Genetics of rare mesenchymal tumors: implications for targeted treatment in DFSP, ASPS, CCS, GCTB and PEComa.

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.

• Authors: Piotr Rutkowski, Joanna Przybył, Tomasz Świtaj
• Journal: The international journal of biochemistry & cell biology (Int J Biochem Cell Biol) Vol. 53 Pg. 466-74 (Aug 2014) ISSN: 1878-5875 [Electronic] Netherlands
• PMID: 24704529 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
• Copyright: Copyright © 2014 Elsevier Ltd. All rights reserved.

Topics

• Dermatofibrosarcoma (genetics, pathology, therapy)
• Giant Cell Tumor of Bone (genetics, pathology, therapy)
• Humans
• Mesoderm (pathology)
• Molecular Targeted Therapy
• Perivascular Epithelioid Cell Neoplasms (genetics, pathology, therapy)
• Sarcoma, Alveolar Soft Part (genetics, pathology, therapy)
• Sarcoma, Clear Cell (genetics, pathology, therapy)