The Five Periampullary Cancers, not Just Different Siblings but Different Families: An International Multicenter Cohort Study

• Published in: Annals of surgical oncology Vol. 31; no. 9; pp. 6157 - 6169
• Main Authors: Uijterwijk, Bas A., Lemmers, Daniël H., Ghidini, Michele, Wilmink, Hanneke, Zaniboni, Alberto, Salvia, Roberto, Kito Fusai, Giuseppe, Groot Koerkamp, Bas, Koek, Sharnice, Ghorbani, Poya, Zerbi, Alessandro, Nappo, Gennaro, Luyer, Misha, Goh, Brian K. P., Roberts, Keith J., Boggi, Ugo, Mavroeidis, Vasileios K., White, Steven, Kazemier, Geert, Björnsson, Bergthor, Serradilla-Martín, Mario, House, Michael G., Alseidi, Adnan, Ielpo, Benedetto, Mazzola, Michele, Jamieson, Nigel, Wellner, Ulrich, Soonawalla, Zahir, Cabús, Santiago Sánchez, Dalla Valle, Raffaele, Pessaux, Patrick, Vladimirov, Miljana, Kent, Tara S., Tang, Chung N., Fisher, William E., Kleeff, Jorg, Mazzotta, Alessandro, Suarez Muñoz, Miguel Angel, Berger, Adam C., Ball, Chad G., Korkolis, Dimitris, Bannone, Elisa, Ferarri, Clarissa, Besselink, Marc G., Abu Hilal, Mohammed
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.09.2024; Springer Nature B.V
• Subjects: Adenocarcinoma; Adenocarcinoma - pathology; Adenocarcinoma - surgery; Aged; Ampulla of Vater - pathology; Ampulla of Vater - surgery; Bile Duct Neoplasms - pathology; Bile Duct Neoplasms - surgery; Carcinoma; Carcinoma, Pancreatic Ductal - pathology; Carcinoma, Pancreatic Ductal - surgery; Cholangiocarcinoma; Cholangiocarcinoma - pathology; Cholangiocarcinoma - surgery; Cohort analysis; Cohort Studies; Common Bile Duct Neoplasms - mortality; Common Bile Duct Neoplasms - pathology; Common Bile Duct Neoplasms - surgery; Duodenal Neoplasms - pathology; Duodenal Neoplasms - surgery; Female; Follow-Up Studies; Humans; Male; Medicin och hälsovetenskap; Medicine; Medicine & Public Health; Middle Aged; Oncology; Pancreatic cancer; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms - surgery; Pancreatic Tumors; Pathology; Prognosis; Retrospective Studies; Surgery; Surgical Oncology; Survival Rate; Tumors
• ISSN: 1068-9265; 1534-4681; 1534-4681
• DOI: 10.1245/s10434-024-15555-8

Background Cancer arising in the periampullary region can be anatomically classified in pancreatic ductal adenocarcinoma (PDAC), distal cholangiocarcinoma (dCCA), duodenal adenocarcinoma (DAC), and ampullary carcinoma. Based on histopathology, ampullary carcinoma is currently subdivided in intestinal (AmpIT), pancreatobiliary (AmpPB), and mixed subtypes. Despite close anatomical resemblance, it is unclear how ampullary subtypes relate to the remaining periampullary cancers in tumor characteristics and behavior. Methods This international cohort study included patients after curative intent resection for periampullary cancer retrieved from 44 centers (from Europe, United States, Asia, Australia, and Canada) between 2010 and 2021. Preoperative CA19-9, pathology outcomes and 8-year overall survival were compared between DAC, AmpIT, AmpPB, dCCA, and PDAC. Results Overall, 3809 patients were analyzed, including 348 DAC, 774 AmpIT, 848 AmpPB, 1,036 dCCA, and 803 PDAC. The highest 8-year overall survival was found in patients with AmpIT and DAC (49.8% and 47.9%), followed by AmpPB (34.9%, P  < 0.001), dCCA (26.4%, P   =  0.020), and finally PDAC (12.9%, P  < 0.001). A better survival was correlated with lower CA19-9 levels but not with tumor size, as DAC lesions showed the largest size. Conclusions Despite close anatomic relations of the five periampullary cancers, this study revealed differences in preoperative blood markers, pathology, and long-term survival. More tumor characteristics are shared between DAC and AmpIT and between AmpPB and dCCA than between the two ampullary subtypes. Instead of using collective definitions for “periampullary cancers” or anatomical classification, this study emphasizes the importance of individual evaluation of each histopathological subtype with the ampullary subtypes as individual entities in future studies.