Genetics of rare mesenchymal tumors: Implications for targeted treatment in DFSP,ASPS, CCS,GCTB and PEComa |
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| Institution: | 1. Biotechnology Research Center, Mashhad University of Medical Sciences, Mashhad 91775–1365, Iran;2. Department of Pharmaceutical Biotechnology, School of Pharmacy, Mashhad University of Medical Sciences, Mashhad 91775–1365, Iran;3. Cancer Research Center, Shohada Hospital, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran 1931746345, Iran;4. Department of Biochemistry, Mashhad Branch, Islamic Azad University of Mashhad, Mashhad 91735/413, Iran;5. Cancer Molecular Pathology Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad 91766–99199, Iran;6. Department of Radiation Oncology, University of Toronto, Toronto M2N0C8, Canada;1. Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO;2. Washington University, St. Louis, MO;3. Division of Endocrinology, Metabolism and Lipid Research, Washington University School of Medicine, St. Louis, MO |
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| Abstract: | Soft tissue and bone sarcomas comprise a heterogeneous group of mesenchymal tumors that include roughly 130 distinct diagnostic entities. Many of them are exceptionally rare, with only few cases diagnosed worldwide each year. Development of novel targeted treatment in this group of tumors is of special importance since many sarcoma subtypes are resistant to conventional chemotherapy and the effective therapeutic options are limited. In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas).This article is part of a Directed Issue entitled: Rare cancers. |
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| Keywords: | Targeted treatment Dermatofibrosarcoma protuberans Alveolar soft part sarcoma Clear cell sarcoma Giant cell tumor of bone Perivascular epithelioid cell neoplasms |
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