Resection represents the single hope for long-term survival in a patient diagnosed with a
hilar cholangiocarcinoma (
Klatskin tumor). However, the largest part of these patients develops a recurrent disease. Second metachronous periampullary
cancers after a curative-intent surgery for a
Klatskin tumor represent an exceptional pathology, and the management of these patients was poorly documented. Hereby, it is presented a 32-year-old patient with bile duct resection, left hemi-
hepatectomy and loco-regional lymph nodes dissection, for a type IIIB
Bismuth-Corlette
Klatskin tumor, which, furthermore, 6 years later, underwent a
pancreaticoduodenectomy for a metachronous
carcinoma of the ampulla of Vater. The management and outcomes were discussed in the reported case, along with a literature review of the previously published patients. In conclusion, a metachronous periampullary
carcinoma after resection of a
Klatskin tumor should be distinguished from a loco-regional recurrent disease. While most of the patients with recurrences are suitable to only
chemotherapy and or
radiotherapy, a second curative-intent surgery (i.e.,
pancreaticoduodenectomy) is feasible in the largest part of the patients with a metachronous
cancer, with good long-term outcomes.