Journal article

Ultra-rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

Silvia Stacchiotti, Anna Maria Frezza, Jean-Yves Blay, Elizabeth H Baldini, Sylvie Bonvalot, Judith VMG Bovee, Dario Callegaro, Paolo G Casali, RuRu Chun-ju Chiang, George D Demetri, Elisabeth G Demicco, Jayesh Desai, Mikael Eriksson, Hans Gelderblom, Suzanne George, Mrinal M Gounder, Alessandro Gronchi, Abha Gupta, Rick L Haas, Andrea Hayes-Jardon Show all

CANCER | WILEY | Published : 2021

Abstract

BACKGROUND: Among sarcomas, which are rare cancers, many types are exceedingly rare; however, a definition of ultra-rare cancers has not been established. The problem of ultra-rare sarcomas is particularly relevant because they represent unique diseases, and their rarity poses major challenges for diagnosis, understanding disease biology, generating clinical evidence to support new drug development, and achieving formal authorization for novel therapies. METHODS: The Connective Tissue Oncology Society promoted a consensus effort in November 2019 to establish how to define ultra-rare sarcomas through expert consensus and epidemiologic data and to work out a comprehensive list of these disease..

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