An undifferentiated carcinoma at Klatskin-position with long-term complete remission after chemotherapy
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Bruno Christian Köhler1,2, Benjamin Goeppert2,3, Nina Waldburger2,3, Kai Schlamp4, Peter Sauer5, Dirk Jäger1,2, Karl Heinz Weiss2,5, Stephan Macher-Göppinger6, Henning Schulze-Bergkamen7, Peter Schirmacher2,3 and Christoph Springfeld1,2
1Department of Medical Oncology, National Center for Tumor Diseases, University Hospital Heidelberg, Heidelberg, Germany
2Liver Cancer Center Heidelberg, University Hospital Heidelberg, Heidelberg, Germany
3Department of Pathology, University of Heidelberg, Heidelberg, Germany
4Department of Neuroradiology, University Hospital Heidelberg, Heidelberg, Germany
5Department of Gastroenterology, University Hospital Heidelberg, Heidelberg, Germany
6Institute of Pathology, University Medical Center Mainz, Mainz, Germany
7Department of Internal Medicine II, Marien-Hospital, Wesel, Germany
Bruno Christian Köhler, email: [email protected]
Keywords: cholangiocarcinoma; Klatskin tumor; undifferentiated carcinoma; chemotherapy; endoscopic retrograde cholangiography
Received: February 09, 2018 Accepted: March 22, 2018 Published: April 24, 2018
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.
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