Presentation and outcome of frequent and rare sarcoma histologic subtypes: A study of 10,262 patients with localized visceral/soft tissue sarcoma managed in reference centers

Nicolas Penel, Jean Michel Coindre, Antoine Giraud, Philippe Terrier, Dominique Ranchere-Vince, Françoise Collin, Sophie L.E. Guellec, Céline Bazille, Marick Lae, Gonzague de Pinieux, Isabelle L. Ray-Coquard, Sylvie Bonvalot, Axel L.E. Cesne, Yves Marie Robin, Eberhardt Stoeckle, Maud Toulmonde, Jean Yves Blay

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    Abstract

    BACKGROUND: The objective of this study was to describe characteristics at diagnosis and outcomes of adults with soft tissue sarcoma. METHODS: The authors conducted a retrospective multicenter study of 12,262 patients who were treated between January 1980 and 31 December 2013 in French Sarcoma Group centers and enrolled in the “Conticabase.” Diagnoses were systematically reviewed by expert pathologists, and entities were classified according to the 2013 World Health Organization classification. Diagnostic characteristics, treatments, and outcomes are described for the entire cohort, for the subgroup of patients with translocation-related sarcomas, and for 9 different histologic subtypes. RESULTS: The results stressed the magnitude of heterogeneity among adult sarcomas. For example, compared with other sarcomas, translocation-related sarcomas (2143 tumors; 20.8%) were associated with a younger age at presentation (40.6 vs 60.0 years; P <.0001), a low rate of predisposing conditions (0.01% vs 22.3%; P <.0001), a higher rate of lymph node involvement (4.7% vs 1.3%; P <.0001), and a higher rate of synchronous metastasis (11.9% vs 6.7%; P <.001); and complete (R0) resection (41.6% vs 31.9%; P <.0001), receipt of (neo)adjuvant radiation therapy (62.6% vs 42.2%; P <.0001), and receipt of (neo)adjuvant chemotherapy (36.6% vs 22.3%; P <.0001) were significantly more frequent. Overall, translocation-related sarcomas were associated with a lower rate of local relapse (18.1% vs 26.0%; P <.0001) but a higher rate of metastatic relapse (42.0% vs 30.7%; P <.0001). CONCLUSIONS: Collaborative efforts are urgently needed to better assess the natural history and management options for every histologic subtype of sarcoma. Cancer 2018;124:1179-87.

    Original languageEnglish
    Pages (from-to)1179-1187
    Number of pages9
    JournalCancer
    Volume124
    Issue number6
    DOIs
    Publication statusPublished - 15 Mar 2018

    Keywords

    • database
    • outcome
    • reference centers
    • soft tissue sarcoma
    • translocation-related sarcoma

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