1 (NF1), a prominent inherited genetic disease in humans. Although loss of the NF1 gene predisposes to MPNST induction, relatively long tumor latency in NF1 patients suggests that additional genetic or epigenetic abnormalities are needed for the development of these nerve sheath malignancies. To study the molecular pathways contributing to the formation of MPNSTs in NF1 patients, we used a zebrafish tumor model defined by nf1 loss in a p53-deficient background together with the overexpression of either wild-type or constitutively activated PDGFRA (platelet-derived growth factor receptor-α) under control of the sox10 neural crest-specific promoter...
August 1, 2016: Oncogene
Shalini Koppisetty, Ricardo C Alessio, Atul Rajpurkar
Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1), but can also occur without a history of NF (isolated MPNST). MPNSTs are most commonly located on the extremities (brachial and sacral plexus), head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney)...
July 2016: Urology Annals
Saravanaraja Muthusamy, Sheila A Conway, J David Pitcher, H Thomas Temple
Peripheral nerve sheath tumors (benign and malignant) usually arise in the soft tissues and are unusual in bone. Intraosseous peripheral nerve sheath tumors are usually benign and constitute approximately 0.2% of all bone tumors. Intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and usually result from secondary invasion. Only a few cases of primary intraosseous MPNSTs have been reported in published studies, and these were localized mostly in the mandible (approximately 50%) or maxilla, spine, and, occasionally, in the appendicular skeleton...
July 19, 2016: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
Jed J Kendall, Katherine E Chaney, Ami V Patel, Tilat A Rizvi, David A Largaespada, Nancy Ratner
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that are a major cause of mortality of Neurofibromatosis type 1 (NF1) patients. MPNST patients have few therapeutic options available and only complete surgical resection can be curative. MPNST formation and survival are dependent on activated β-catenin signaling. The goal of this study was to determine if inhibition of the CK2 enzyme can be therapeutically exploited in MPNSTs, given CK2's role in mainta ining oncogenic phenotypes including stabilization of β-catenin...
July 18, 2016: Oncotarget
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