Hepatic resection for hepatocellular carcinoma in a patient with situs ambiguous with polysplenia: report of a case

We report a case of successful resection of hepatocellular carcinoma (HCC) in a patient with situs ambiguous with polysplenia (PS), a rare congenital anomaly. A 62-year-old Japanese man was admitted to our hospital for investigation and treatment of a hepatic tumor. Imaging studies revealed two HCCs, 2.5 and 0.7 cm in diameter, in the anterior section and segment 3 of the reversed liver, respectively. He also had multiple spleens in the right side of the abdomen, right side of the stomach, and duodenum, as well as azygous continuation of the inferior vena cava (IVC) with absence of the retro-hepatic IVC, a truncated pancreas, rare variation of the hepatic artery, and dextrocardia. We performed anterior sectionectomy of the liver and partial resection of segment 3. We found only two previous reports of HCC in patients with situs ambiguous with PS, but our patient is the first to have undergone anatomical hepatic resection of the reversed liver. Accurate anatomical assessment is essential during surgery on such patients.