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https://www.readbyqxmd.com/read/28635234/-clinicopathological-features-and-prognosis-of-malignant-peripheral-nerve-sheath-tumor-a-retrospective-study-of-140-cases
#1
Z N Yuan, L B Xu, Z G Zhao, S F Xu, X X Zhang, T Liu, S G Zhang, S J Yu
Objective: To investigate the clinicopathological features and prognosis of malignant peripheral nerve sheath tumors (MPNST). Methods: We retrospectively reviewed the clinical data of MPNST patients who were treated at Cancer Institute & Hospital, Chinese Academy of Medical Science from January 1999 to January 2016. A total of 140 patients with 66 male and 74 female with MPNST were enrolled in the study. The median age was 40 at the time of diagnosis. Survival analysis were estimated by Kaplan-Meier method and Log rank test...
June 23, 2017: Zhonghua Zhong Liu za Zhi [Chinese Journal of Oncology]
https://www.readbyqxmd.com/read/28611304/immune-profiling-of-nf1-associated-tumors-reveals-histologic-subtype-distinctions-and-heterogeneity-implications-for-immunotherapy
#2
Kellie B Haworth, Michael A Arnold, Christopher R Pierson, Kwangmin Choi, Nicholas D Yeager, Nancy Ratner, Ryan D Roberts, Jonathan L Finlay, Timothy P Cripe
Successful treatment of neurofibromatosis type 1 (NF1)-associated tumors poses a significant clinical challenge. While the primary underlying genetic defect driving RAS signaling is well described, recent evidence suggests immune dysfunction contributes to tumor pathogenesis and malignant transformation. As immunologic characterizations, prognostic and predictive of immunotherapeutic clinical response in other cancers, are not fully described for benign and malignant NF1-related tumors, we sought to define their immunologic profiles...
May 30, 2017: Oncotarget
https://www.readbyqxmd.com/read/28602926/radiation-induced-malignant-peripheral-nerve-sheath-tumors-a-systematic-review
#3
REVIEW
Ryuya Yamanaka, Azusa Hayano
OBJECTIVE: Radiation-induced malignant peripheral nerve sheath tumors (MPNSTs) are an uncommon late risk of irradiation. We conducted the largest systematic review to date, of individual patient data for patients with these tumors. METHODS: We conducted a systematic search using the PubMed database, and compiled a systematic literature review. We used Kaplan-Meier analysis and log-rank test to estimate survival. RESULTS: We analyzed 65 radiation-induced and 26 radiation-associated MPNSTs in patients with neurofibromatosis...
June 8, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28592921/malignant-peripheral-nerve-sheath-tumors-state-of-the-science-leveraging-clinical-and-biological-insights-into-effective-therapies
#4
REVIEW
AeRang Kim, Douglas R Stewart, Karlyne M Reilly, David Viskochil, Markku M Miettinen, Brigitte C Widemann
Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and biology of MPNST through imaging and genomic advances since 2002. Genetically engineered mouse models that develop MPNST spontaneously have greatly facilitated preclinical evaluation of novel drugs for translation into clinical trials led by consortia efforts...
2017: Sarcoma
https://www.readbyqxmd.com/read/28558056/hypoxia-inducible-factor-1-alpha-is-a-poor-prognostic-factor-and-potential-therapeutic-target-in-malignant-peripheral-nerve-sheath-tumor
#5
Suguru Fukushima, Makoto Endo, Yoshihiro Matsumoto, Jun-Ichi Fukushi, Tomoya Matsunobu, Ken-Ichi Kawaguchi, Nokitaka Setsu, Keiichiro IIda, Nobuhiko Yokoyama, Makoto Nakagawa, Kenichiro Yahiro, Yoshinao Oda, Yukihide Iwamoto, Yasuharu Nakashima
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma with poor prognosis. Hypoxia-inducible factor 1 (HIF-1) plays a crucial role in the cellular response to hypoxia and regulates the expression of multiple genes involved in tumor progression in various cancers. However, the importance of the expression of HIF-1α in MPNSTs is unclear. METHODS: The expression of HIF-1α was examined immunohistochemically in 82 MPNST specimens...
2017: PloS One
https://www.readbyqxmd.com/read/28558025/doxycycline-potentiates-antitumor-effect-of-5-aminolevulinic-acid-mediated-photodynamic-therapy-in-malignant-peripheral-nerve-sheath-tumor-cells
#6
Ming-Jen Lee, Shih-Hsuan Hung, Mu-Ching Huang, Tsuimin Tsai, Chin-Tin Chen
Neurofibromatosis type 1 (NF1) is one of the most common neurocutaneous disorders. Some NF1 patients develop benign large plexiform neurofibroma(s) at birth, which can then transform into a malignant peripheral nerve sheath tumor (MPNST). There is no curative treatment for this rapidly progressive and easily metastatic neurofibrosarcoma. Photodynamic therapy (PDT) has been developed as an anti-cancer treatment, and 5-aminolevulinic (ALA) mediated PDT (ALA-PDT) has been used to treat cutaneous skin and oral neoplasms...
2017: PloS One
https://www.readbyqxmd.com/read/28556483/drug-sensitivity-and-resistance-testing-identifies-plk1-inhibitors-and-gemcitabine-as-potent-drugs-for-malignant-peripheral-nerve-sheath-tumors-mpnst
#7
Matthias Kolberg, Jarle Bruun, Astrid Murumägi, John P Mpindi, Christian H Bergsland, Maren Høland, Ina A Eilertsen, Stine A Danielsen, Olli Kallioniemi, Ragnhild A Lothe
Patients with malignant peripheral nerve sheath tumor (MPNST), a rare soft tissue cancer associated with loss of the tumor suppressor neurofibromin (NF1), have poor prognosis and typically respond poorly to adjuvant therapy. We evaluated the effect of 299 clinical and investigational compounds on seven MPNST cell lines, two primary cultures of human Schwann cells, and five normal bone marrow aspirates, to identify potent drugs for MPNST treatment with few side effects. Top hits included Polo-like kinase 1 (PLK1) inhibitors (volasertib and BI2536) and the fluoronucleoside gemcitabine, which were validated in orthogonal assays measuring viability, cytotoxicity and apoptosis...
May 29, 2017: Molecular Oncology
https://www.readbyqxmd.com/read/28551330/histopathologic-evaluation-of-atypical-neurofibromatous-tumors-and-their-transformation-into-malignant-peripheral-nerve-sheath-tumor-in-neurofibromatosis-1-patients-a-consensus-overview
#8
Markku M Miettinen, Cristina R Antonescu, Christopher D M Fletcher, Aerang Kim, Alexander J Lazar, Martha M Quezado, Karlyne M Reilly, Anat Stemmer-Rachamimov, Douglas R Stewart, David Viskochil, Brigitte Widemann, Arie Perry
Neurofibromatosis 1 (NF1) patients develop multiple neurofibromas, with 8-15% of patients experiencing malignant peripheral nerve sheath tumor (MPNST) during their lifetime. Prediction of transformation, typically from plexiform neurofibroma, is clinically and histologically challenging. In this overview, following a consensus meeting in October 2016, we outline the histopathologic features and molecular mechanisms involved in the malignant trans-formation of neurofibromas. Nuclear atypia alone is generally insignificant...
May 24, 2017: Human Pathology
https://www.readbyqxmd.com/read/28549031/chd4-as-a-potential-biomarker-in-differentiating-between-cellular-schwannoma-and-malignant-peripheral-nerve-sheath-tumor
#9
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/28546782/neoadjuvant-ifosfamide-and-epirubicin-in-the-treatment-of-malignant-peripheral-nerve-sheath-tumors
#10
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/28546722/malignant-peripheral-nerve-sheath-tumour-of-the-small-bowel-presenting-with-intussusception-and-perforation-a-double-jeopardy
#11
Ananth P Abraham, Joshua Franklyn, Jagan Chandramohan, Pranay Gaikwad, John C Muthusami
Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcomas which largely occur in the extremities and the head and neck region. The tumours are aggressive with a high rate of recurrence. Radical surgical resection remains the treatment of choice with adjuvant radiation therapy and chemotherapy still failing to demonstrate a clear benefit. The gastrointestinal tract is an exceedingly rare site for these tumours. We report an unusual case of a young male with an MPNST of the small bowel who presented with an ileocolic intussusception and sigmoid perforation...
June 2017: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28534510/targeting-group-i-p21-activated-kinases-to-control-malignant-peripheral-nerve-sheath-tumor-growth-and-metastasis
#12
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/28530162/radiation-induced-glandular-malignant-peripheral-nerve-sheath-tumor
#13
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/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#14
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28508686/malignant-peripheral-nerve-sheath-tumor-of-the-femur-a-rare-diagnosis-supported-by-complete-immunohistochemical-loss-of-h3k27me3
#15
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
#16
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/28502478/the-cellular-retinoic-acid-binding-protein-2-promotes-survival-of-malignant-peripheral-nerve-sheath-tumor-cells
#17
Susan Fischer-Huchzermeyer, Anna Dombrowski, Christian Hagel, Victor F 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...
July 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28469964/comprehensive-pharmacological-profiling-of-neurofibromatosis-cell-lines
#18
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
https://www.readbyqxmd.com/read/28462660/malignant-peripheral-nerve-sheath-tumour-in-the-submandibular-space
#19
J B Olivier
Malignant peripheral nerve sheath tumours (MPNSTs) are a rare but aggressive form of soft tissue sarcoma with few reported cases in the anatomic location seen with the case presented here. This case involves a 23-year-old man presenting with a soft tissue mass under the mandible found to be a MPNST, which was investigated and fully excised. Adjuvant radiotherapy was used postoperatively. Although uncommon, these tumours have a poor prognosis and suitable management therefore needs to be put in place as soon as possible...
May 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28452074/clinicopathologic-features-and-immunohistochemical-spectrum-of-11-cases-of-epithelioid-malignant-peripheral-nerve-sheath-tumors-including-ini1-smarcb1-results-and-braf-v600e-analysis
#20
Bharat Rekhi, Kemal Kosemehmetoglu, Gaye Guler Tezel, Sergulen Dervisoglu
Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare, relatively less chemosensitive sarcoma. We report clinicopathologic features of 11 epithelioid MPNSTs, including rare forms, along with INI1 immunostaining and BRAF V600E mutation results. BRAF V600E mutation was tested by Real-time polymerase chain reaction (PCR) technique. Eleven tumors occurred in six men and five women (M:F ratio = 0.85:1) within an age range of 5-73 years (average = 44), mostly in lower limbs (five), followed by upper limbs (four)...
April 27, 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
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