CTICasesPleuropulmonary Blastoma Type 1r (Regressed) with DICER1 Syndrome
Imaging findings
CT in a 46-year-old man demonstrates multiple large, thin-walled pulmonary cysts along with previously resected cysts evidenced by surgical staples. Abdominal CT demonstrates multiple calcified bilateral renal lesions, representing regressed cystic nephromas.
Key takeaways
Pleuropulmonary blastoma (PPB) Type 1r (regressed) is the benign regressed form of Type 1 PPB, consisting of residual cystic lung disease without viable tumor. It is associated with germline DICER1 mutations, a cancer predisposition syndrome also linked to cystic nephroma, thyroid neoplasms, and other tumors. Adult presentation with large pulmonary cysts in the setting of calcified renal lesions from childhood and a resection history should prompt consideration of PPB Type 1r and DICER1 syndrome.
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