Soft tissue sarcomas

Lewis, Jonathan J.; Brennan, Murray F.

doi:10.1016/s0011-3840(96)80013-x

Cited by 224 publications

(161 citation statements)

References 141 publications

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“…23 Although MPNSTs occur sporadically, after radiotherapy and in individuals with the autosomal dominant tumor susceptibility syndrome neurofibromatosis type 1 (NF1), our current understanding of the mechanisms responsible for MPNST pathogenesis in humans is derived almost exclusively from studies of NF1-associated MPNSTs. I begin by discussing the mechanisms responsible for the pathogenesis of plexiform neurofibromas, the benign precursors that give rise to MPNSTs in NF1 patients, and their transformation into MPNSTs.…”

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confidence: 99%

The Challenge of Cancer Genomics in Rare Nervous System Neoplasms

Carroll

2016

The American Journal of Pathology

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Comprehensive genomic analyses of common nervous system cancers provide new insights into their pathogenesis, diagnosis, and treatment. Although analogous studies of rare nervous system tumors are needed, there are major barriers to performing such studies. Cross-species comparative oncogenomics, identifying driver mutations in mouse cancer models and validating them in human tumors, is a promising alternative. Although still in its infancy, this approach is being applied to malignant peripheral nerve sheath tumors (MPNSTs), rare Schwann cellederived malignancies that occur sporadically, after radiotherapy, and in neurofibromatosis type 1. Studies of human neurofibromatosis type 1eassociated tumors suggest that NF1 tumor suppressor loss in Schwann cells triggers cell-autonomous and intercellular changes, resulting in development of benign neurofibromas; subsequent neurofibroma-MPNST progression is caused by aberrant growth factor signaling and mutations affecting the p16 INK4A -cyclin D1-CDK4-Rb and p19 ARF -Mdm2-p53 cell cycle pathways. Mice with Nf1, Trp53, and/or Cdkn2a mutations that overexpress the Schwann cell mitogen neuregulin-1 or overexpress the epidermal growth factor receptor validate observations in human tumors and, to various degrees, model human tumorigenesis. Genomic analyses of MPNSTs arising in neuregulin-1 and epidermal growth factor receptor-overexpressing mice and forward genetic screens with Sleeping Beauty transposons implicate additional signaling cascades in MPNST pathogenesis. These studies confirm the utility of mouse models for MPNST driver gene discovery and provide new insights into the complexity of MPNST pathogenesis. (Am J Pathol 2016, 186: 464e477; http://dx

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The Challenge of Cancer Genomics in Rare Nervous System Neoplasms

Carroll

2016

The American Journal of Pathology

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“…[1][2][3] The overall 5-and 10-year survival rate were 34 and 22%, respectively. 4 The rates of successful treatment of MPNST are generally low, and the available literature provides only limited guidance regarding the management of this disease.…”

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Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB

Aoki

Nabeshima

Koga

et al. 2007

Laboratory Investigation

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Malignant peripheral nerve sheath tumor (MPNST) is rare, highly aggressive, resistant to radiochemotherapy, and associated with poor prognosis. Basic research to develop new treatment regimes is critically needed. This study was designed to identify motogenic factor(s) involved in MPNST cell invasion and inhibitor(s) of such invasive activity. We profiled the invasion-inducing activities of eight motogenic growth factors on two human MPNST cell lines, FU-SFT8611 and 9817, using in vitro Matrigel invasion assays. Platelet-derived growth factor-BB (PDGF-BB) was identified as the most effective MPNST cell invasion-inducing factor. Epidermal growth factor (EGF) and hepatocyte growth factor (HGF) also stimulated invasion in one MPNST cell line. Expressions of PDGF-BB and EGF receptors (PDGFR-b and EGFR) mRNAs were detected more frequently and their proteins were expressed at higher levels in MPNST tissues than benign peripheral nerve sheath tumors (schwannomas and neurofibromas). In both MPNST cell lines, PDGF-BB induced tyrosine phosphorylation of PDGFR-b but not of PDGFR-a, and specific PDGFR-b inhibition by small interfering RNA to the receptor inhibited PDGF-BBstimulated MPNST cell invasion, suggesting the predominant role of PDGFR-b. Inhibition of PDGFR-b phosphorylation by pretreatment with herbimycin A and imatinib mesylate effectively suppressed basement membrane invasion and cell growth in vitro. No mutations were present in exons 12 and 18 of PDGFR-b in both MPNST cell lines and 10 human MPNST tissues examined. Our results indicated that PDGF-BB enhanced the invasive activity of MPNST cells through PDGFR phosphorylation and that imatinib inhibited such activity. The results provide the ground for further assessment of the therapeutic potential of imatinib in suppressing the invasion and growth of MPNST.

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“…The presence of tumor extension beyond the principal tumor mass has also been described for other types of soft tissue sarcomas. 26,27 In the largest reported series to date, 4 HPC recurred in17% of the cases. Our experience is that conventional HPC has two-and five year overall survival rates of 93% and 86%, respectively.…”

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confidence: 99%

Conventional hemangiopericytoma

Espat

Lewis

Leung

et al. 2002

Cancer

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183

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BACKGROUNDHemangiopericytoma (HPC) is a rare vascular tumor, and pathologic distinction from synovial sarcoma and solitary fibrous tumor is a significant problem due to shared histologic features. In the current report the authors defined the clinical behavior and prognosis for patients with HPC.METHODSBetween July 1982 and February 1998, 62 patients with a diagnosis of primary, recurrent, or metastatic HPC were identified from a prospectively maintained database. The pathology of all cases for which material was available (57 cases) was re‐reviewed for histologic confirmation of the HPC diagnosis. Using strict pathologic criteria, including immunohistochemistry and electron microscopy, tumors from 25 of 57 patients qualified for the diagnosis of conventional hemangiopericytoma; those tumors formed the basis of the current report. Survival was determined by the Kaplan‐Meier method.RESULTSAt the time of initial presentation, 19 patients had primary tumors, 3 had locally recurrent disease, and 3 had metastatic disease. The most frequent anatomic sites for HPC were the extremities, the pelvis, and the head and neck, accounting for 80% of the total cases. The median followup (n = 25) was 49 months (range, 1 to 160 months). The two and five year overall survival rates (n = 25) were 93% and 86% respectively. The disease‐specific survival was 86% at last followup. Patients undergoing complete resection (n = 16) showed a 100% median survival at 60 months.CONCLUSIONSAt present, complete tumor resection for patients with conventional HPC is recommended. However, considering the favorable outcome in this disease, the authors caution against performing operations that may potentially cause loss of function or are limb threatening. Cancer 2002;95:1746–51. © 2002 American Cancer Society.DOI 10.1002/cncr.10867

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Soft tissue sarcomas

Cited by 224 publications

References 141 publications

The Challenge of Cancer Genomics in Rare Nervous System Neoplasms

The Challenge of Cancer Genomics in Rare Nervous System Neoplasms

Imatinib mesylate inhibits cell invasion of malignant peripheral nerve sheath tumor induced by platelet-derived growth factor-BB

Conventional hemangiopericytoma

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