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  1. Article ; Online: Complex Elucidation of Cells-of-Origin in Pediatric Soft Tissue Sarcoma: From Concepts to Real Life, Hide-and-Seek through Epigenetic and Transcriptional Reprogramming.

    Savary, Clara / Picard, Cécile / Corradini, Nadège / Castets, Marie

    International journal of molecular sciences

    2022  Volume 23, Issue 11

    Abstract: Soft tissue sarcoma (STS) comprise a large group of mesenchymal malignant tumors with heterogeneous cellular morphology, proliferative index, genetic lesions and, more importantly, clinical features. Full elucidation of this wide diversity remains a ... ...

    Abstract Soft tissue sarcoma (STS) comprise a large group of mesenchymal malignant tumors with heterogeneous cellular morphology, proliferative index, genetic lesions and, more importantly, clinical features. Full elucidation of this wide diversity remains a central question to improve their therapeutic management and the identity of cell(s)-of-origin from which these tumors arise is part of this enigma. Cellular reprogramming allows transitions of a mature cell between phenotypes, or identities, and represents one key driver of tumoral heterogeneity. Here, we discuss how cellular reprogramming mediated by driver genes in STS can profoundly reshape the molecular and morphological features of a transformed cell and lead to erroneous interpretation of its cell-of-origin. This review questions the fact that the epigenetic context in which a genetic alteration arises has to be taken into account as a key determinant of STS tumor initiation and progression. Retracing the cancer-initiating cell and its clonal evolution, notably via epigenetic approach, appears as a key lever for understanding the origin of these tumors and improving their clinical management.
    MeSH term(s) Cellular Reprogramming/genetics ; Epigenesis, Genetic ; Epigenomics ; Humans ; Sarcoma/genetics ; Sarcoma/therapy ; Soft Tissue Neoplasms/therapy
    Language English
    Publishing date 2022-06-04
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms23116310
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Infantile fibrosarcoma: Is spontaneous regression possible?

    Orbach, Daniel / Sparber-Sauer, Monika / Corradini, Nadege / Ferrari, Andrea / Owens, Cormac / Casanova, Michela

    Pediatric blood & cancer

    2023  Volume 70, Issue 11, Page(s) e30623

    MeSH term(s) Humans ; Infant ; Soft Tissue Neoplasms ; Fibrosarcoma
    Language English
    Publishing date 2023-08-14
    Publishing country United States
    Document type Letter
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.30623
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  3. Article ; Online: Metronomic Maintenance Therapy for Rhabdomyosarcoma.

    André, Nicolas / Corradini, Nadège / Shaked, Yuval

    Trends in cancer

    2019  Volume 5, Issue 12, Page(s) 756–759

    Abstract: In a recent article, Bisogno et al. reported in a randomized trial that addition of metronomic maintenance therapy (MMT) with vinorelbine plus cyclophosphamide for children with rhabdomyosarcoma resulted in a significant increase in overall and event- ... ...

    Abstract In a recent article, Bisogno et al. reported in a randomized trial that addition of metronomic maintenance therapy (MMT) with vinorelbine plus cyclophosphamide for children with rhabdomyosarcoma resulted in a significant increase in overall and event-free survival. Although the mechanism of action remains to be fully elucidated, this study paves the way for further evaluation of MMT as a potential therapeutic strategy in pediatric patients with high-risk disease.
    MeSH term(s) Administration, Metronomic ; Antineoplastic Combined Chemotherapy Protocols ; Child ; Cyclophosphamide ; Humans ; Maintenance Chemotherapy ; Rhabdomyosarcoma ; Vinorelbine
    Chemical Substances Cyclophosphamide (8N3DW7272P) ; Vinorelbine (Q6C979R91Y)
    Language English
    Publishing date 2019-11-04
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 2852626-0
    ISSN 2405-8025 ; 2405-8033 ; 2405-8033
    ISSN (online) 2405-8025 ; 2405-8033
    ISSN 2405-8033
    DOI 10.1016/j.trecan.2019.10.004
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  4. Article ; Online: PET metabolic tumor volume as a new prognostic factor in childhood rhabdomyosarcoma.

    Helio Fayolle / Nina Jehanno / Valerie Lauwers-Cances / Marie-Pierre Castex / Daniel Orbach / Thomas Mognetti / Corradini Nadège / Pierre Payoux / Anne Hitzel

    PLoS ONE, Vol 17, Iss 1, p e

    2022  Volume 0261565

    Abstract: Purpose Childhood RMS is a rare malignant disease in which evaluation of tumour spread at diagnosis is essential for therapeutic management. F-18 FDG-PET imaging is currently used for initial RMS disease staging. Materials and methods This multicentre ... ...

    Abstract Purpose Childhood RMS is a rare malignant disease in which evaluation of tumour spread at diagnosis is essential for therapeutic management. F-18 FDG-PET imaging is currently used for initial RMS disease staging. Materials and methods This multicentre retrospective study in six French university hospitals was designed to analyse the prognostic accuracy of MTV at diagnosis for patients with RMS between 1 January 2007 and 31 October 2017, for overall (OS) and progression-free survival (PFS). MTV was defined as the sum of the primitive tumour and the largest metastasis, where relevant, with a 40% threshold of the primary tumour SUVmax. Additional aims were to define the prognostic value of SUVmax, SUVpeak, and bone lysis at diagnosis. Results Participants were 101 patients with a median age of 7.4 years (IQR [4.0-12.5], 62 boys), with localized disease (35 cases), regional nodal spread (43 cases), or distant metastases (23). 44 patients had alveolar subtypes. In a univariate analysis, a MTV greater than 200 cm3 was associated with OS (HR = 3.47 [1.79;6.74], p<0.001) and PFS (HR = 3.03 [1.51;6.07], p = 0.002). SUVmax, SUVpeak, and bone lysis also influenced OS (respectively p = 0.005, p = 0.004 and p = 0.007) and PFS (p = 0.029, p = 0.019 and p = 0.015). In a multivariate analysis, a MTV greater than 200 cm3 was associated with OS (HR = 2.642 [1.272;5.486], p = 0.009) and PFS (HR = 2.707 [1.322;5.547], p = 0.006) after adjustment for confounding factors, including SUVmax, SUVpeak, and bone lysis. Conclusion A metabolic tumor volume greater than 200 cm3, SUVmax, SUVpeak, and bone lysis in the pre-treatment assessment were unfavourable for outcome.
    Keywords Medicine ; R ; Science ; Q
    Subject code 616
    Language English
    Publishing date 2022-01-01T00:00:00Z
    Publisher Public Library of Science (PLoS)
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  5. Article ; Online: High-dose chemotherapy followed by whole lung irradiation in pulmonary relapse Ewing's sarcomas: a retrospective multicenter study.

    Duverge, Loig / Demoor-Goldschmidt, Charlotte / Laprie, Anne / Cervellera, Mathilde / Castex, Marie Pierre / Corradini, Nadège / Marec-Berard, Perrine / Claude, Line

    The British journal of radiology

    2022  Volume 95, Issue 1136, Page(s) 20220212

    Abstract: Objective: Regarding the efficiency of Bu-Mel-based high-dose-chemotherapy (Bu-Mel-HDCT) and whole lung irradiation (WLI), the objective was to evaluate the efficiency and safety of this salvage sequence in Ewing sarcoma (ES) lung relapses.: Methods: ...

    Abstract Objective: Regarding the efficiency of Bu-Mel-based high-dose-chemotherapy (Bu-Mel-HDCT) and whole lung irradiation (WLI), the objective was to evaluate the efficiency and safety of this salvage sequence in Ewing sarcoma (ES) lung relapses.
    Methods: All eligible pediatric ES patients (1991-2020) identified in SFCE departments were retrospectively reviewed. Seven patients were (1) diagnosed with a pulmonary relapse, isolated or not, (2) naïve from both HCDT and WLI (3) treated by the salvage sequence of conventional chemotherapy, Bu-Mel-HDCT and WLI. The main endpoint was OS evaluation. WLI toxicities were scored using CTC-V5.
    Results: With a 13 years median follow-up (FU), 5/7 patients are alive and in complete remission. 10y-EFS is 71.4%. Three patients experienced transitory radio-induced pneumopathy (RIP). A patient developed RIP (gr.3) and finally progressive lung fibrosis leading to death.
    Conclusion: This study reports seven ES patients treated for lung metastatic relapses, using an aggressive strategy, with favorable survival long-term results which should be balanced with the risk of lung toxicity.
    Advances in knowledge: The approach of surgery, Bu-Mel HDCT followed by WLI can be discussed in selected ES patients with lung relapse, naive from HDCT or WLI, providing an optimal chemosensitivity. A special vigilance is necessary regarding the incidence rate of lung toxicity which can be mitigated by limiting the radiotherapy dose, and observing optimal timing of radiotherapy after HDCT.
    MeSH term(s) Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Bone Neoplasms/drug therapy ; Bone Neoplasms/radiotherapy ; Child ; Combined Modality Therapy ; Humans ; Lung/pathology ; Lung Neoplasms ; Neoplasm Recurrence, Local/etiology ; Neuroectodermal Tumors, Primitive, Peripheral ; Retrospective Studies ; Sarcoma, Ewing/drug therapy ; Sarcoma, Ewing/radiotherapy
    Language English
    Publishing date 2022-07-12
    Publishing country England
    Document type Journal Article ; Multicenter Study
    ZDB-ID 2982-8
    ISSN 1748-880X ; 0007-1285
    ISSN (online) 1748-880X
    ISSN 0007-1285
    DOI 10.1259/bjr.20220212
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Epiphyseal-sparing biological reconstruction of bone sarcoma in children: Focus on articular function and growth.

    Raux, Sébastien / Ducrot, Coline / Marec-Bérard, Perrine / Corradini, Nadège / Claude, Line / Dijoud, Frédérique / Picard, Cécile / Bouhamama, Amine / Chotel, Franck

    Orthopaedics & traumatology, surgery & research : OTSR

    2022  Volume 109, Issue 3, Page(s) 103540

    Abstract: Introduction: Epiphyseal preservation surgery and biological reconstruction after resection of metaphyseal bone sarcoma in children is a surgical challenge which can only be justified if future joint function is maintained.: Hypothesis: The main ... ...

    Abstract Introduction: Epiphyseal preservation surgery and biological reconstruction after resection of metaphyseal bone sarcoma in children is a surgical challenge which can only be justified if future joint function is maintained.
    Hypothesis: The main hypothesis of this work was that long-term function was maintained. The secondary hypotheses were that local control of the disease and growth restoration were achieved, at the cost of an acceptable number of complications.
    Material and method: This was a retrospective study of 14 children with a median age of 8 years [2-14] at the time of surgery. The tumors (Ewing's sarcoma or osteosarcoma) were mostly situated at the knee (n=9) and hip (n=3). The reconstruction used an induced membrane (n=7) or an allograft (n=7). We studied joint function, mechanisms contributing to loss of growth, surgical complications and survival at the last follow-up.
    Results: At the median follow-up of 76 months [24-130], 9 out of 14 patients required revision for non-union, and 4 of them required a second revision. At the last follow-up, 82% of the length had been restored, due to 3 bone lengthenings and 7 contralateral epiphysiodeses. Preserved joint function was excellent with an average modified MSTS score of 28.3/30 [24-30]. No local recurrence was reported.
    Discussion: Our experience of epiphyseal preservation allows local control of the disease and very good function but at the cost of a cumbersome surgical program (12 out of 14 patients were reoperated on, with an average of 1.2 interventions per patient). The main difficulty is the growth management, most often by complex programs of alternating bone lengthening and shortening.
    Level of evidence: IV, retrospective study.
    MeSH term(s) Humans ; Child ; Retrospective Studies ; Bone Neoplasms/surgery ; Osteosarcoma/surgery ; Sarcoma, Ewing/surgery ; Plastic Surgery Procedures ; Treatment Outcome ; Bone Transplantation
    Language English
    Publishing date 2022-12-30
    Publishing country France
    Document type Journal Article
    ISSN 1877-0568
    ISSN (online) 1877-0568
    DOI 10.1016/j.otsr.2022.103540
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Added value of whole-exome and RNA sequencing in advanced and refractory cancer patients with no molecular-based treatment recommendation based on a 90-gene panel.

    Dufresne, Armelle / Attignon, Valéry / Ferrari, Anthony / Tonon, Laurie / Boyault, Sandrine / Tabone-Eglinger, Séverine / Cassier, Philippe / Trédan, Olivier / Corradini, Nadège / Vinceneux, Armelle / Swalduz, Aurélie / Viari, Alain / Chabaud, Sylvie / Pérol, David / Blay, Jean Yves / Saintigny, Pierre

    Cancer medicine

    2024  Volume 13, Issue 7, Page(s) e7115

    Abstract: Introduction: The objective was to determine the added value of comprehensive molecular profile by whole-exome and RNA sequencing (WES/RNA-Seq) in advanced and refractory cancer patients who had no molecular-based treatment recommendation (MBTR) based ... ...

    Abstract Introduction: The objective was to determine the added value of comprehensive molecular profile by whole-exome and RNA sequencing (WES/RNA-Seq) in advanced and refractory cancer patients who had no molecular-based treatment recommendation (MBTR) based on a more limited targeted gene panel (TGP) plus array-based comparative genomic hybridization (aCGH).
    Materials and methods: In this retrospective analysis, we selected 50 patients previously included in the PROFILER trial (NCT01774409) for which no MBT could be recommended based on a targeted 90-gene panel and aCGH. For each patient, the frozen tumor sample mirroring the FFPE sample used for TGP/aCGH analysis were processed for WES and RNA-Seq. Data from TGP/aCGH were reanalyzed, and together with WES/RNA-Seq, findings were simultaneously discussed at a new molecular tumor board (MTB).
    Results: After exclusion of variants of unknown significance, a total of 167 somatic molecular alterations were identified in 50 patients (median: 3 [1-10]). Out of these 167 relevant molecular alterations, 51 (31%) were common to both TGP/aCGH and WES/RNA-Seq, 19 (11%) were identified by the TGP/aCGH only and 97 (58%) were identified by WES/RNA-Seq only, including two fusion transcripts in two patients. A MBTR was provided in 4/50 (8%) patients using the information from TGP/aCGH versus 9/50 (18%) patients using WES/RNA-Seq findings. Three patients had similar recommendations based on TGP/aCGH and WES/RNA-Seq.
    Conclusions: In advanced and refractory cancer patients in whom no MBTR was recommended from TGP/aCGH, WES/RNA-Seq allowed to identify more alterations which may in turn, in a limited fraction of patients, lead to new MBTR.
    MeSH term(s) Humans ; Comparative Genomic Hybridization ; Exome ; Neoplasms/drug therapy ; Neoplasms/genetics ; Neoplasms/pathology ; Retrospective Studies ; RNA ; Sequence Analysis, RNA ; Clinical Trials as Topic
    Chemical Substances RNA (63231-63-0)
    Language English
    Publishing date 2024-03-30
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2659751-2
    ISSN 2045-7634 ; 2045-7634
    ISSN (online) 2045-7634
    ISSN 2045-7634
    DOI 10.1002/cam4.7115
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  8. Article ; Online: Oral vinorelbine in young patients with desmoid-type fibromatosis.

    Kornreich, Laure / Orbach, Daniel / Nicolas, Nayla / Brisse, Hervé J / Berlanga, Pablo / Defachelles, Anne-Sophie / Mansuy, Ludovic / Verite, Cécile / Saumet, Laure / Karanian, Marie / Corradini, Nadège

    Tumori

    2023  Volume 109, Issue 5, Page(s) 511–518

    Abstract: Background: Desmoid-type fibromatosis are rare intermediate tumors in children and adolescents. Owing to local aggressiveness and relapse, systemic treatment for symptomatic advanced or progressive forms is recommended. Following promising results in ... ...

    Abstract Background: Desmoid-type fibromatosis are rare intermediate tumors in children and adolescents. Owing to local aggressiveness and relapse, systemic treatment for symptomatic advanced or progressive forms is recommended. Following promising results in adult patients, oral vinorelbine is investigated in young patients.
    Methods: A retrospective review of young patients (<25 years old) with advanced or progressive desmoid type fibromatosis treated with oral vinorelbine in eight large centers of the Société Française des Cancers de l'Enfant was performed. In addition to tumor assessment according to RECIST 1.1, pre-treatment and during-treatment imagery were reviewed centrally to assess tumor volume and estimate fibrosis score through the change in percentage in hypoT2 signal intensity.
    Results: From 2005 to 2020, 24 patients (median age 13.9 years [range, 1.0-23.0]) received oral vinorelbine. Median number of prior systemic lines of treatment was 1 (range, 0-2), mainly based on intravenous low dose methotrexate and vinblastine. Before vinorelbine initiation, all patients had a progressive disease: radiological for 19, radiological and clinical (pain) for three and only clinical for two. Oral vinorelbine was delivered for a median duration of 12 months (range, 1-42). The toxicity profile was favorable, with no grade 3-4 event. Overall response estimated on 23 evaluable patients according to RECIST 1.1 criteria was three partial responses (13%), 18 stabilization (78%) and two progressive disease (9%). Overall progression-free survival was 89.3% (95% confidential intervals 75.2-100) at 24 months. Four stable tumors according to standard RECIST criteria displayed a partial response with > 65% tumor volume reduction. Among 21 informative patients, the estimated fibrosis score decreased for 15 patients, was stable for four patients and increased for two patients.
    Conclusion: Oral vinorelbine seems to be effective to control advanced or progressive desmoid type fibromatosis in young patients, with a well-tolerated profile. These results support testing this drug as first-line alone or in combination to improve response rate while preserving quality of life.
    MeSH term(s) Adult ; Child ; Adolescent ; Humans ; Vinorelbine ; Fibromatosis, Aggressive/drug therapy ; Quality of Life ; Antineoplastic Combined Chemotherapy Protocols/adverse effects ; Neoplasm Recurrence, Local/drug therapy ; Vinblastine/therapeutic use ; Fibrosis ; Treatment Outcome
    Chemical Substances Vinorelbine (Q6C979R91Y) ; Vinblastine (5V9KLZ54CY)
    Language English
    Publishing date 2023-04-28
    Publishing country United States
    Document type Journal Article
    ZDB-ID 280962-x
    ISSN 2038-2529 ; 0300-8916
    ISSN (online) 2038-2529
    ISSN 0300-8916
    DOI 10.1177/03008916231169806
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  9. Article ; Online: Prognostic Factors for Local Recurrence after Cryoablation of Desmoid Tumors.

    Bouhamama, Amine / Wdowik, Quentin / Grillet, Franck / Brahmi, Mehdi / Sunyach, Marie Pierre / Vaz, Gualter / Meeus, Pierre / Gouin, François / Corradini, Nadege / Dufresne, Armelle / Chabaud, Sylvie / Blay, Jean-Yves / Pilleul, Frank

    Journal of vascular and interventional radiology : JVIR

    2023  Volume 34, Issue 9, Page(s) 1538–1546

    Abstract: Purpose: To determine the risk factors for local of adult patients treated for desmoid tumors by cryoablation.: Materials and methods: Eighty-four patients treated for nonabdominopelvic desmoid tumors by cryoablation from July 2012 to July 2020 were ... ...

    Abstract Purpose: To determine the risk factors for local of adult patients treated for desmoid tumors by cryoablation.
    Materials and methods: Eighty-four patients treated for nonabdominopelvic desmoid tumors by cryoablation from July 2012 to July 2020 were included in a retrospective study. The population was composed of 64 women (76.19%) and 20 men (23.81%), aged from 16 to 75 years (median, 35 years ± 14.25). Each patient underwent preprocedural gadolinium-enhanced magnetic resonance imaging and was followed up to 36 months with the same technique. Clinical features, such as tumor size and previous treatment, epidemiological features, and the technical parameters of cryoablation, were studied.
    Results: Local relapse was found in 19 (22.62%) of 84 patients. The 12-, 24-, and 36-month progression-free survival rates were 89% (95% confidence interval [CI], 79-94), 74% (95% CI, 60-83), and 68% (95% CI, 53-79), respectively. In univariate analysis, significant prognostic factors associated with local recurrence were non-abdominal wall location (P = .042), debulking strategy (P = .0105), risk of visceral injury (P = .034) or peripheral nerve injury during cryoablation (P = .033), previous radiation therapy (P = .043), and treatment before 2016 (P = .008). In multivariate analysis, abdominal wall tumors displayed the best outcome, whereas the neck and trunk showed a high rate of recurrence (hazard ratio, 7.307 [95% CI, 1.396-38.261]).
    Conclusions: The local recurrence of desmoid tumors after cryoablation depends on a number of prognostic factors, in particular, a non-abdominal wall location of the tumor and previous local treatment such as surgery or radiation therapy.
    MeSH term(s) Adult ; Male ; Humans ; Female ; Fibromatosis, Aggressive/diagnostic imaging ; Fibromatosis, Aggressive/surgery ; Fibromatosis, Aggressive/pathology ; Retrospective Studies ; Prognosis ; Cryosurgery/adverse effects ; Cryosurgery/methods ; Neoplasm Recurrence, Local/surgery ; Treatment Outcome
    Language English
    Publishing date 2023-05-12
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1137756-2
    ISSN 1535-7732 ; 1051-0443
    ISSN (online) 1535-7732
    ISSN 1051-0443
    DOI 10.1016/j.jvir.2023.05.012
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  10. Article ; Online: Clinical characteristics and outcomes for children, adolescents and young adults with "CIC-fused" or "BCOR-rearranged" soft tissue sarcomas: A multi-institutional European retrospective analysis.

    Sparber-Sauer, Monika / Corradini, Nadège / Affinita, Maria Carmen / Milano, Giuseppe Maria / Pierron, Gaelle / Carton, Matthieu / Tirode, Franck / Pissaloux, Daniel / Alaggio, Rita / Vokuhl, Christian / Bisogno, Gianni / Berlanga, Pablo / Ferrari, Andrea / Orbach, Daniel

    Cancer medicine

    2023  Volume 12, Issue 13, Page(s) 14346–14359

    Abstract: Background: In certain rare undifferentiated small round cell sarcomas new specific molecular CIC-DUX4/other partner, BCOR-CCNB3/other partner, YWHAE fusions, or BCOR-ITD (internal tandem duplication) were identified. These new "CIC fused" (CIC-fused/ ... ...

    Abstract Background: In certain rare undifferentiated small round cell sarcomas new specific molecular CIC-DUX4/other partner, BCOR-CCNB3/other partner, YWHAE fusions, or BCOR-ITD (internal tandem duplication) were identified. These new "CIC fused" (CIC-fused/ATXN1::NUTM1) and "BCOR rearranged" (BCOR fused/ITD/ YWHAE) soft tissue sarcomas (STS) are not well described.
    Methods: Multi-institutional European retrospective analysis of young patients (0-24 years) with CIC-fused and BCOR rearranged STS.
    Results: Overall, out of the 60 patients selected, the fusion status was CIC-fused (n = 29), ATXN1::NUTM1 (n = 2), BCOR::CCNB3 (n = 18), BCOR-ITD (n = 7), and YWHAE (n = 3), MAML::BCOR STS (n = 1). The main primaries were abdomen-pelvic (n = 23) and limbs (n = 18). Median age was 14 years (0.9-23.8) and 0.9 (0.1-19.1) for CIC-fused and BCOR-rearranged groups, respectively (n = 29; p < 0.001). IRS stages were I (n = 3), II (n = 7), III (n = 35), and IV (n = 15). Overall, 42 patients had large tumors (>5 cm) but only six had lymph node involvement. Patients received mainly chemotherapy (n = 57), local surgery (n = 50), and/or radiotherapy (n = 34). After a median follow-up of 47.1 months (range, 3.4-230), 33 (52%) patients had an event and 23 patients died. Three-year event-free survivals were 44.0% (95% CI 28.7-67.5) and 41.2% (95% CI 25.4-67.0) for CIC and BCOR groups (p = 0.97), respectively. Three-year overall survivals were 46.3% (95% CI 29.6-72.4) and 67.1% (95% CI 50.4-89.3; p = 0.24), respectively.
    Conclusions: Pediatric patients often present with large tumors and metastatic disease, especially CIC sarcomas. Overall outcome is dismal. New treatment options are needed.
    MeSH term(s) Humans ; Child ; Adolescent ; Young Adult ; Repressor Proteins/genetics ; Retrospective Studies ; Proto-Oncogene Proteins/genetics ; Proto-Oncogene Proteins/analysis ; Transcription Factors ; Sarcoma/genetics ; Sarcoma/therapy ; Soft Tissue Neoplasms ; Biomarkers, Tumor/analysis ; Oncogene Proteins, Fusion
    Chemical Substances Repressor Proteins ; Proto-Oncogene Proteins ; Transcription Factors ; Biomarkers, Tumor ; Oncogene Proteins, Fusion ; BCOR protein, human
    Language English
    Publishing date 2023-05-22
    Publishing country United States
    Document type Multicenter Study ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2659751-2
    ISSN 2045-7634 ; 2045-7634
    ISSN (online) 2045-7634
    ISSN 2045-7634
    DOI 10.1002/cam4.6113
    Database MEDical Literature Analysis and Retrieval System OnLINE

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