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

Abstract

BackgroundNeoplasms 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 PresentationA 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.ConclusionsOur 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.

Aggressive behavior of anaplastic undifferentiated carcinoma arising from the hilar bile duct

“…In terms of non-surgical treatment for perihilar undifferentiated carcinoma, two cases reportedly received systemic chemotherapy: adjuvant chemotherapy with gemcitabine and FOLFOX6 for advanced undifferentiated carcinoma at Klatskin-position [ 15 , 29 ]. The former achieved tumor-free survival of 16 months after surgery, while the latter was in complete remission for 4 years.…”

Incidence and prognosis of undifferentiated cancers of the digestive system: a population-based cohort study

“…Owing to the relative rarity of UCDS and the lack of clinical trials, therapeutic guidelines or recommendations are not available (31). Literature reports have suggested a variety of regional and systemic treatment strategies; however, complete response and long-time survival were achieved in only a few cases (32,33). Reis Neto et al studied 287 patients with rectal adenocarcinoma treated with radiotherapy followed by surgery and reported that preoperative radiotherapy decreased the proportion of undifferentiated cells, indicating that undifferentiated rectal cancer cells were more sensitive to the radiation than differentiated cancer cells (34).…”