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Type II and Type III
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
For patients with Type II and Type III pleuropulmonary blastoma, a multimodal sarcoma treatment approach is recommended. This approach usually includes rhabdomyosarcoma regimens and either upfront or delayed surgery.[3,4,8] Anthracycline-containing regimens appear to be the most effective.[4]
In the Pleuropulmonary Blastoma Registry series, the 5-year DFS and OS rates were 59% and 71%, respectively, for Type II, and 37% and 53%, respectively, for Type III.[3] In this group of patients, approximately 50% of relapses occurred in the brain.[3]
The International Pleuropulmonary Blastoma/DICER1 Registry reported the outcomes of children with Type II and Type III pleuropulmonary blastoma whose first treatment was ifosfamide, vincristine, dactinomycin, and doxorubicin (IVADo). From 1987 to 2021, 314 children with centrally confirmed Type II and Type III pleuropulmonary blastoma who received upfront chemotherapy were enrolled, 132 of whom (75 with Type II and 57 with Type III) received IVADo chemotherapy.[9]
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Adjusted analyses suggested improved OS for children treated with IVADo, compared with historical controls, with an estimated hazard ratio (HR) of 0.65 (95% confidence interval [CI], 0.39–1.08).
Compared with localized disease, distant metastasis at diagnosis was associated with worse pleuropulmonary blastoma event-free survival and OS, with HRs of 4.23 (95% CI, 2.42–7.38) and 4.69 (95% CI, 2.50–8.80), respectively.
The role of radiation therapy is not well defined. While the use of radiation did not impact survival in the International Pleuropulmonary Blastoma Registry series, only 20% of patients with Type II and Type III received it.[3]
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Preserved source evidence · Not medical advice