Immunohistochemical Examination of a Resected Advanced Hilar Cholangiocarcinoma Arising in a 29-Year-Old Male without Primary Sclerosing Cholangitis

Abstract

A 29-year-old man with advanced hilar cholangiocarcinoma was successfully treated with an extended right lobectomy. The carbohydrate antigen 19-9 (CA19-9) level was elevated to 939 IU/l, and the pathological findings revealed moderately differentiated tubular adenocarcinoma which involved almost the entire thickness of the hepatic duct and the adjacent liver tissue (T3) and which was associated with lymph node metastasis (N1). It was a stage IIB (T3N1M0) tubular adenocarcinoma according to UICC pathological staging. Immunohistochemical examination revealed that Ki-67, cyclin D1, and MMP-7 were positive, and 14-3-3sigma and p27 were negative. The pathological and immunohistochemical findings indicated high malignant potential indicating poor prognosis. We administrated the postoperative adjunct gemcitabine combined with S-1 chemotherapy. The patient is alive without recurrence and doing well two years after surgery. We also review other reports of cholangiocarcinoma patients aged less than 30 years.

Fig. 1

Imaging and operative findings. a A computed tomography scan demonstrated hypovascular masses (arrows) at the hilum and slight dilation of the intrahepatic bile duct of the right lobe. b An ERCP showed complete obstruction of the right hepatic duct and narrowing of the common hepatic duct (arrow). c A FDG positron emission tomographic scan; the mass showed high FDG uptake with a maximal standardized uptake value of 5.8. d Operative findings. The tumor invasion into the right branch of the portal vein (arrows). e A resected specimen. The tumor was 2.5 cm in diameter and involved the right hepatic duct (HD) and right portal vein (PV).

Fig. 2

Pathological and immunohistochemical findings of the resected specimen. a Hematoxylin and eosin staining revealing a moderately differentiated tubular adenocarcinoma. b Ki-67 staining of cancer cell nuclei; the labeling index was 24%. c Cyclin D1 staining of cancer cell nuclei; the labeling index was 30.4%. d Dense MMP-7 staining of the cytoplasm of almost all cancer cells.