A1 Journal article (refereed)
Clinical characteristics of pancreatic and biliary tract cancers in Lynch syndrome : A retrospective analysis from the Finnish National Lynch Syndrome Research Registry (2023)
Zalevskaja, K., Mecklin, J.-P., & Seppälä, T. T. (2023). Clinical characteristics of pancreatic and biliary tract cancers in Lynch syndrome : A retrospective analysis from the Finnish National Lynch Syndrome Research Registry. Frontiers in Oncology, 13, Article 1123901. https://doi.org/10.3389/fonc.2023.1123901
JYU authors or editors
Publication details
All authors or editors: Zalevskaja, Kristina; Mecklin, Jukka-Pekka; Seppälä, Toni T.
Journal or series: Frontiers in Oncology
eISSN: 2234-943X
Publication year: 2023
Publication date: 01/02/2023
Volume: 13
Article number: 1123901
Publisher: Frontiers Media SA
Publication country: Switzerland
Publication language: English
DOI: https://doi.org/10.3389/fonc.2023.1123901
Publication open access: Openly available
Publication channel open access: Open Access channel
Publication is parallel published (JYX): https://jyx.jyu.fi/handle/123456789/85372
Abstract
Methods: In this retrospective study of the Lynch Syndrome Registry of Finland (LSRFi), records of genetically verified LS patients diagnosed with PC or BTC between 1982 and 2020 were analyzed.
Results: Thirty-nine patients were included: tumor(s) were in the pancreas in 26 patients, in the biliary tract in 10, and in the ampulla of Vater in three. A pathogenic germline variant was found in MLH1 in 33 of 39 patients. Twenty-six patients with 28 tumors located in the pancreas were identified: 23 pancreatic ductal adenocarcinomas (PDACs) and five neuroendocrine tumors (NETs). The median age at diagnosis of PC was 64 years (range of 38–81). In PC, the 5-year overall survival (OS) rate was 20%, and in PDAC, it was 13.6%. Ten patients with BTC were diagnosed: two intrahepatic, five perihilar, two distal extrahepatic cholangiocarcinomas, and one gallbladder carcinoma. Eight patients were male, and the median age at diagnosis was 54 years (range of 34–82). The 5-year OS rate for BTC was 30%. Metachronous tumors were diagnosed in 28 patients (70%). Colorectal cancer was the most common metachronous tumor, diagnosed in 20 patients (51%), and diagnosed prior to PC or BTC in all cases. Curative surgery was attempted on 17 of 39 patients. For 30 patients (91%), the cause of death was PC or BTC; two patients died from another LS-associated cancer, and one died from a stroke.
Conclusion: Although the survival of LS patients with PC or BTC is better than in sporadic cancers, it is still poor and may be reflected by the relatively higher surgical resectability accounted for by the earlier age of onset. More studies on analyses of the molecular and immune profile, screening, and management of LS-associated pancreaticobiliary cancers are warranted.
Keywords: Lynch syndrome; cancer of the large intestine; pancreatic cancer; microsatellites
Free keywords: Lynch syndrome; hereditary nonpolyposis colon cancer; pancreatic cancer; biliary tract cancer; microsatellite instability
Contributing organizations
Ministry reporting: Yes
VIRTA submission year: 2023
JUFO rating: 1