Metachronous Ampulla of Vater Carcinoma after Curative-Intent Surgery for Klatskin Tumor

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.