A Case of Cholangiolocellular Carcinoma Preoperatively Diagnosed With Typical Imaging Findings

Abstract

Introduction:Cholangiolocellular carcinoma (CoCC) is a rare primary liver neoplasm. A recent integrative genomic analysis has revealed that CoCC represents a distinct biliary-derived molecular entity. Several cases of CoCC have been reported so far, but accurate preoperative diagnosis was difficult in most cases.Case presentation:We report a case of 70-year-old woman with CoCC. Preoperative imaging findings revealed several typical signs of CoCC (i.e., thick early ring enhancement in the peripheral area of the tumor and its prolongation, vessel penetration through the tumor, no dilatation of the peripheral bile ducts, and dot-/band-like internal enhancement or a target appearance on contrast-enhanced magnetic resonance imaging). We strongly suspected CoCC from these preoperative imaging findings of the tumor and performed extended left hepatectomy. Pathologic diagnosis was CoCC, and the histologic findings such as peripheral highly cellular areas, central abundant hyalinized/edematous fibrotic stroma, and retained Glisson's sheath structures in the tumor, corresponded closely to each preoperative imaging finding. Immunohistochemical study revealed the tumor cells were positive for cytokeratin 7 and epithelial membrane antigen. The postoperative course was uneventful, and the patient is alive without recurrence for 15 months. The prognosis of CoCC is known to be better than that of cholangiocellular carcinoma, indicating the importance of preoperative differential diagnosis of these tumors.Conclusion:Even though preoperative diagnosis of CoCC is difficult because of its rarity, cautious investigation of preoperative typical imaging findings can possibly lead to accurate diagnosis of CoCC.