Nerve Sheath Neoplasms (Nerve Sheath Neoplasm)

Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.

Also Known As:

Networked: 552 relevant articles (26 outcomes, 63 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Nerve Sheath Neoplasms (Nerve Sheath Neoplasm)
3.	Sarcoma (Soft Tissue Sarcoma)
4.	Synovial Sarcoma (Synovioma)
5.	Liposarcoma

Experts

1.	Carroll, Steven L: 17 articles (06/2015 - 08/2003)
2.	Ratner, Nancy: 15 articles (12/2015 - 01/2005)
3.	Gutmann, David H: 12 articles (09/2015 - 09/2002)
4.	Upadhyaya, Meena: 12 articles (07/2015 - 02/2004)
5.	Kluwe, Lan: 12 articles (01/2014 - 03/2006)
6.	von Deimling, Andreas: 11 articles (04/2014 - 02/2004)
7.	Guha, Abhijit: 11 articles (04/2012 - 09/2002)
8.	Byer, Stephanie J: 10 articles (06/2015 - 08/2005)
9.	Lev, Dina: 10 articles (01/2015 - 01/2009)
10.	Mautner, Victor: 10 articles (04/2014 - 04/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Nerve Sheath Neoplasms:

1.	imatinib (Gleevec)FDA Link 06/15/2014 - "Taken together, our data strongly suggest that PLX3397 is superior to imatinib in the treatment of MPNSTs, and the combination of PLX3397 with a TORC1 inhibitor could provide a new therapeutic approach for the treatment of this disease." 01/01/2013 - "The results suggest that imatinib mesylate may be useful in the treatment of MPNST patients and in vitro studies may help select cells that are sensitive to imatinib mesylate in vivo." 06/15/2014 - "We tested the c-Kit inhibitor, imatinib, and PLX3397, a selective c-Fms and c-Kit inhibitor, to evaluate their efficacy against MPNST cells in vitro and in vivo. " 04/01/2014 - "Our data showed that the imatinib-mediated autophagy can function as a cytotoxic mechanism and that appropriate modulation of autophagy may sensitize MPNST cells to imatinib, which in turn may be a novel therapeutic strategy for MPNST. " 04/01/2014 - "We found that the treatment of MPNST cell lines with imatinib induced autophagy. "
2.	Sirolimus (Rapamycin)FDA Link 05/01/2008 - "Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors." 01/15/2010 - "This study demonstrates the therapeutic potential and limitations of rapamycin in NF1-associated, and likely sporadic, MPNSTs." 01/01/2012 - "To determine the significance of Pten status for treatment with the mTOR inhibitor rapamycin we treated 5 MPNST cell lines with rapamycin. " 05/01/2009 - "MPNST cells were sensitive to rapamycin; however, rapamycin enhanced pAKT and peIF4E expression. " 01/01/2010 - "Finally, increased knowledge of molecular and cellular mechanisms involved with NF1 tumorigenesis has led to multiple preclinical and clinical studies of targeted therapy, including the mTOR inhibitor rapamycin, which is demonstrating promising preclinical results for treatment of MPNSTs and gliomas."
3.	Doxorubicin (Adriamycin)FDA LinkGeneric 02/01/2013 - "We also discovered that the increased Bcl-xL expression caused an increase in drug resistance to doxorubicin in MPNST-derived SCs. " 06/15/2009 - "These findings indicate that constitutive hyaluronan-CD44 interactions contribute to drug transporter localization and function at the plasma membrane, and that attenuating hyaluronan-CD44 interactions sensitizes MPNSTs to doxorubicin in vitro and in vivo. " 01/01/2013 - "In a subset of MPNSTs, high expression of ABCC1 is observed, serving as a predicted contra-indication for doxorubicin and related therapeutics in these patients. " 06/15/2009 - "MPNSTs are multidrug resistant, and thus long-term patient survival rates are poor after standard doxorubicin or multiagent chemotherapies. " 01/01/2013 - "Despite TOP2A overexpression in these tumors, doxorubicin resistance is common, and the mechanisms of chemotherapy resistance in MPNST are poorly understood. "
4.	TamoxifenFDA LinkGeneric 01/01/2011 - "Tamoxifen was also effective in vivo, demonstrating potent antitumor activity in mice orthotopically xenografted with human MPNST cells. " 01/01/2011 - "The in vivo effectiveness of tamoxifen provides a rationale for clinical trials in cases of MPNSTs." 01/01/2011 - "Further, ablation of ERβ and GPER had no effect on MPNST proliferation, survival, or tamoxifen sensitivity, indicating that tamoxifen acts via an ER-independent mechanism. " 01/01/2011 - "To test this hypothesis, we examined tamoxifen effects on MPNST cell proliferation and survival, MPNST xenograft growth, and the mechanism by which tamoxifen impeded these processes. " 11/01/2014 - "Kinomic analyses showed that tamoxifen and trifluoperazine have both shared and distinct targets in MPNSTs. "
5.	Dermatofibrosarcoma protuberansIBA 06/01/2012 - "In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). " 10/15/1996 - "Two striking observations were made: (a) an increase in copy number of the distal part of the long arm of chromosome 17, with the smallest region of overlap 17q24-qter, was seen in five of seven MPNSTs and in the only dermatofibrosarcoma protuberans, all of which were from patients with neurofibromatosis, whereas none of the three sporadic MPNSTs had this alteration; and (b) loss of 13q, with the smallest region of overlap 13q14-q21, was found in 6 of 10 MPNSTs. " 10/15/1996 - "We used comparative genomic hybridization to analyze seven MPNSTs and one dermatofibrosarcoma protuberans from eight patients with von Recklinghausen's disease (neurofibromatosis type 1), as well as three sporadic MPNSTs. " 07/01/2001 - "A differential diagnosis of fibrous histiocytoma, dermatofibrosarcoma protuberans, acquired (digital) fibrokeratoma, sclerosing perineurioma, cutaneous myxoma (superficial angiomyxoma), and acral myxoinflammatory fibroblastic sarcoma is discussed." 09/01/2002 - "The revised cases included 78 leiomyosarcomas (57%; ordinary leiomyosarcoma, 45 cases; pleomorphic leiomyosarcoma, 23 cases; myxoid leiomyosarcoma, 10 cases), 12 liposarcomas (9%; pleomorphic liposarcoma, 11 cases; dedifferentiated liposarcoma, one case), seven dermatofibrosarcoma protuberans (5%), six unclassified sarcomas (4%), five primary or metastatic carcinomas (4%), four low-grade fibromyxoid sarcomas (3%), four inflammatory myofibroblastic tumors (3%), three rhabdomyosarcomas (2%), three malignant peripheral nerve sheath tumors (2%), three acral myxoinflammatory fibroblastic sarcomas (2%) and two atypical fibroxanthomas (1.5%). "
6.	MicroRNAs (MicroRNA)IBA 03/05/2013 - "Differential expression was validated by reverse transcription quantitative-PCR, and functional studies were performed after transfection of miRNA oligonucleotide mimics into MPNST cells. " 09/01/2012 - "Analysis of miRNA expression profiles in MPNST and NF revealed significantly altered expression levels of nine miRNAs, one of those, miR-21, and its putative target, programmed cell death protein 4 (PDCD4), were selected for further studies. " 12/01/2013 - "Genetic variants in the miRNA biogenesis pathway genes may modify MPNST risk both individually and jointly." 12/01/2013 - "A case-control study was performed to analyze 53 SNPs in 11 miRNA biogenesis pathway genes in 356 patients (200 patients with NF1 and 156 patients with both NF1 and MPNST) in China. " 06/01/2013 - "The combination of the three miRNAs (MiR-801, miR-214, and miR-24) could distinguish NF1 MPNST patients from NF1 patients with a sensitivity of 0.820 and a specificity of 0.844. "
7.	S100 Proteins (S 100 Protein)IBA 07/01/2009 - "This study indicates that the TMA technique can be used in the IHC study of MPNSTs, even with the use of heterogeneous markers such as S-100 protein." 10/01/2013 - "S-100 protein was expressed in 17 cases, including 81.8% of BPNSTs and 57.14% of MPNSTs. " 07/01/2013 - "Similar to S-100 protein, some MPNSTs show scattered positivity but did not show diffuse positivity seen in DM." 05/01/2011 - "The lack of S-100 protein expression made Schwann cells an unlikely cell of origin, as opposed to peripheral nerve sheath tumors, which typically express S-100 protein, at least in some neoplastic cells. " 03/01/2006 - "For example, S-100 protein is widely used in the diagnosis of MPNST. "
8.	Messenger RNA (mRNA)IBA 05/01/2004 - "This study represents a comprehensive gene-expression analysis of an MPNST-derived cell line and the first comprehensive global mRNA profile of nhSCs in culture. " 01/01/2013 - "Then it is confirmed that miR-210 can regulate EFNA3 mRNA and protein expression in MPNST ST88-14 (NF1 wild-type) and sNF96.2 (NF1 mutant type) cell lines. " 10/01/2012 - "MPNSTs (n=28) showed variable levels of Sox10 and S100 mRNA expression, and these expression levels were highly correlated (Pearson correlation coefficient r=0.79). " 05/01/2012 - "Human MPNST cell lines exhibited survivin mRNA and protein overexpression; expression in both nuclear and cytoplasmic compartments was noted. " 01/21/2010 - "EYA4 mRNA was elevated more than 100-fold as estimated by quantitative real-time PCR in most MPNST cell lines. "
9.	O-(chloroacetylcarbamoyl)fumagillol (TNP 470)IBA 03/01/1993 - "This study determined that AGM-1470 is effective in inhibiting angiogenesis and the growth of human nerve-sheath tumors." 03/01/1993 - "Inhibition of angiogenesis and growth of human nerve-sheath tumors by AGM-1470."
10.	Carbon MonoxideIBA 03/30/2014 - "These data suggest that co-targeting mTOR and MEK may be effective in patients with sporadic or NF1-associated MPNSTs. " 03/01/2015 - "While treatment has traditionally focused on attempts at radical resection, a lack of consensus among surgeons has hindered the establishment of a well-defined algorithm to guide the management of these highly co-morbid peripheral nerve sheath tumors. " 11/01/2014 - "Additionally, we identified frequent somatic alterations of CDKN2A (81% of all MPNSTs) and NF1 (72% of non-NF1-associated MPNSTs), both of which significantly co-occur with PRC2 alterations. " 07/01/2014 - "Human gene copy number alteration data, microarray expression data, and TMA analysis indicate that TP53 haploinsufficiency and increased EGFR expression co-occur in human MPNST samples. " 08/01/2013 - "For the subset of these genes found in human MPNST CNAs, only one quarter of their orthologues were co-gained or co-lost in zebrafish, dramatically narrowing the list of candidate cancer drivers for both focal and large CNAs. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 06/01/2015 - "On multivariate analysis, MPNST histology, chemotherapy-resistant histology, and higher risk group were significantly poor prognostic factors for OS and CSS. " 01/01/2014 - "There is not enough data about the role of systemic chemotherapy in the management of MPNSTs and it still remains controversial. " 02/01/2013 - "These findings suggest that FTS, alone or in combination with chemotherapy agents, may be worth developing as a possible treatment for MPNSTs." 01/01/2013 - "Also, mechanisms involving drug transport and DNA damage repair are primary mediators of MPNST chemotherapy resistance. " 01/01/2011 - "this series indicates the role of chemotherapy in treatment of advanced MPNST. "
2.	Radiotherapy 12/01/2015 - "Surgical resection confers survival benefit in patients with early stage MPNST, while radiotherapy improves survival in cases with metastatic disease. " 07/01/2015 - "Unfortunately, the patient's MPNST was inoperable, and he received palliative radiotherapy for local control of the disease. " 04/01/2015 - "All 43 MPNST patients (25 men and 18 women; median age 49 years) underwent surgical resection, of whom 15 patients also underwent postoperative radiotherapy. " 01/01/2015 - "In addition, six locally advanced/metastatic MPNST were treated with chemo/radiotherapy, and two BPNST had no progression after a follow-up of 14 and 39 months, respectively. " 11/01/2014 - "Using comprehensive genomic approaches, we identified loss-of-function somatic alterations of the Polycomb repressive complex 2 (PRC2) components (EED or SUZ12) in 92% of sporadic, 70% of NF1-associated and 90% of radiotherapy-associated MPNSTs. "
3.	Heterologous Transplantation (Xenotransplantation) 10/02/2015 - "These effects were confirmed in vivo, such that TAK-960 more effectively inhibited CHP100 than MPNST xenografts. " 01/01/2015 - "In vivo growth effects of HDAC8i were evaluated using MPNST xenograft models. " 05/01/2014 - "In vivo growth effects of the drug combination with local radiation therapy (RT) were assessed in MPNST xenografts. " 01/01/2014 - "Furthermore, in two different human MPNST xenograft models, the human sAXL could be detected in the mouse plasma. " 12/01/2013 - "In xenografts of S462 MPNST cells in athymic nude mice, PEDF suppressed MPNST tumour burden, due mainly to inhibition of angiogenesis. "
4.	Therapeutics 01/01/2013 - "Alternative therapeutics supported by the molecular-guided therapy predictions are reported and tested in MPNST-derived cells. " 02/01/2007 - "Testing of therapeutics for MPNST has been hampered by lack of adequate xenograft models. " 07/01/2004 - "We conclude that distinct molecular classes of MPNST exist and that the ability to stratify these tumors based on unique and biologically relevant gene expression profiles may be important for future targeted therapeutics." 12/01/2015 - "Our results show that MPNSTs depend on lipid metabolic pathways and suggest that disrupting lipid metabolism could be a potential new strategy for the development of MPNST therapeutics." 01/21/2010 - "Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas without effective therapeutics. "
5.	Adjuvant Radiotherapy 01/01/2014 - "Our data series confirms that a combined-modality approach with complete surgical resection and adjuvant radiotherapy leads to improved outcomes in MPNSTs of the head and neck. " 06/01/2013 - "We present a case of MPNST of the chest wall in a patient with NF-1 who developed local recurrence 5 months after complete surgical resection and postoperative adjuvant radiotherapy. " 03/01/1995 - "Adjuvant radiotherapy was used in 48% of deep and high-grade MPNSTs, and 3-year local disease-free survival was 70%. " 01/01/2010 - "Adjuvant radiotherapy and chemotherapy were not administered since these adjuvant therapies generally do not improve survival in MPNST and may cause additional neurovascular damage. " 05/01/2015 - "The 2 patients with MPNST were free of recurrence after tumor resection and in one case after adjuvant radiotherapy. "