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The following is a summary of “Clinical features, treatment and prognosis of primary pulmonary rhabdomyosarcoma: A systemic review,” published in the March 2025 issue of BMC Pediatrics by Tao et al. 

Researchers conducted a retrospective study to summarize clinical characteristics, pathology, treatment, and outcomes of primary pulmonary rhabdomyosarcoma (RMS) and analyze prognostic risk factors.  

They searched 5 databases (MEDLINE, Scopus, the Cochrane Central Register of Controlled Trials, EMBASE, and Web of Science) using terms such as “pulmonary,” “lung,” “alveolar,” “ERMS,” “ARMS,” “RMS”. Cases were included if they had a confirmed pathological diagnosis of RMS, complete treatment details, a follow-up duration of at least 3 months, and comprehensive follow-up records. The primary endpoints analyzed were cancer-specific survival (CSS) and progression-free survival (PFS).  

The results showed that 22 articles (1955–2023) met the inclusion criteria. The median age was 10.5 years (yrs) (2.0, 52.5). Respiratory symptoms due to pulmonary masses were present in 22 cases (78.6%) at diagnosis. The embryonal type was identified in 10 cases, and surgical treatment was performed in 20 cases (71.4%). The average PFS was 60.9 ± 14.8 months. Progression risk was higher in those at TNM stage IV, while CSS was linked to age ≥ 18 years, tumor size ≥ 10 cm, and non-surgical treatment. Surgery significantly reduced progression risk (Hazard ratio (HR)= 4.58 [1.32–15.90], P = 0.017) and improved tumor-related survival (HR = 8.11 [1.45–45.50], P = 0.017).  

Investigators concluded that surgery unaided improved CSS rates, while tumor size and patient age was related with increased mortality. 

Source: bmcpediatr.biomedcentral.com/articles/10.1186/s12887-025-05521-y