An undifferentiated carcinoma at Klatskin-position with long-term complete remission after chemotherapy

Abstract

Background: Neoplasms anatomically adjacent to the bile duct usually derive from malignantly transformed cholangiocytes forming cholangiocarcinoma (CCA). CCAs are divided in extrahepatic (eCCA) and intrahepatic (iCCA) tumors. Patients with irresectable CCAs are treated with systemic chemotherapy and have an unfavorable prognosis with a median survival of about one year. Here, we report a case of an undifferentiated carcinoma in Klatskin-position with long-term remission after systemic chemotherapy.

Case presentation: A 65-year-old Caucasian male presented with painless jaundice caused by an undifferentiated carcinoma in Klatskin-position (Type IIIb). Alpha fetoprotein (AFP; 3675 IU/mL) and carbohydrate antigen 19-9 (CA 19-9; 183 U/ml) were elevated. An exploratory laparotomy was carried out, but the patient was found to be irresectable due to severe fibrosis caused by biliary obstruction. Histology showed an undifferentiated carcinoma with high proliferation rate, and the patient was therefore subjected to poly-chemotherapy treatment according to the FOLFOX6-protocol. During therapy, AFP decreased to normal. Subsequent CT scans and ERC revealed a complete remission. Four years past initial diagnosis, a new suspicious lesion in the liver is visible on MRT; however, AFP and CA 19-9 are still in the normal range.

Conclusions: Our case demonstrates that histopathological defined diagnosis may significantly inform therapeutic decision-making in irresectable cholangiocarcinoma even in regard to conventional systemic therapy. In case of an undifferentiated carcinoma poly-chemotherapy may provide significant success.

Keywords: Klatskin tumor; chemotherapy; cholangiocarcinoma; endoscopic retrograde cholangiography; undifferentiated carcinoma.

Figure 1. Response to therapy in MRI and ERC

(A) ERC before placement of endoprothesis (left) and after successful therapy (right). Red Arrow heads indicate index lesion and relevant stenosis respectively. (B and C) MRI scan before (left) and after (right) 6 cycles of treatment according to FOLFOX6 regime. T1-weighted, Enlarged inset shows intraluminal tumor in the bile duct (C left).

Figure 2. Course of bilirubin and AFP during treatment

Shown are bilirubin and alpha fetoprotein (AFP) course from first referral. Bilirubin declined after ERC therapy, AFP declined to normal during therapy indicating a complete and sustained remission.

Figure 3. Immunohistochemistry of an undifferentiated carcinoma in Klatskin-position

All stainings were performed on a biopsy taken during ERC after paraffin embedding. (A) Hematoxylin and Eosin (H&E), (B) pan-cytokeratin marker AE1/3 (C) CK20 staining. Representative images are shown.