Inherited Pancreatic Cancer Syndromes and High-Risk Screening

Abstract

Pancreatic cancer is the third leading cause of cancer death in the United States, with a 5-year survival rate of 9%. Individuals with inherited pancreatic cancer syndromes are at an increased risk for developing pancreatic cancer and may benefit from pancreatic cancer surveillance with the goal to detect and intervene on early-stage cancer or high-risk precursor lesions. Given the screening implications for family members and therapeutic implications for probands, all patients diagnosed with pancreatic cancer are recommended to undergo germline genetic testing.

Keywords: Cancer risk; Hereditary; Pancreatic cyst; Surveillance.

Figure 1:. Approach to surveillance intervals for high-risk pancreatic cancer screening (per CAPS Consortium guidelines)

* For concerning features (including findings listed in the 6 month follow-up and 3 month follow-up boxes), fine needle aspiration and/or CT imaging should be considered to better characterize the lesion and assess risk for malignancy. In addition to MRI and EUS findings, patient specific risk factors (including clinical factors and family history) may also be incorporated into risk assessment and lead to earlier tissue sampling or more frequent follow-up per provider discretion. ** Worrisome features of cystic lesions include: mural nodule, enhanced solid component, thickened or enhanced cyst walls, abrupt main pancreatic duct caliber change or size ≥ 10mm, or patient symptoms (pancreatitis, jaundice, pain)