A1 Alkuperäisartikkeli tieteellisessä aikakauslehdessä
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-tekijät tai -toimittajat


Julkaisun tiedot

Julkaisun kaikki tekijät tai toimittajat: Zalevskaja, Kristina; Mecklin, Jukka-Pekka; Seppälä, Toni T.

Lehti tai sarja: Frontiers in Oncology

eISSN: 2234-943X

Julkaisuvuosi: 2023

Ilmestymispäivä: 01.02.2023

Volyymi: 13

Artikkelinumero: 1123901

Kustantaja: Frontiers Media SA

Julkaisumaa: Sveitsi

Julkaisun kieli: englanti

DOI: https://doi.org/10.3389/fonc.2023.1123901

Julkaisun avoin saatavuus: Avoimesti saatavilla

Julkaisukanavan avoin saatavuus: Kokonaan avoin julkaisukanava

Julkaisu on rinnakkaistallennettu (JYX): https://jyx.jyu.fi/handle/123456789/85372


Tiivistelmä

Introduction: Patients with Lynch syndrome (LS) have an increased lifetime risk of pancreatic cancer (PC) and biliary tract cancer (BTC). These cancers have a notoriously pessimistic prognosis due to late diagnosis and limited therapeutic options. There are limited data based on small cohorts reviewing PC and BTC in LS patients.

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.


YSO-asiasanat: Lynchin oireyhtymä; paksusuolisyöpä; haimasyöpä; mikrosatelliitit

Vapaat asiasanat: Lynch syndrome; hereditary nonpolyposis colon cancer; pancreatic cancer; biliary tract cancer; microsatellite instability


Liittyvät organisaatiot


OKM-raportointi: Kyllä

VIRTA-lähetysvuosi: 2023

JUFO-taso: 1


Viimeisin päivitys 2024-12-10 klo 15:45