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Malignant nerve sheath tumor

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https://www.readbyqxmd.com/read/28075051/bilateral-multifocal-upper-extremity-atypical-granular-cell-tumors-presenting-as-long-standing-right-wrist-and-left-hand-masses-in-a-15-year-old-african-american-female
#1
Michael DeWall, Corey O Montgomery, Jerad M Gardner
Granular cell tumor (GrCT) is a benign nerve sheath tumor. Atypical and malignant variants of GrCT are rare but have been well described. We report a case of multifocal symmetric atypical GrCT in the bilateral hand/wrists of a 15 year old African American female. The initial clinical impression for both masses were favored to be ganglion cysts. Ultrasound findings of both masses revealed hypoechoic soft tissue lesions with some internal echogenicity favoring complex cysts. On excision, both masses were histologically circumscribed, lobulated, and attached to tendon...
January 11, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28068329/the-primacy-of-nf1-loss-as-the-driver-of-tumorigenesis-in-neurofibromatosis-type-1-associated-plexiform-neurofibromas
#2
A Pemov, H Li, R Patidar, N F Hansen, S Sindiri, S W Hartley, J S Wei, A Elkahloun, S C Chandrasekharappa, J F Boland, S Bass, J C Mullikin, J Khan, B C Widemann, M R Wallace, D R Stewart
Neurofibromatosis type 1 (NF1) is a common tumor-predisposition disorder due to germline mutations in the tumor suppressor gene NF1. A virtually pathognomonic finding of NF1 is the plexiform neurofibroma (PN), a benign, likely congenital tumor that arises from bi-allelic inactivation of NF1. PN can undergo transformation to a malignant peripheral nerve sheath tumor, an aggressive soft-tissue sarcoma. To better understand the non-NF1 genetic contributions to PN pathogenesis, we performed whole-exome sequencing, RNASeq profiling and genome-wide copy-number determination for 23 low-passage Schwann cell cultures established from surgical PN material with matching germline DNA...
January 9, 2017: Oncogene
https://www.readbyqxmd.com/read/28067315/kank1-inhibits-cell-growth-by-inducing-apoptosis-though-regulating-cxxc5-in-human-malignant-peripheral-nerve-sheath-tumors
#3
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
#4
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
#5
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...
December 31, 2016: Oncotarget
https://www.readbyqxmd.com/read/28052660/outcomes-of-treatment-for-malignant-peripheral-nerve-sheath-tumors-different-clinical-features-associated-with-neurofibromatosis-type-1
#6
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/28018768/rare-intradural-cervical-nerve-root-metastasis-of-follicular-thyroid-carcinoma
#7
Joseph Keen, Elena Milosavljevic, George Hanna, Vadim Gospodarev, Ravi Raghavan, Samer Ghostine
Intradural extramedullary nerve root metastasis is extremely unusual with only a handful of cases reported, and it presents most commonly in the thoracic and lumbosacral regions. We report the first case of metastasis to a ventral cervical nerve root in a patient with low-grade follicular thyroid carcinoma thought to be in remission for several years. Histopathology demonstrated malignant transformation and invasion of the nerve root. This case underscores that any history of malignancy regardless of staging, grading, or remission status should raise the suspicion of metastasis as it can mimic other spine and nerve sheath tumors and represent malignant transformation...
November 24, 2016: Curēus
https://www.readbyqxmd.com/read/28002805/expression-of-psma-in-tumor-neovasculature-of-high-grade-sarcomas-including-synovial-sarcoma-rhabdomyosarcoma-undifferentiated-sarcoma-and-mpnst
#8
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/27994888/a-rare-malignant-peripheral-nerve-sheath-tumor-of-the-maxilla-mimicking-a-periapical-lesion
#9
José Alcides Arruda, Pamella Álvares, Luciano Silva, Alexandrino Pereira Dos Santos Neto, Cleomar Donizeth Rodrigues, Antônio Caubi, Marcia Silveira, Sandra Sayão, Ana Paula Sobral
Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature...
2016: Case Reports in Dentistry
https://www.readbyqxmd.com/read/27992966/the-role-of-phosphorylated-signal-transducer-and-activator-of-transcription-3-pstat3-in-peripheral-nerve-sheath-tumors
#10
Gauri Panse, Cheuk Hong Leung, Davis Ingram, Khalida Wani, Keila E Torres, Heather Lin, Alexander Lazar, Wei-Lien Wang
AIMS: STAT3 is a pro-oncogenic transcription factor that requires phosphorylation for transcriptional activation. We evaluated the role of pSTAT3 expression in neurofibromas, schwannomas and malignant peripheral nerve sheath tumors (MPNSTs). METHODS AND RESULTS: 26 neurofibromas, 62 schwannomas and 39 MPNSTs from a formalin-fixed paraffin-embedded tissue microarray were examined. Immunohistochemical analysis was performed using an anti-p-STAT3 (Tyr705) antibody...
December 19, 2016: Histopathology
https://www.readbyqxmd.com/read/27938614/-malignant-peripheral-nerve-sheath-tumor-of-the-nasal-cavity-and-nasopharynx-in-child-a-case-report
#11
H G Pan, J Cao, Z B Wu, L Li
No abstract text is available yet for this article.
November 7, 2016: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/27920939/epigenetic-mechanisms-drive-the-progression-of-neurofibromas-to-malignant-peripheral-nerve-sheath-tumors
#12
REVIEW
Krish Suresh, Tamara Kliot, Andrea Piunti, Michel Kliot
THINKING OUTSIDE THE BOX: The polycomb repressive complex 2 (PRC2) is a histone methyltransferase complex known to repress gene expression. There is a large body of experimental evidence that supports its role in promoting tumorigenicity by suppressing tumor suppressor genes. Here, we discuss the surprising findings that, in neurofibromas, it may have a completely different role as a tumor suppressor; mutations of PRC2 lead to conversion of benign neurofibromas into malignant peripheral nerve sheath tumors (MPNSTs) by de-repressing and thereby activating genes driving cell growth and development...
2016: Surgical Neurology International
https://www.readbyqxmd.com/read/27912995/rehabilitation-strategies-and-outcomes-of-the-sarcoma-patient
#13
REVIEW
Sean Robinson Smith
Sarcomas are a relatively rare cancer that, depending on the location, can cause significant neuromusculoskeletal dysfunction and require rehabilitation interventions to reduce pain, restore function, and improve quality of life. This review focuses on sarcoma subtypes that frequently cause these complications: bony and soft tissue sarcomas leading to limb salvage or amputation, desmoid tumors, and malignant peripheral nerve sheath tumors. Rehabilitation approaches and outcomes are discussed, as well as considerations for childhood sarcoma survivors transitioning to adulthood...
February 2017: Physical Medicine and Rehabilitation Clinics of North America
https://www.readbyqxmd.com/read/27904856/primary-hepatic-malignant-peripheral-nerve-sheath-tumor-successfully-treated-with-combination-therapy-a-case-report-and-literature-review
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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