Understanding pleuropulmonary blastoma: A SEER-based analysis of clinical characteristics, treatment patterns, and survival outcomes

Pleuropulmonary blastoma (PPB) is a rare and aggressive pediatric malignancy originating from the pleuropulmonary mesenchyme. PPB poses significant clinical challenges due to its variable presentation, rapid progression, and poor prognosis in advanced stages. This study leverages the SEER database to analyze the clinical characteristics, treatment patterns, and survival outcomes of 85 PPB patients diagnosed between 2000 and 2020. The mean age at diagnosis was 3.9 years, with an equal sex distribution. Most cases were localized (54.1%), while 27.1% presented with regional and 12.9% with distant disease. Treatment included surgery (92.9%), chemotherapy (60.0%), and radiation therapy (8.2%). Survival was strongly influenced by age and disease stage. Localized disease showed the best outcomes, while regional (HR 19.135, p = 0.005) and distant stages (HR 27.900, p = 0.002) were associated with significantly worse survival. Multivariate analysis confirmed age and stage as independent prognostic factors. These findings underscore the importance of early detection and timely surgical intervention, which are critical for improved outcomes, especially for localized PPB. Chemotherapy remains central to advanced-stage management, while radiation therapy is sparingly used due to potential long-term toxicities. This analysis represents the first population-level evaluation of PPB using SEER data, providing a comprehensive overview of its epidemiology, survival trends, and prognostic factors. These results emphasize the need for early diagnosis, tailored treatment approaches, and further research to optimize outcomes in this rare but aggressive malignancy.