Situs Inversus Totalis (SIT) with Hepatocellular Carcinoma (HCC): A Rare Case Report and Review of 12 Other Cases

Hepatocellular carcinoma (HCC) is the seventh-most common malignancy in males and ninth in females with incidence of one million new cases every year. Situs inversus totalis (SIT) is a rare congenital condition, in which there is a mirror-image transposition of both the abdominal and thoracic viscera. There are very few reported cases of HCC developing in people with SIT. In this review, we present a new case of HCC with SIT, and a review of literatures published between 1983 and 2011 on it. The literatures in English were searched through PubMed and Google Scholar, while those in Japanese language were accessed through J-EAST and translated in English with the help of Google translator on 22 April 2012. There are 6 English and 6 Japanese literatures showing 12 published cases, of which 10 cases were from Japan, 1 from Taiwan and 1 from China. Our case is probably the first case in the world beyond these regions. The articles containing adequate information, such as patient age and sex, investigations, diagnosis, type of congenital anomalies and methods of surgery, were reviewed. On reviewing the literature, we observed that clinical manifestations, laboratory findings and etiology correlate well with HCC, while anomalous hepatic vascularity correlates well with SIT. The reason for high incidence of HCC with SIT in Japan is not well correlated, but may be explained by higher incidence of SIT. All varieties of hepatic resection were feasible in cases of SIT.