CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl)
Copyright © 2023 Elsevier Ltd. All rights reserved..
BACKGROUND: 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.
CCNB3-rearranged round cell sarcomas, (3) unclassified URCSs. Treatment, prognostic factors and outcome were reviewed.
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:183 |
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| Enthalten in: |
European journal of cancer (Oxford, England : 1990) - 183(2023) vom: 01. Apr., Seite 11-23 |
| Sprache: |
Englisch |
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| Links: |
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| Anmerkungen: |
Date Completed 22.03.2023 Date Revised 25.03.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.ejca.2023.01.003 |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM352982055 |
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| 024 | 7 | |a 10.1016/j.ejca.2023.01.003 |2 doi | |
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| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Palmerini, Emanuela |e verfasserin |4 aut | |
| 245 | 1 | 2 | |a A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl) |
| 264 | 1 | |c 2023 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 22.03.2023 | ||
| 500 | |a Date Revised 25.03.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2023 Elsevier Ltd. All rights reserved. | ||
| 520 | |a BACKGROUND: 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 | ||
| 520 | |a 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 | ||
| 520 | |a 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) | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a BCOR::CCNB3 | |
| 650 | 4 | |a Bone sarcoma | |
| 650 | 4 | |a CIC::DUX4 | |
| 650 | 4 | |a Pediatric tumors | |
| 650 | 4 | |a RNA-seq | |
| 650 | 4 | |a Soft tissue sarcoma | |
| 650 | 4 | |a Ultra rare sarcoma | |
| 650 | 4 | |a Undifferentiated round cell sarcoma | |
| 650 | 7 | |a BCOR protein, human |2 NLM | |
| 650 | 7 | |a Biomarkers, Tumor |2 NLM | |
| 650 | 7 | |a CCNB3 protein, human |2 NLM | |
| 650 | 7 | |a Cyclin B |2 NLM | |
| 650 | 7 | |a Oncogene Proteins, Fusion |2 NLM | |
| 650 | 7 | |a Proto-Oncogene Proteins |2 NLM | |
| 650 | 7 | |a Repressor Proteins |2 NLM | |
| 650 | 7 | |a CIC protein, human |2 NLM | |
| 700 | 1 | |a Gambarotti, Marco |e verfasserin |4 aut | |
| 700 | 1 | |a Italiano, Antoine |e verfasserin |4 aut | |
| 700 | 1 | |a Nathenson, Michael J |e verfasserin |4 aut | |
| 700 | 1 | |a Ratan, Ravin |e verfasserin |4 aut | |
| 700 | 1 | |a Dileo, Palma |e verfasserin |4 aut | |
| 700 | 1 | |a Provenzano, Salvatore |e verfasserin |4 aut | |
| 700 | 1 | |a Jones, Robin L |e verfasserin |4 aut | |
| 700 | 1 | |a DuBois, Steven G |e verfasserin |4 aut | |
| 700 | 1 | |a Martin-Broto, Javier |e verfasserin |4 aut | |
| 700 | 1 | |a de Alava, Enrique |e verfasserin |4 aut | |
| 700 | 1 | |a Baldi, Giacomo G |e verfasserin |4 aut | |
| 700 | 1 | |a Grignani, Giovanni |e verfasserin |4 aut | |
| 700 | 1 | |a Ferraresi, Virginia |e verfasserin |4 aut | |
| 700 | 1 | |a Brunello, Antonella |e verfasserin |4 aut | |
| 700 | 1 | |a Paoluzzi, Luca |e verfasserin |4 aut | |
| 700 | 1 | |a Bertulli, Rossella |e verfasserin |4 aut | |
| 700 | 1 | |a Hindi, Nadia |e verfasserin |4 aut | |
| 700 | 1 | |a Montemurro, Michael |e verfasserin |4 aut | |
| 700 | 1 | |a Rothermundt, Christian |e verfasserin |4 aut | |
| 700 | 1 | |a Cocchi, Stefania |e verfasserin |4 aut | |
| 700 | 1 | |a Salguero-Aranda, Carmen |e verfasserin |4 aut | |
| 700 | 1 | |a Donati, Davide |e verfasserin |4 aut | |
| 700 | 1 | |a Martin, Juan D |e verfasserin |4 aut | |
| 700 | 1 | |a Abdelhamid Ahmed, Amr H |e verfasserin |4 aut | |
| 700 | 1 | |a Mazzocca, Alessandro |e verfasserin |4 aut | |
| 700 | 1 | |a Carretta, Elisa |e verfasserin |4 aut | |
| 700 | 1 | |a Cesari, Marilena |e verfasserin |4 aut | |
| 700 | 1 | |a Pierini, Michela |e verfasserin |4 aut | |
| 700 | 1 | |a Righi, Alberto |e verfasserin |4 aut | |
| 700 | 1 | |a Sbaraglia, Marta |e verfasserin |4 aut | |
| 700 | 1 | |a Laginestra, Maria A |e verfasserin |4 aut | |
| 700 | 1 | |a Scotlandi, Katia |e verfasserin |4 aut | |
| 700 | 1 | |a Dei Tos, Angelo P |e verfasserin |4 aut | |
| 700 | 1 | |a Ibrahim, Toni |e verfasserin |4 aut | |
| 700 | 1 | |a Stacchiotti, Silvia |e verfasserin |4 aut | |
| 700 | 1 | |a Vincenzi, Bruno |e verfasserin |4 aut | |
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