Critical reviews in oncology/hematology · 2023
Special features of sarcomas developed in patients with Lynch syndrome: A systematic review.
Систематический обзорСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Poumeaud François, Valentin Thibaud, Vande Perre Pierre, Jaffrelot Marion, Bonnet Delphine, Chibon Frédéric, Chevreau Christine, Selves Janick, Guimbaud Rosine, Fares Nadim
Аннотация
Lynch syndrome (LS) is a genetic predisposition leading to colorectal and non-colorectal tumors such as endometrial, upper urinary tract, small intestine, ovarian, gastric, biliary duct cancers and glioblastoma. Though not classically associated with LS, growing literature suggests that sarcomas might develop in patients with LS. This systematic review of literature identified 44 studies (N = 95) of LS patients who developed sarcomas. It seems that most sarcomas developed in patients with a germline mutation of MSH2 (57 %) exhibit a dMMR (81 %) or MSI (77 %) phenotype, as in other LS-tumors. Although undifferentiated pleomorphic sarcoma (UPS), leiomyosarcoma, and liposarcoma remain the most represented histologic subtype, a higher proportion of rhabdomyosarcoma (10 %, especially pleomorphic rhabdomyosarcoma) is reported. Further studies are required to better characterize this sub-population.