Familial pancreatic cancer

Clin Res Hepatol Gastroenterol. 2023 Mar;47(3):102079. doi: 10.1016/j.clinre.2023.102079. Epub 2023 Jan 18.

Abstract

Pancreatic cancer (PC) carries a poor prognosis with an overall 5-year survival of less than 10%. Early diagnosis, though cumbersome, is essential to allow complete surgical resection. Therefore, primary and secondary prevention are critical to reduce the incidence and to potentially prevent mortality. Given a relatively low lifetime risk of developing PC, identification of high-risk individuals is crucial to allow identification of pre-malignant lesions and small, localized tumors. Although 85-90% of PC cases are sporadic, we could consider risk stratification for the 5-10% of patients with a family history and the 3-5% of cases due to inherited genetic syndromes. These high-risk populations should be considered for screening and surveillance of PC. MRI/MRCP and EUS are the preferred modalities, due to their high sensitivity in lesion detection. Surveillance should be personalized, considering genetics and family history, and assessment of risk factors that may increase cancer risk. Screening programs should be limited to tertiary referral center, with high-volumes and adequate facilities to manage these patients.

Keywords: Familial pancreatic cancer; Hereditary pancreatic cancer; Pancreatic EUS; Pancreatic cancer screening.

Publication types

  • Review

MeSH terms

  • Early Detection of Cancer
  • Genetic Predisposition to Disease*
  • Humans
  • Pancreatic Neoplasms* / diagnosis
  • Risk Factors

Supplementary concepts

  • Pancreatic carcinoma, familial