Situs ambiguous with polysplenia complicated by renal adenocarcinoma

Two men developed renal adenocarcinoma in association with situs ambiguous off with polysplenia (SAP) (also known as the polysplenia syndrome). Features of their diseases included (1) no normal spleen--just splenuli, (2) interruption of the inferior vena cava with azygos or hemiazygos continuation, (3) bilateral hyparterial bronchi, (4) cardiac malformations, (5) renal adenocarcinomas originating from the kidneys, ipsilateral to the anomalous spleens. The association of renal adenocarcinomas and SAP has not been previously reported, to our knowledge. We suggest that renal adenocarcinoma and SAP may share a common pathogenetic link.