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IV期横纹肌肉瘤患儿从长期维持治疗中显著获益：CWS-IV 2002和CWS DOK IV 2004试验结果

Pediatric Patients with Stage IV Rhabdomyosarcoma Significantly Benefit from Long-Term Maintenance Therapy: Results of the CWS-IV 2002 and the CWS DOK IV 2004-Trials.

作者信息

Tramsen Lars, Bochennek Konrad, Sparber-Sauer Monika, Salzmann-Manrique Emilia, Scheer Monika, Dantonello Tobias, Borkhardt Arndt, Dirksen Uta, Thorwarth Anne, Greiner Jeanette, Ebinger Martin, Weclawek-Tompol Jadwiga, Ladenstein Ruth, Ljungman Gustaf, Hallmen Erika, Lehrnbecher Thomas, Koscielniak Ewa, Klingebiel Thomas

机构信息

Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel, 24105 Kiel, Germany.

Department for Children and Adolescents, University Hospital, Goethe-University, 60590 Frankfurt, Germany.

出版信息

Cancers (Basel). 2023 Mar 30;15(7):2050. doi: 10.3390/cancers15072050.

DOI:10.3390/cancers15072050
PMID:37046711
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10093505/
Abstract

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma (STS) in childhood. Whereas more than 90% of patients with localized low-risk RMS can be cured, metastatic RMS have a dismal outcome, with survival rates of less than 30%. The HD CWS-96 trial showed an improved outcome for patients receiving maintenance therapy after completing intensive chemotherapy. Consequently, the international clinical trials CWS-IV 2002 and CWS DOK IV 2004 on metastatic disease of STS of the Cooperative Weichteilsarkom Studiengruppe (CWS) were designed in addition to the CWS-2002P trial for localized RMS disease. All patients received a multimodal intensive treatment regimen. To maintain remission, three options were compared: long-term maintenance therapy (LTMT) versus allogeneic hematopoietic stem cell transplantation (alloHSCT) versus high-dose chemotherapy (HDCT). A total of 176 pediatric patients with a histologically confirmed diagnosis of metastatic RMS or RMS-like tumor were included. A total of 89 patients receiving LTML showed a significantly better outcome, with an event-free survival (EFS) of 41% and an overall survival (OS) of 53%, than alloHSCT ( = 21, EFS 19%, = 0.02, OS 24%, = 0.002). The outcome of LTML was slightly improved compared to HDCT ( = 13, EFS 35%, OS 34%). In conclusion, our data suggest that in patients suffering from metastatic RMS, long-term maintenance therapy is a superior strategy in terms of EFS and OS compared to alloHSCT. EFS and OS of HDCT are similar in these strategies; however, the therapeutic burden of LTMT is much lower.

摘要

横纹肌肉瘤（RMS）是儿童期最常见的软组织肉瘤（STS）。虽然超过90%的局限性低风险RMS患者可以治愈，但转移性RMS的预后很差，生存率低于30%。HD CWS - 96试验表明，完成强化化疗后接受维持治疗的患者预后有所改善。因此，除了针对局限性RMS疾病的CWS - 2002P试验外，合作软组织肉瘤研究组（CWS）还设计了关于STS转移性疾病的国际临床试验CWS - IV 2002和CWS DOK IV 2004。所有患者均接受多模式强化治疗方案。为维持缓解，比较了三种选择：长期维持治疗（LTMT）与异基因造血干细胞移植（alloHSCT）与大剂量化疗（HDCT）。总共纳入了176例经组织学确诊为转移性RMS或RMS样肿瘤的儿科患者。与alloHSCT（n = 21，无事件生存率[EFS]为19%，P = 0.02，总生存率[OS]为24%，P = 0.002）相比，总共89例接受LTML的患者显示出明显更好的预后，EFS为41%，OS为53%。与HDCT（n = 13，EFS 35%，OS 34%）相比，LTML的预后略有改善。总之，我们的数据表明，对于转移性RMS患者，就EFS和OS而言，长期维持治疗是优于alloHSCT的策略。在这些策略中，HDCT的EFS和OS相似；然而，LTMT的治疗负担要低得多。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/8f2110d69a84/cancers-15-02050-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/cfe8638ff483/cancers-15-02050-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/109e9a4df571/cancers-15-02050-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/80464b24bc12/cancers-15-02050-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/ffe6b543875a/cancers-15-02050-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/3ce3e0086f07/cancers-15-02050-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/6f4e5f8763be/cancers-15-02050-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/8f2110d69a84/cancers-15-02050-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/cfe8638ff483/cancers-15-02050-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/109e9a4df571/cancers-15-02050-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/80464b24bc12/cancers-15-02050-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/ffe6b543875a/cancers-15-02050-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/3ce3e0086f07/cancers-15-02050-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/6f4e5f8763be/cancers-15-02050-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/31cf/10093505/8f2110d69a84/cancers-15-02050-g007.jpg

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[2]
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[3]
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[4]
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[5]
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[6]
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本文引用的文献

[1]
Haploidentical hematopoietic stem cell transplantation as individual treatment option in pediatric patients with very high-risk sarcomas.

Front Oncol. 2023-2-21

[2]
Nonmetastatic Rhabdomyosarcoma in Children and Adolescents: Overall Results of the European Pediatric Soft Tissue Sarcoma Study Group RMS2005 Study.

J Clin Oncol. 2023-5-1

[3]
Metastatic Rhabdomyosarcoma: Results of the European Soft Tissue Sarcoma Study Group MTS 2008 Study and Pooled Analysis With the Concurrent BERNIE Study.

J Clin Oncol. 2022-11-10

[4]
No Improvement of Survival for Alveolar Rhabdomyosarcoma Patients After HLA-Matched Versus -Mismatched Allogeneic Hematopoietic Stem Cell Transplantation Compared to Standard-of-Care Therapy.

Front Oncol. 2022-5-10

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Long-Term Clinical Outcome and Prognostic Factors of Children and Adolescents with Localized Rhabdomyosarcoma Treated on the CWS-2002P Protocol.

Cancers (Basel). 2022-2-11

[6]
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Pediatr Blood Cancer. 2022-4

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J Clin Oncol. 2021-9-10

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Pathology of childhood rhabdomyosarcoma: A consensus opinion document from the Children's Oncology Group, European Paediatric Soft Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe.

Pediatr Blood Cancer. 2021-3

[9]
Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre, open-label, randomised, phase 3 trial.

Lancet Oncol. 2019-9-24

[10]
Refinement of risk stratification for childhood rhabdomyosarcoma using FOXO1 fusion status in addition to established clinical outcome predictors: A report from the Children's Oncology Group.

Cancer Med. 2019-8-27

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