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Peripheral nerve sheath tumor

Verena Stahn, Inga Nagel, Susan Fischer-Huchzermeyer, Florian Oyen, Reinhard Schneppenheim, Stefan Gesk, Axel Bohring, Levan Chikobava, Peter Young, Burkhard Gess, Mathias Werner, Volker Senner, Anja Harder
Neurofibromas and schwannomas are benign Schwann cell-derived peripheral nerve sheath tumors arising sporadically and within neurofibromatoses. Multiple tumors are a hallmark of neurofibromatosis type 1 (NF1) and type 2 (NF2) and schwannomatosis. Neurofibromas in NF1 and schwannomas in NF2 or schwannomatosis are defined by distinctive molecular hits. Among these, multiple hybrid neurofibromas/schwannomas may also appear, not yet being defined by a molecular background. We therefore performed molecular analysis of 22 hybrid neurofibromas/schwannomas using array comparative genomic hybridization, immunohistochemistry, quantitative RT-PCR, and functional analyses of cultured Schwann cells...
October 17, 2016: American Journal of Pathology
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
Marcelo P N Carvalho, Natalia C C A Fernandes, Viviane C Nemer, Ramiro N Dias Neto, Rodrigo H F Teixeira, Bruna S Miranda, Maria J Mamprim, José L Catão-Dias, Rodrigo A Réssio
Peripheral nerve sheath tumors are a heterogeneous group of neoplasms that comprise neurofibromas, schwannomas, neurilemmomas, and perineuromas. In animals, peripheral nerve sheath neoplasms are most commonly diagnosed in dogs and cattle, followed by horses, goats, and cats, but their occurrence is uncommon in birds. An adult, free-living, male toco (common) toucan ( Ramphastos toco ) was admitted to the zoo animal clinic with weight loss, dehydration, and presence of a soft nodule adhered to the medial portion of the left pectoral muscle...
September 2016: Journal of Avian Medicine and Surgery
Ayca Ersen, Melike Pekmezci, Andrew L Folpe, Tarik Tihan
No abstract text is available yet for this article.
October 12, 2016: Pathology Oncology Research: POR
Shao-Yan Xu, Ke Sun, Kwabena Gyabaah Owusu-Ansah, Hai-Yang Xie, Lin Zhou, Shu-Sen Zheng, Wei-Lin Wang
Schwannomas are mesenchymal tumors originating from Schwann cells in peripheral nerve sheaths. Although the tumor can be located in any part of the human body, the most common locations are the head, neck, trunk and extremities. Pancreatic schwannomas are rare. To our knowledge, only 64 cases of pancreatic schwannoma have been reported in the English literature over the past 40 years. In this paper, we present a pancreatic schwannoma in a 59-year-old female. Ultrasound, computed tomography and magnetic resonance imaging revealed the tumor located in the pancreatic body; however, accurate diagnosis was hard to obtain preoperatively and a pancreatic cystadenoma was preliminarily considered...
October 7, 2016: World Journal of Gastroenterology: WJG
Sameer Agnihotri, Shahrzad Jalali, Mark R Wilson, Arnavaz Danesh, Mira Li, George Klironomos, Jonathan R Krieger, Alireza Mansouri, Osaama Khan, Yasin Mamatjan, Natalie Landon-Brace, Takyee Tung, Mark Dowar, Tiantian Li, Jeffrey P Bruce, Kelly E Burrell, Peter D Tonge, Amir Alamsahebpour, Boris Krischek, Pankaj Kumar Agarwalla, Wenya Linda Bi, Ian F Dunn, Rameen Beroukhim, Michael G Fehlings, Vera Bril, Stefano M Pagnotta, Antonio Iavarone, Trevor J Pugh, Kenneth D Aldape, Gelareh Zadeh
Schwannomas are common peripheral nerve sheath tumors that can cause debilitating morbidities. We performed an integrative analysis to determine genomic aberrations common to sporadic schwannomas. Exome sequence analysis with validation by targeted DNA sequencing of 125 samples uncovered, in addition to expected NF2 disruption, recurrent mutations in ARID1A, ARID1B and DDR1. RNA sequencing identified a recurrent in-frame SH3PXD2A-HTRA1 fusion in 12/125 (10%) cases, and genomic analysis demonstrated the mechanism as resulting from a balanced 19-Mb chromosomal inversion on chromosome 10q...
October 10, 2016: Nature Genetics
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
Tarun Dusad, D S Meena, Narendra Saini, Yogesh Sharma, Deepak Khurana
INTRODUCTION: Schwannomas are also known as neurilemmoma that usually originate from Schwann cells located in the peripheral nerve sheaths. It usually occurs in the age group of 20 to 70 years. These are the commonest tumors of the peripheral nerves, 5% of which occur in the adults and 19% of the tumors occur in upper extremities. Schwannomas are generally presented as an asymptomatic mass. Discomfort may be the only presenting complaint of the patient. Paresthesia may be elicited on tapping the swelling...
April 2016: Journal of Orthopaedic Case Reports
Corrine R Davis, Beth A Valentine, Emma Gordon, Sean P McDonough, Paula A Schaffer, Andrew L Allen, Patricia Pesavento
A diagnosis of neoplasia was noted in 125 of 357 donkeys (35%) in our review of medical records from 5 veterinary schools in the United States and Canada. Equine sarcoid was the most common tumor in our study, accounting for 72% of all tumors and 82% of cutaneous tumors. Soft-tissue sarcomas were the second most common skin tumors. All other types of neoplasia were rare. Important differences in the occurrence of neoplasia in donkeys compared to horses included the rarity or absence of squamous cell carcinoma in any organ system and gray horse melanoma...
November 2016: Journal of Veterinary Diagnostic Investigation
Christopher R Jackson, Eugen C Minca, Jyoti P Kapil, Steven Christopher Smith, Steven D Billings
Malignant peripheral nerve sheath tumors are rare soft tissue sarcomas with histological and immunohistochemical similarities to spindle cell melanoma. Although spindle cell melanoma is significantly more common, both tumors may express S100 and lack staining for HMB-45, Melan-A, or MITF. Here we present a case of superficial malignant peripheral nerve sheath tumor with diffuse S100 positivity arising in a subtle neurofibroma in close proximity to an intradermal melanocytic nevus. This configuration had led to prior misdiagnosis as a desmoplastic melanoma arising in the nevus and to sentinel lymph node biopsy...
September 30, 2016: Journal of Cutaneous Pathology
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
Elizabeth Shurell, Maria E Vergara-Lluri, Yunfeng Li, Crompton Joe, Arun Singh, Nicholas Bernthal, Hong Wu, Fritz C Eilber, Sarah M Dry
BACKGROUND: Immunotherapy targeting cancer-testis antigen NY-ESO-1 shows promise for tumors with poor response to chemoradiation. Malignant peripheral nerve sheath tumors (MPNSTs) and liposarcomas (LPS) are chemoresistant and have few effective treatment options. MATERIALS METHODS: Using a comprehensive tissue microarray (TMA) of both benign and malignant tumors in primary, recurrent, and metastatic samples, we examined NY-ESO-1 expression in peripheral nerve sheath tumor (PNST) and adipocytic tumors...
September 17, 2016: Oncotarget
Eunmyong Lee, Yongjie Wei, Zhongju Zou, Kathryn Tucker, Dinesh Rakheja, Beth Levine, James F Amatruda
Autophagy is an evolutionarily conserved lysosomal degradation pathway that plays an essential role in enabling eukaryotic organisms to adapt to nutrient deprivation and other forms of environmental stress. In metazoan organisms, autophagy is essential for differentiation and normal development; however, whether the autophagy pathway promotes or inhibits tumorigenesis is controversial, and the possible mechanisms linking defective autophagy to cancer remain unclear. To determine if autophagy is important for tumor suppression, we inhibited autophagy in transgenic zebrafish via stable, tissue-specific expression of a dominant-negative autophagy protein Atg5K130R...
September 16, 2016: Oncotarget
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
Masayuki Oki, Tomihisa Nanao, Takuma Shinoda, Ayumi Tsuda, Atsushi Yasuda, Toshiro Seki, Hideki Ozawa, Naoya Nakamura, Atsushi Takagi
To date, there are only 15 case reports of lymphoma in patients with neurofibromatosis type 1 (NF1), a common autosomal dominant tumor predisposition syndrome. Here, we present the first report of a primary effusion lymphoma (PEL)-like lymphoma (PEL-L), which is a human herpes virus 8/Kaposi sarcoma herpes virus-unrelated PEL, in a 73-year-old woman with NF1. The woman presented with pleural effusion following surgery for a small intestinal gastrointestinal stromal tumor and a malignant peripheral nerve sheath tumor...
2016: Tokai Journal of Experimental and Clinical Medicine
Philip R Cohen
BackgroundSegmental neurofibromatosis, referred to as mosaic neurofibromatosis 1, patients present with neurofibromas or café au lait macules or both in a unilateral segment of the body.PurposeA woman with segmental neurofibromatosis and triple cancer (renal cell carcinoma, mixed thyroid carcinoma, and lentigo maligna) is described and cancers observed in patients with segmental neurofibromatosis are reviewed.MethodsPubMed was used to search the following terms, separately and in combination: cancer, malignancy, mosaic, neoplasm, neurofibroma, neurofibromatosis, segment, segmental, tumor...
2016: Dermatology Online Journal
Hiroshi Urakawa, Eiji Kozawa, Kunihiro Ikuta, Shunsuke Hamada, Naoki Ishiguro, Yoshihiro Nishida
The aim of this study is to examine the impact of disease free (DF) status on the prognosis in patients with metastatic non-small round cell soft tissue sarcoma (STS). We retrospectively reviewed 51 metastatic STS patients who were treated in Nagoya University Hospital from 2005 to 2015. The relation between various clinical factors and overall survival (OS) was analyzed. The log rank test and Cox's proportional hazards test were used to evaluate the differences between groups. p-values of <0.05 were considered to indicate significance...
September 7, 2016: Clinical & Experimental Metastasis
G A Frank, Yu Yu Andreeva, L V Moskvina, G D Efremov, S I Samoilova
The paper reviews the 2016 WHO classification of prostate tumors, notes the alterations made, and describes approaches to the diagnosis of cancer types and grades. It also gives original photomicrographs from the authors' collection. The main alterations were as follows: - The types of prostate adenocarcinoma were added by pleomorphic giant-cell carcinoma; oncocytic (8290/3) and lymphoepithelial (8082/3) carcinomas were excluded. - Grade III prostatic intraepithelial neoplasia (PIN) was substituted for high grade PIN (8148/2)...
July 2016: Arkhiv Patologii
Yasha Parikh, Kush J Sharma, Samir J Parikh, Diane Hall
Schwannomas are benign tumors arising from the peripheral nerve sheath, commonly occurring in the head, neck, and extensor surfaces of the extremities. They can be associated with neurofibromatosis type II. Our case describes a 48-year-old woman with a 2-week history of a left-sided palpable breast mass. She was referred to radiology, where additional imaging revealed a 1.1-cm mass. A biopsy was performed; histology revealed an intramammary schwannoma. Mammography findings include a well-defined mass without calcification...
September 2016: Radiology case reports
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