Type I pleuropulmonary blastoma incidentally diagnosed in an infant with RSV bronchiolitis.

Abstract

In summary, PPB, although rare, is the most common primary lung malignancy in children. Early diagnosis and treatment is important in preventing disease progression to a higher-grade lesion. Given its association with the DICER1 cancer predisposition syndrome, children with PPB should undergo DICER1 mutation testing so the appropriate malignancy screening protocols can be instituted. Diagnosing PPB based on symptoms and imaging is challenging as children are often asymptomatic or present with non-specific symptoms, and chest CT is not highly sensitive for predicting malignant lesions. Due to the absence of distinguishing clinical or radiographic features, risk for disease progression and association with the DICER1 cancer predisposition syndrome, it is important to consider PPB in the differential diagnosis for children with cystic lung lesions. This article is protected by copyright. All rights reserved.