A Cystic Renal Mass in the Setting of a Pneumothorax: More Than Meets the Eye?

Urology. 2021 Aug;154:275-277. doi: 10.1016/j.urology.2021.01.049. Epub 2021 Feb 9.


DICER1 syndrome is a rare hereditary cancer predisposition syndrome that has relevance to pediatric urology providers due to its association with many various pediatric genitourinary malignancies. We describe the case of a pediatric patient who was eventually diagnosed with a pathogenic DICER1 germline variant after undergoing resection of a cystic nephroma and pleuropulmonary blastoma.