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Peripheral nerve sheath tumor

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https://www.readbyqxmd.com/read/28549031/chd4-as-a-potential-biomarker-in-differentiating-between-cellular-schwannoma-and-malignant-peripheral-nerve-sheath-tumor
#1
Chun-Chieh Wu, Mei-Ren Pan, Yu-Ching Wei, Chih-Hung Lin, Sheau-Fang Yang, Hung-Pei Tsai, Chi-Wen Luo, Chee-Yin Chai
Cellular schwannoma is an uncommon variant of benign peripheral nerve sheath tumors, but is commonly misdiagnosed as malignant peripheral sheath tumor (MPNST). Conventional methods that are used to distinguish cellular schwannoma from MPNST include immunohistochemistry (IHC) staining. However, most markers cannot precisely differentiate these 2 tumor types, and thus identification of a better marker is needed to improve the accuracy of diagnosis. Here, we evaluate the use of chromodomain helicase DNA-binding protein 4 (CHD4) as a specific marker for cellular schwannoma by comparing CHD4 and S-100 IHC staining in 14 cellular schwannoma and 17 MPNST tissue samples...
May 25, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28548933/kir2dl5-mutation-and-loss-underlies-sporadic-dermal-neurofibroma-pathogenesis-and-growth
#2
Corina Anastasaki, Sonika Dahiya, David H Gutmann
Dermal neurofibromas (DNFs) are benign peripheral nerve sheath tumors thought to originate from Schwann cell progenitors. These tumors represent one of the hallmarks of the neurofibromatosis type 1 (NF1) tumor predisposition syndrome, where they can number in the thousands. While NF1-DNFs arise due to mutations in the NF1 gene, the vast majority of DNFs occur sporadically (sp-DNFs), where the genetic etiology is currently unknown. Herein, we employed whole-exome sequencing of sp-DNFs to identify a recurrent mutation in the KIR2DL5 gene, which codes for a protein suppressor of natural killer (NK) cell activity...
May 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/28548697/metastatic-angiosarcoma-arising-in-malignant-peripheral-nerve-sheath-tumor-in-a-young-patient-with-neurofibromatosis-type-1
#3
Amanda C Winters, Jennifer O Black, Carrye R Cost
Neurofibromatosis type 1 (NF1) is a cancer predisposition syndrome with an incidence of approximately one in 3,000 and a lifetime risk of malignancy estimated at 8-13%. Here, we report the case of a patient with NF1 who developed synchronous malignant peripheral nerve sheath tumors, one with a focus of angiosarcoma. He succumbed to metastatic angiosarcoma despite local resection and adjuvant chemotherapy. This case highlights the need for monitoring for malignancy in NF1 patients, the risks of sampling error during tumor biopsy, and the clinical decision - making involved in choosing a therapeutic plan for a patient with multiple simultaneous malignancies...
May 26, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28546782/neoadjuvant-ifosfamide-and-epirubicin-in-the-treatment-of-malignant-peripheral-nerve-sheath-tumors
#4
Angela C Hirbe, Pippa F Cosper, Sonika Dahiya, Brian A Van Tine
Background and Objectives. Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with poor overall survival. Response to chemotherapy has been debated for these tumors. Methods. We performed a retrospective analysis of the patients at our institution with a biopsy-proven diagnosis of MPNST that underwent neoadjuvant chemotherapy prior to surgery. Results. We retrospectively identified five patients who received neoadjuvant chemotherapy with epirubicin and ifosfamide that demonstrated a 30% reduction in tumor growth and a 60% response rate by RECIST criteria...
2017: Sarcoma
https://www.readbyqxmd.com/read/28544597/metastasis-of-soft-tissue-sarcomas-in-lymph-node-a-cytomorphological-study
#5
Jahnavi Gandhi, Shailee Mehta, Trupti Patel, Amisha Gami, Majal Shah, Dhaval Jetly
BACKGROUND: Soft-tissue sarcoma (STS) rarely metastasizes to lymph node compared to carcinoma. Fine-needle aspiration cytology (FNAC) carries a pivotal role in diagnosis of metastatic tumor to lymph node. This study highlights the role of FNAC in diagnosis of STS metastasis to lymph node. METHOD: A retrospective study over a period by 4 years carried out. FNAC of enlarged lymph node was performed in patients with STS. Cytology smears were examined in conjunction with clinical details...
May 24, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28542306/gene-signature-associated-with-benign-neurofibroma-transformation-to-malignant-peripheral-nerve-sheath-tumors
#6
Marta Martínez, Carlos O S Sorzano, Alberto Pascual-Montano, Jose M Carazo
Benign neurofibromas, the main phenotypic manifestations of the rare neurological disorder neurofibromatosis type 1, degenerate to malignant tumors associated to poor prognosis in about 10% of patients. Despite efforts in the field of (epi)genomics, the lack of prognostic biomarkers with which to predict disease evolution frustrates the adoption of appropriate early therapeutic measures. To identify potential biomarkers of malignant neurofibroma transformation, we integrated four human experimental studies and one for mouse, using a gene score-based meta-analysis method, from which we obtained a score-ranked signature of 579 genes...
2017: PloS One
https://www.readbyqxmd.com/read/28534510/targeting-group-i-p21-activated-kinases-to-control-malignant-peripheral-nerve-sheath-tumor-growth-and-metastasis
#7
G Semenova, D S Stepanova, C Dubyk, E Handorf, S M Deyev, A J Lazar, J Chernoff
Malignant peripheral nerve sheath tumors (MPNSTs) are devastating sarcomas for which no effective medical therapies are available. Over 50% of MPSNTs are associated with mutations in NF1 tumor suppressor gene, resulting in activation of Ras and its effectors, including the Raf/Mek/Erk and PI3K/Akt/mTORC1 signaling cascades, and also the WNT/β-catenin pathway. As Group I p21-activated kinases (Group I Paks, PAK1/2/3) have been shown to modulate Ras-driven oncogenesis, we asked if these enzymes might regulate signaling in MPNSTs...
May 22, 2017: Oncogene
https://www.readbyqxmd.com/read/28532923/radiation-induced-schwannomas-and-neurofibromas-a-systematic-review
#8
REVIEW
Ryuya Yamanaka, Azusa Hayano
OBJECTIVE: Radiation-induced benign peripheral nerve sheath tumors are uncommon late complications of irradiation. We conducted the largest systematic review of individual patient data. METHODS: We performed a systematic search of PubMed databases, and compiled a comprehensive literature review. Kaplan-Meier analysis was used to investigate survival, and statistical significance was assessed using a log-rank test. RESULTS: We analyzed 40 cases of radiation-induced benign peripheral nerve sheath tumors...
May 19, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28530162/radiation-induced-glandular-malignant-peripheral-nerve-sheath-tumor
#9
Ivy John, David L Bartlett, Uma N M Rao
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft-tissue tumors. They can occur in patients with neurofibromatosis type-1 (NF-1) or as sporadic tumors. Only 10% of MPNSTs are radiation induced. Divergent differentiation in MPNSTs can occur in 15% of cases and may include cartilage, bone, skeletal muscle, blood vessels, and very rarely well-formed glands, the latter typically described in NF-1-associated MPNSTs. We report an exceedingly rare case of radiation induced glandular MPNST arising in a neurofibroma of the femoral nerve in a patient previously irradiated for endometrial carcinoma...
May 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28526004/hybrid-peripheral-nerve-sheath-tumors-report-of-five-cases-and-detailed-review-of-literature
#10
Nasir Ud Din, Zubair Ahmad, Jamshid Abdul-Ghafar, Rashida Ahmed
BACKGROUND: Hybrid peripheral nerve sheath tumors (PNSTs) have been recognized recently and were first included in the 4th edition of World Health Organization (WHO) Classification of Tumors of Soft tissue and Bone, published in 2013. These tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. The most common combinations are those of schwannoma/perineurioma followed by combinations of neurofibroma/schwannoma and neurofibroma/perineurioma...
May 19, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28515751/malignant-triton-tumor-of-the-lung-infiltrating-the-left-atrium-and-left-ventricle-with-metastasis-to-the-small-intestine
#11
Dariusz Tomaszewski, Sebastian Beta, Jan A Rogowski
Malignant triton tumors are a rare subtype of malignant peripheral nerve sheath tumors showing rhabdomyosarcomatous differentiation. We report the case of a 33-year-old patient who underwent an excision of such a tumor by thoracic and cardiac surgery. He was reoperated on 1 month later because of a tumor of the small intestine and received adjuvant radiation.
March 2017: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/28508686/malignant-peripheral-nerve-sheath-tumor-of-the-femur-a-rare-diagnosis-supported-by-complete-immunohistochemical-loss-of-h3k27me3
#12
Masato Sugawara, Eisuke Kobayashi, Naofumi Asano, Akihiko Yoshida, Akira Kawai
The histological diagnosis of malignant peripheral nerve sheath tumor (MPNST) is challenging because of the wide morphological spectrum and suboptimal performance of conventional immunohistochemical markers. MPNST arising primarily in the bone is exceptional, and its definitive diagnosis, particularly out of the neurofibromatosis type 1 (NF1) context, is even more problematic. Recurrent inactivation of EED or SUZ12 in a majority of MPNSTs results in a complete loss of trimethylated histone H3 at lysine 27 (H3K27me3) immunoreactivity, making it a highly specific biomarker of MPNSTs...
May 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28505957/spindle-cell-type-malignant-peripheral-nerve-sheath-tumor-arising-in-benign-schwannoma-with-multiple-intraosseous-spinal-metastasis-a-case-report
#13
Jae Yoon Chung, Sung Sun Kim, Sung Kyu Kim
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) arising in benign schwannoma with multiple intraosseous spinal metastasis is extremely rare, having a highly aggressive progression and poor prognosis. In such cases, the malignant cells of MPNST usually have an epithelioid morphology. Here, the authors present a very rare case of spindle cell type MPNST arising in benign schwannoma. CASE: A 47-year-old woman had a history of wide marginal excision of right buttock spindle cell sarcoma previously...
May 5, 2017: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/28502561/acute-hydrocephalus-due-to-a-primary-malignant-peripheral-nerve-sheath-tumor-of-the-cervicothoracic-junction-a-case-report-and-review-of-the-literature
#14
Y Samanci, H S Togay, R Yakar, F Kabukcuoglu, S E Celik
BACKGROUND: The estimated incidence of malignant nerve sheath tumors is 0.001% per year, and only 2-3% of those tumors involve the spinal nerves. We present a rare case of acute hydrocephalus caused by primary malignant peripheral nerve sheath tumor of the cervicothoracic junction. CASE DESCRIPTION: A 29-year-old previously healthy male patient, except for a history of two previous surgeries for ulnar nerve entrapment and progressive left upper extremity weakness, presented with acute onset somnolence...
May 11, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28502478/the-cellular-retinoic-acid-binding-protein-2-promotes-survival-of-malignant-peripheral-nerve-sheath-tumor-cells
#15
Susan Fischer-Huchzermeyer, Anna Dombrowski, Christian Hagel, Victor Felix Mautner, Jens Schittenhelm, Anja Harder
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive neoplasms that commonly occur in patients with neurofibromatosis type 1 (NF1). Effective chemotherapy is not available. To characterize a therapeutic target for treatment, we investigated the role of cellular retinoic acid binding protein 2 (CRABP2) in MPNST in vitro. CRABP2 is a transcriptional co-activator of retinoic acid signaling. Although overexpression of CRABP2 is described in several cancers, it has not yet been studied in MPNSTs. We investigated CRABP2 expression in cultured Schwann cells and formalin-fixed, paraffin-embedded specimens of human peripheral nerve sheath tumors...
May 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28498283/synovial-sarcoma-of-peripheral-nerves-analysis-of-15-cases
#16
John S A Chrisinger, Usama I Salem, Lars-Gunnar Kindblom, Behrang Amini, Magnus Hansson, Jeanne M Meis
Synovial sarcoma of peripheral nerve (SSPN) is rare with only 26 cases reported in English. SSPN is often mistaken for a benign or malignant peripheral nerve sheath tumor (PNST) by clinicians and pathologists. Fifteen cases of SSPN were retrieved from the pathology files of 3 institutions. All tumors arose in a nerve based on imaging and/or operative findings and the diagnoses were histologically confirmed. Neuropathic symptoms predominated in 11 women and 4 men, 19- to 62-year-old (median, 39 years) with tumors involving the ulnar (5), median (3), peroneal (3) or sciatic (2) nerve, or the L4 or T1 nerve root (2)...
May 11, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28493803/soft-tissue-sarcomas-of-the-abdomen-and-pelvis-radiologic-pathologic-features-part-2-uncommon-sarcomas
#17
Angela D Levy, Maria A Manning, Markku M Miettinen
Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies. Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification. GIST is the most common intraperitoneal sarcoma. Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common. GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article...
May 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28481797/incidental-prostate-specific-membrane-antigen-uptake-in-a-peripheral-nerve-sheath-tumor
#18
Shankar Vamadevan, Ken Le, Lily Shen, Leo Ha, Robert Mansberg
A 66-year-old man with recently diagnosed prostate cancer (Gleason score 9) was referred for Ga-prostate-specific membrane antigen (PSMA) PET/CT with prostate-specific antigen level of 7.5 μg/L. PET/CT demonstrated increased PSMA uptake (SUVmax 4.7) in a soft tissue density in the left adductor compartment. MRI and cytopathology of the biopsied soft tissue density was compatible with a peripheral nerve sheath tumor. This case illustrates that PSMA uptake can occur in a peripheral nerve sheath tumor and should be taken into consideration as a benign cause of PSMA uptake...
May 6, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28475798/the-surgical-management-of-symptomatic-benign-peripheral-nerve-sheath-tumors-of-the-neck-and-extremities-an-experience-of-442-cases
#19
Ketan I Desai
BACKGROUND: The occurrence of benign peripheral nerve sheath tumors (PNSTs) is not uncommon. The surgical excision of symptomatic benign PNSTs along with preservation of the affected nerve and its function is an ideal treatment option. OBJECTIVE: To analyze the outcome with respect to morbidity, extent of resection, and recurrence, and to review and compare our results with those reported in literature. METHODS: A retrospective review of clinical and radiological findings of 442 patients with benign PNSTs involving the neck and extremities treated surgically from 2000 to 2014 was performed...
May 5, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28469964/comprehensive-pharmacological-profiling-of-neurofibromatosis-cell-lines
#20
Jianman Guo, Michael R Grovola, Hong Xie, Grace E Coggins, Patrick Duggan, Rukhsana Hasan, Jiale Huang, Danny W Lin, Claire Song, Gabriela M Witek, Simon Berritt, David C Schultz, Jeffrey Field
Patients with Neurofibromatosis type 1 (NF1) and Neurofibromatosis type 2 (NF2) are predisposed to tumors of the nervous system. NF1 patients predominantly develop neurofibromas, and Malignant Peripheral Nerve Sheath Tumors (MPNST) while NF2 patients develop schwannomas and meningiomas. Here we quantified the drug sensitivities of NF1 and NF2 tumor cell lines in a high throughput platform. The platform contained a comprehensive collection of inhibitors of MEK, RAF, RAS, farnesyl transferase, PAK and ERK, representative drugs against many other cancer pathways including Wnt, Hedgehog, p53, EGF, HDAC, as well as classical cytotoxic agents recommended for treating MPNST, such as doxorubicin and etoposide...
2017: American Journal of Cancer Research
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