%0 Journal Article
%T A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl).
%A Palmerini E
%A Gambarotti M
%A Italiano A
%A Nathenson MJ
%A Ratan R
%A Dileo P
%A Provenzano S
%A Jones RL
%A DuBois SG
%A Martin-Broto J
%A de Alava E
%A Baldi GG
%A Grignani G
%A Ferraresi V
%A Brunello A
%A Paoluzzi L
%A Bertulli R
%A Hindi N
%A Montemurro M
%A Rothermundt C
%A Cocchi S
%A Salguero-Aranda C
%A Donati D
%A Martin JD
%A Abdelhamid Ahmed AH
%A Mazzocca A
%A Carretta E
%A Cesari M
%A Pierini M
%A Righi A
%A Sbaraglia M
%A Laginestra MA
%A Scotlandi K
%A Dei Tos AP
%A Ibrahim T
%A Stacchiotti S
%A Vincenzi B
%J Eur J Cancer
%V 183
%N 0
%D 04 2023
%M 36791667
%F 10.002
%R 10.1016/j.ejca.2023.01.003
%X Undifferentiated small round cell sarcomas (URCSs) represent a diagnostic challenge, and their optimal treatment is unknown. We aimed to define the clinical characteristics, treatment, and outcome of URCS patients.<BR>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.<BR>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).<BR>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.