A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl).
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Palmerini Emanuela, Gambarotti Marco, Italiano Antoine, Nathenson Michael J, Ratan Ravin, Dileo Palma, Provenzano Salvatore, Jones Robin L, DuBois Steven G, Martin-Broto Javier, de Alava Enrique, Baldi Giacomo G, Grignani Giovanni, Ferraresi Virginia, Brunello Antonella, Paoluzzi Luca, Bertulli Rossella, Hindi Nadia, Montemurro Michael, Rothermundt Christian, Cocchi Stefania, Salguero-Aranda Carmen, Donati Davide, Martin Juan D, Abdelhamid Ahmed Amr H, Mazzocca Alessandro, Carretta Elisa, Cesari Marilena, Pierini Michela, Righi Alberto, Sbaraglia Marta, Laginestra Maria A, Scotlandi Katia, Dei Tos Angelo P, Ibrahim Toni, Stacchiotti Silvia, Vincenzi Bruno
Аннотация
METHODS: URCS patients treated from 1983 to 2019 at 21 worldwide sarcoma reference centres were retrospectively identified. Based on molecular assessment, cases were classified as follows: (1) CIC-rearranged round cell sarcomas, (2) BCOR::CCNB3-rearranged round cell sarcomas, (3) unclassified URCSs. Treatment, prognostic factors and outcome were reviewed.
RESULTS: In total, 148 patients were identified [88/148 (60%) CIC-rearranged sarcoma (median age 32 years, range 7-78), 33/148 (22%) BCOR::CCNB3-rearranged (median age 17 years, range 5-91), and 27/148 (18%) unclassified URCSs (median age 37 years, range 4-70)]. One hundred-one (68.2%) cases presented with localised disease; 47 (31.8%) had metastases at diagnosis. Male prevalence, younger age, bone primary site, and a low rate of synchronous metastases were observed in BCOR::CCNB3-rearranged cases. Local treatment was surgery in 67/148 (45%) patients, and surgery + radiotherapy in 52/148 (35%). Chemotherapy was given to 122/148 (82%) patients. At a 42.7-month median follow-up, the 3-year overall survival (OS) was 92.2% (95% CI 71.5-98.0) in BCOR::CCNB3 patients, 39.6% (95% CI 27.7-51.3) in CIC-rearranged sarcomas, and 78.7% in unclassified URCSs (95% CI 56.1-90.6; p < 0.0001).
CONCLUSIONS: This study is the largest conducted in URCS and confirms major differences in outcomes between URCS subtypes. A full molecular assessment should be undertaken when a diagnosis of URCS is suspected. Prospective studies are needed to better define the optimal treatment strategy in each URCS subtype.