Prevalence of lung cysts in adolescents and adults with a germlineDICER1pathogenic/likely pathogenic variant: a report from the National Institutes of Health and International Pleuropulmonary Blastoma/DICER1Registry

Abstract excerpt

Background Pleuropulmonary blastoma (PPB), the hallmark tumour associated withDICER1-related tumour predisposition, is characterised by an age-related progression from a cystic lesion (type I) to a high-grade sarcoma with mixed cystic and solid features (type II) or purely solid lesion (type III). Not all cystic PPBs progress; type Ir (regressed), hypothesised to represent regressed or non-progressed type I PPB,...

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