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Malignant Peripheral Nerve Sheath Tumor

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https://www.readbyqxmd.com/read/27904856/primary-hepatic-malignant-peripheral-nerve-sheath-tumor-successfully-treated-with-combination-therapy-a-case-report-and-literature-review
#1
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/27900106/giant-intrapelvic-malignant-peripheral-nerve-sheath-tumor-mimicking-disc-herniation-a-case-report
#2
Peng Wang, Cong Chen, Xiaotang Xin, Bo Liu, Wei Li, Dezhen Yin, Weidong Mu
Giant intrapelvic malignant peripheral nerve sheath tumors arising in the sciatic nerve in the pelvic cavity are a rare occurrence and their symptomatology is usually misdiagnosed as intervertebral disc herniation. We herein report the case of a 46-year old woman presenting with pain, hypesthesia and weakness of the left lower extremity due to a giant intrapelvic malignant peripheral nerve sheath tumor of the sciatic nerve. Prior to being referred to our institution, the patient was misdiagnosed as a case of sciatica due to a lumbar disc herniation and underwent an operation unsuccessfully, as there was little symptomatic improvement 2 months after the surgery...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27900013/surgical-treatment-of-rare-giant-malignant-tumors-of-the-scalp-a-report-of-3-cases-with-different-tumor-types
#3
Xiaoliang Liu, Wenzhong Li, Hepei Yuan, Weihong Gu, Dawei Chen
The scalp is the most frequent site of occurrence of malignant tumors. As an area that is generally neglected by the patient and not closely monitored during physical examinations, scalp tumors can go unnoticed until they become malignant. The present study reports 3 cases of rare giant malignant tumors of the scalp, namely a peripheral nerve sheath tumor, a fibrous tumor and a malignant proliferating trichilemmal tumor, that were treated at The First Bethune Hospital of Jilin University (Changchun, China)...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27890347/isolated-limb-perfusion-for-the-management-limb-threatening-soft-tissue-sarcomas-the-role-of-histological-type-on-clinical-outcomes
#4
M Rastrelli, S Mocellin, R Stramare, A Brunello, M Maruzzo, U Basso, G Scarzello, M S Buzzaccarini, P Pilati, L M Saadeh, S P Del Fiore, A Tosi, C Montesco, L G Campana, S Tropea, C R Rossi
BACKGROUND: Hyperthermic isolated limb perfusion (HILP) is an effective neoadjuvant treatment to avoid amputation in patients with locally advanced extremity soft tissue sarcomas (STS). We aimed to investigate whether STS histological type plays a role in predicting clinical outcomes. METHODS: This study reports a retrospective analysis of 125 patients with limb threatening STS (liposarcoma, n = 41; malignant peripheral nerve sheath tumor, n = 20; leiomyosarcoma, n = 20; miscellany, n = 44), who underwent HILP from 1990 through 2015 at our institution...
November 18, 2016: European Journal of Surgical Oncology
https://www.readbyqxmd.com/read/27879515/atypical-spindle-cell-lipomatous-tumor-clinicopathologic-characterization-of-232-cases-demonstrating-a-morphologic-spectrum
#5
Adrian Mariño-Enriquez, Alessandra F Nascimento, Azra H Ligon, Cherwei Liang, Christopher D M Fletcher
The classification of atypical adipocytic neoplasms with spindle cell features remains challenging. To better define this category of low-grade lipomatous neoplasms, we present herein the clinical, histologic, and immunohistochemical characteristics of a large series of 232 atypical spindle cell lipomatous tumors. The lesions affected 140 males and 92 females, at an average age of 54 years (range, 6 to 87 y), clinically presenting as a persistent or enlarging mass with a median size of 5 cm. The anatomic distribution of the tumors was wide, predominating in the limbs and limb girdles (147 cases, 63%), mainly in the hands and feet (17% and 11%, respectively), with equal distribution between subcutaneous and deeper locations...
November 22, 2016: American Journal of Surgical Pathology
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
#6
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...
November 22, 2016: 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
#7
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
#8
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
#9
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/27871009/malignant-nerve-sheath-tumor-involving-glossopharyngeal-vagus-and-spinal-nerve-with-intracranial-extracranial-extension-and-systemic-metastases-in-a-patient-with-type-1-neurofibromatosis-a-case-report
#10
José Raúl Guerra-Mora, Juan D Del Castillo-Calcáneo, María Elena Córdoba-Mosqueda, Jorge Yáñez-Castro, Ulises García-González, Eduardo Soriano-Navarro, Leticia Llamas-Ceras, Rosa María Vicuña-González
INTRODUCTION: Intracranial malignant peripheral nerve sheath tumors are an extremely rare pathology with a high morbidity and mortality. Epidemiological, clinical and prognostic data are scarce and with little certainty in the literature. The aim of this paper is to report for first time in English literature, the case of a patient with type 1 neurofibromatosis, who presented a malignant peripheral nerve sheath tumor that involved the left glossopharyngeal, vagus and spinal nerves with intracranial and extracranial extension through jugular foramen and systemic metastases...
November 5, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27867771/prospect-and-progress-of-oncolytic-viruses-for-treating-peripheral-nerve-sheath-tumors
#11
Slawomir Antoszczyk, Samuel D Rabkin
INTRODUCTION: Peripheral nerve sheath tumors (PNSTs) are an assorted group of neoplasms originating from neuroectoderm and growing in peripheral nerves. Malignant transformation leads to a poor prognosis and is often lethal. Current treatment of PNSTs is predominantly surgical, which is often incomplete or accompanied by significant loss of function, in conjunction with radiotherapy and/or chemotherapy, for which the benefits are inconclusive. Oncolytic viruses (OVs) efficiently kill tumor cells while remaining safe for normal tissues, and are a novel antitumor therapy for patients with PNSTs...
2016: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/27834629/malignant-peripheral-nerve-sheath-tumors-of-the-spine-results-of-surgical-management-from-a-multicenter-study
#12
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
#13
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/27818132/plexiform-schwannoma-an-unusual-clitoral-mass
#14
Anne G Sammarco, Noor M Abualnadi, Elizabeth A Andraska, Paige V Tracy, Mitchell B Berger, Hope K Haefner
Acquired clitoral enlargement is a rare condition resulting from a variety of etiologies, including tumors and excess androgens. Few cases of nonmalignant schwannoma, a benign tumor of the peripheral nerve sheath, have been reported in the literature as causes of clitoral enlargement in patients without known neurofibromatosis. These painless, slow-growing tumors rarely recur once excised. We present the initial investigation of a patient with a large clitoral schwannoma and subsequent treatment with partial vulvectomy...
November 3, 2016: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27816720/multivacuolated-mucin-filled-cells-a-unique-cell-characteristic-of-plexiform-neurofibroma-a-report-of-11-cases
#15
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/27815467/molecular-heterogeneity-of-canine-cutaneous-peripheral-nerve-sheath-tumors-a-drawback-in-the-diagnosis-refinement
#16
Sílvia Teixeira, Irina Amorim, Alexandra Rêma, Fátima Faria, Fátima Gärtner
BACKGROUND/AIM: Peripheral nerve sheath tumors (PNSTs) belong to a very heterogeneous group of neoplasms occurring both in dogs and humans. The aim of the present study was to evaluate the histological and immunohistochemical features of canine cutaneous PNSTs contributing to further refine their diagnosis. MATERIALS AND METHODS: The histopathological phenotype and biological behavior of 40 canine cutaneous PNSTs were evaluated and vimentin, S-100, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), desmin, alpha-smooth muscle actin (α-SMA) and Ki-67 immunoreactivity were assessed...
November 2016: In Vivo
https://www.readbyqxmd.com/read/27805920/benign-glandular-schwannoma-with-ancient-change
#17
Alex C Holliday, Sean E Mazloom, Garrett C Coman, Michael S Kolodney, Rahul N Chavan, Douglas J Grider
Benign glandular schwannomas are rare and should be distinguished from malignant peripheral nerve sheath tumors with similar divergent tissue differentiation. The authors present a benign glandular schwannoma with ancient change that developed in the subcutis of a 46-year-old man's posterior calf. He lacked stigmata of neurofibromatosis type 1 (NF1). The glandular elements stained positively for epithelial membrane antigen and pancytokeratin. The spindled cells stained positively for SOX10 and S100 protein, supporting schwannian (neural crest) differentiation...
October 31, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27761835/evaluating-the-effect-of-hdac8-inhibition-in-malignant-peripheral-nerve-sheath-tumors
#18
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
https://www.readbyqxmd.com/read/27739316/-multimodal-therapy-of-recurrent-malignant-schwannoma
#19
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
https://www.readbyqxmd.com/read/27734293/comparision-of-new-diagnostic-tools-for-malignant-peripheral-nerve-sheath-tumors
#20
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
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