Combined Hepatocellular Cholangiocarcinoma: A Case Report and Review of Literature

Abstract

The two commonly seen subtypes of primary liver cancer are hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC). They both have a distinct clinico-pathologic profile. Combined hepatocellular cholangiocarcinoma (cHCC-CC) is a far more uncommon tumor with histologic evidence of both hepatocellular and biliary epithelial differentiation [1, 2]. It accounts for about 1.0–14.2 % of all primary liver cancers with incidence varying in different studies [3, 4]. The reported incidence of cHCC-CC ranges from 1.4 to 6.5 % and varies geographically [5]. In 1949, Allen and Lisa first described this entity in detail and morphologically classified into three subtypes [6]. Since then there have been a few case series and single case reports describing this malignancy. Owing to its rarity, the natural history, clinical characteristics and optimal management of cHCC-CC is not well understood. We report a case of cHCC-CC along with review of literature. Wherever necessary, data from the Surveillance, Epidemiology and End Results (SEER) cancer registry (http://seer.cancer.gov/) was used to supplement the literature review. The results of our review suggests that cHCC-CC is closer to CC than to HCC. Further, surgery appears to be the modality of choice and regional lymph node dissection is recommended, but radiation therapy adds little to the overall survival of these patients.