Table 3.
Who? | ||
► All patients with Peutz-Jeghers syndrome (carriers of a germline LKB1/STK11 gene mutation) ► All carriers of a germline CDKN2A mutation ► Carriers of a germline BRCA2, BRCA1, PALB2, ATM, MLH1, MSH2, or MSH6 gene mutation with at least one affected first-degree blood relative ► Individuals who have at least one first-degree relative with pancreatic cancer who in turn also has a first-degree relative with pancreatic cancer (familial pancreatic cancer kindred) | ||
When (at what age)? | ||
► Age to initiate surveillance depends on an individual's gene mutation status and family history | ||
Familial pancreatic cancer kindred (without a known germline mutation) | Start at age 50 or 55* or 10 years younger than the youngest affected blood relative | |
Mutation carriers: For CDKN2A†, Peutz-Jegher syndrome, start at age 40; BRCA2,ATM, PALB2 BRCA1, MLH1/MSH2 start at age 45 or 50 or 10 years younger than youngest affected blood relative | ||
► There is no consensus on the age to end surveillance | ||
How? | ||
At baseline | ► MRI/MRCP+EUS + fasting blood glucose and/or HbAlc | |
During follow-up | ► Alternate MRI/MRCP and EUS (no consensus if and how to alternate) ► Routinely test fasting blood glucose and/or HbA1c |
|
On indication | ► Serum CA 19–9 | ► If concerning features on imaging |
► EUS-FNA only for | ► Solid lesions of ≥5 mm ► Cystic lesions with worrisome features ► Asymptomatic MPD strictures (with or without mass) |
|
► CT only | ► Solid lesions, regardless o ► Asymptomatic MPD strictures of unknown aetiology (without mass) |
|
Intervals and surgery | ||
12 Months | ► If no abnormalities, or only non-concerning abnormalities (eg, pancreatic cysts without worrisome features) | |
3 or 6 Months | ► If concerning abnormalities for which immediate surgery is not indicated (see figure 2 for details) | |
Surgery | ► If positive FNA and/or a high suspicion of malignancy on imaging (see figure 2 for details) | |
► When surgery is indicated, perform an oncological radical resection at a specialty centre | ||
Goals | ||
The goal of surveillance is to detect and treat the following pathological lesions | ► Stage I pancreatic cancer, confined to the pancreas, resected with negative margins ► Pancreatic cancer precursor lesions with high-grade dysplasia (PanIN or IPMN) |
Consensus as to when to start surveillance was not reached.
Literature-based recommendation.
ATM, ataxia telangiectasia mutated; BRCA2, breast cancer 2; CDKN2A, cyclin-dependent kinase inhibitor 2A; CT, computed tomography; EUS, endoscopic ultrasound; FNA, fine-needle aspiration; HbA1c, hemoglobin A1c; IPMN, intraductal papillary mucinous neoplasm; MLH1, mutL homolog 1; MPD, main pancreatic duct; MRI/MRCP, magnetic resonance imaging/magnetic retrograde cholangiopancreatography; MSH2, mutS homolog 2; MSH6, mutS homolog 6; PALB2, partner and localizer of BRCA2; PanIN, pancreatic intraepithelial neoplasia; STK11, serine/ threonine kinase 11.