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Treatment of Progressive or Recurrent Pleuropulmonary Blastoma
Source: Childhood Pleuropulmonary Blastoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 3, 2025 · Captured 2026-09-09.
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A retrospective review included 35 children with Type II or Type III pleuropulmonary blastoma and progressive or recurrent disease who were registered in national and European databases and trials (2000–2018).[1] Patients had a median age of 3.9 years (range, 0.5–17.8 years).
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The median time to progression was 0.58 years (range, 0.02–1.27 years) from diagnosis despite surgery, chemotherapy (n = 9), and radiation therapy (n = 1). All of these patients died.
Patients were diagnosed with recurrent disease at a median age of 4.3 years (range, 1.7–15.1 years) and had a median delay to relapse of 1.03 years (range, 0.03–2.95 years).
Recurrent disease occurred locally (n = 12), in combined sites (locally and metastatic) (n = 1), and in metastatic sites (n = 13), including the central nervous system (n = 11) and unspecified sites (n = 2).
The 5-year event-free survival rate and overall survival (OS) rates for patients with recurrent disease were both 37% (± 19%; 95% confidence interval).
Local therapy (surgery and radiation therapy) had a favorable impact on OS (P = .03 and .02, respectively).
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