Rhabdomyosarcoma with isolated lung metastases: A report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group

Juan C. Vasquez, Leo Y. Luo, Susan M. Hiniker, Daniel S. Rhee, Roshni Dasgupta, Sonja Chen, Brenda J. Weigel, Wei Xue, Rajkumar Venkatramani, Carola A. Arndt

Research output: Contribution to journalArticlepeer-review


Background: To determine outcomes of children with rhabdomyosarcoma (RMS) with isolated lung metastases. Methods: Data were analyzed for 428 patients with metastatic RMS treated on COG protocols. Categorical variables were compared using Chi-square or Fisher's exact tests. Event-free survival (EFS) and overall survival (OS) were estimated using Kaplan–Meier method and compared using the log-rank test. Results: Compared with patients with other metastatic sites (n = 373), patients with lung-only metastases (n = 55) were more likely to be <10 years of age, have embryonal histology (embryonal rhabdomyosarcoma), have N0 disease, and less likely to have primary extremity tumors. Lung-only patients had significantly better survival outcomes than patients with all other sites of metastatic disease (p <.0001) with 5-year EFS of 48.1 versus 18.8% and 5-year OS of 64.1 versus 26.9%. Patients with lung-only metastases, and those with a single extrapulmonary site of metastasis, had better survival compared with patients with two or more sites of metastatic disease (p <.0001). In patients with ERMS and lung-only metastases, there was no significant difference in survival between patients ≥10 years and 1–9 years (5-year EFS: 58.3 vs. 68.2%, 5-year OS: 66.7 vs. 67.7%). Conclusions: With aggressive treatment, patients with ERMS and lung-only metastatic disease have superior EFS and OS compared with patients with other sites of metastatic disease, even when older than 10 years of age. Consideration should be given to including patients ≥10 years with ERMS and lung-only metastases in the same group as those <10 years in future risk stratification algorithms.

Original languageEnglish (US)
Article numbere30293
JournalPediatric Blood and Cancer
Issue number6
StatePublished - Jun 2023

Bibliographical note

Funding Information:
This work was supported by grants from the National Cancer Institute, National Institutes of Health to the Children's Oncology Group (U10CA180886 and U10CA180899) and grants from St. Baldrick's foundation. JCV is funded in part by the Robert Wood Johnson Harold Amos Medical Faculty Development Program and the Fund to Retain Clinical Scientists at Yale, sponsored by the Doris Duke Charitable Foundation award #2015216, and the Yale Center for Clinical Investigation. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

Publisher Copyright:
© 2023 Wiley Periodicals LLC.


  • Children's Oncology Group
  • lung metastases
  • rhabdomyosarcoma

PubMed: MeSH publication types

  • Journal Article
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't


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