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Type I and Type Ir
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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Context: Treatment of Childhood Pleuropulmonary Blastoma
Surgery is the treatment of choice for patients with Type I and Type Ir pleuropulmonary blastoma. In the International Pleuropulmonary Blastoma Registry series, the 5-year disease-free survival (DFS) and overall survival (OS) rates were 90% and 98%, respectively, for Type I, and 96% and 100%, respectively, for Type Ir. Approximately 10% of cases progressed to Type II or Type III after surgery. However, adjuvant chemotherapy has been used in almost 40% of patients with Type I disease, and it may be useful for patients at increased risk of recurrence or progression.[3,4,7]
The International Pleuropulmonary Blastoma/DICER1 Registry reported that between 2006 and 2022, there were 205 children who had centrally reviewed Type I or Type Ir pleuropulmonary blastoma. Of these children, 39% with Type I and 5% with Type Ir received chemotherapy.[7]
Source links and citations
Patient outcomes were favorable, although 11 children (9 with Type I and 2 with Type Ir) experienced progression to Type II or III (n = 8) or regrowth of Type I at the surgical site (n = 3). None of these 11 children received chemotherapy before progression.
The combination of age and cyst size was more useful than either factor alone in predicting whether a particular lesion was Type I or Type Ir.
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Preserved source evidence · Not medical advice