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. Author manuscript; available in PMC: 2018 Aug 1.
Published in final edited form as: Trends Mol Med. 2017 Jul 20;23(8):737–755. doi: 10.1016/j.molmed.2017.06.004

Figure 2. Familial Syndromes and Osteosarcoma.

Figure 2

A cluster of familial syndromes predispose patients to the development of osteosarcomas and are of relevance to understanding the underlying genetics of these tumors. These include LFS, RB, RTS, RAPA, WS, BS, DBA, and PDB. Each inherited syndrome harbors distinct gene mutations but shares a cancer predisposition to osteosarcoma. AD, autosomal dominant; AR, autosomal recessive. The dysregulation of variant biological processes (e.g., imprinted gene network, osteogenic differentiation, genomic integrity, protein translation, cell cycle and autophagy) and signaling (e.g., p53 and NF-κB) contributes to the syndrome-associated osteosarcomagenesis. The syndrome-associated risk of osteosarcoma is stated as percentage of patients with disorder developing osteosarcoma. *RTS indicates RTS Type II.