Another subset of patients that develop recurrent AP with a subsequently increased risk of pancreatic cancer includes those with hereditary pancreatitis (HP). Patients with HP have a strong family history of AP, and can have attacks of AP at ages as young as ten.5

The inheritance of HP can be either autosomal dominant or recessive; the most common genetic mutations seen are in CFTR, PRSS1, and SPINK1.6 These mutations can lead to premature activation of pancreatic enzymes within the pancreas before they reach the duodenum, leading to auto-digestion of pancreatic parenchyma and recurrent attacks of AP.

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Patients diagnosed with HP have a significantly increased risk of developing pancreatic cancer. The estimated risk varies among studies, though the reported range is between 20% and 40% by age 70.7 This risk substantially increases after 50 years old.

Smoking also doubles the risk of pancreatic cancer among patients with HP and is associated with earlier development.8

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Two sets of guidelines recommend screening for pancreatic cancer in patients with HP, though there is no consensus between the 2 about which is the best age to begin screening.9,10 There is also no consensus about which test to use for screening, though the most common tests are endoscopic ultrasound (EUS) and MRI with magnetic resonance cholangiopancreatography (MRCP). Screening is typically performed every year and can use the same modality or alternate between EUS and MRI/MRCP.

References

  1. Tummala P, Tariq SH, Chibnall JT, Agarwal B. Clinical predictors of pancreatic carcinoma causing acute pancreatitis. Pancreas. 2013;42(1):108-13.
  2. Munigala S, Kanwal F, Xian H, Scherrer JF, Agarwal B. Increased risk of pancreatic adenocarcinoma after acute pancreatitis. Clin Gastroenterol Hepatol. 2014 Jul;12(7):1143-1150.
  3. Sankaran SJ, Xiao AY, Wu LM, Windsor JA, Forsmark CE, Petrov MS. Frequency of progression from acute to chronic pancreatitis and risk factors: a meta-analysis. Gastroenterology. 2015;149(6):1490.
  4. Lowenfels AB, Maisonneuve P, DiMagno EP, et al. Hereditary pancreatitis and the risk of pancreatic cancer: International Hereditary Pancreatitis Study Group. J Natl Cancer Inst. 1997;89(6):442.
  5. Rebours V, Boutron-Ruault MC, Schnee M, et al. The natural history of hereditary pancreatitis: a national series. Gut. 2009;58(1):97.
  6. LaRusch J, Barmada MM, Solomon S, Whitcomb DC. Whole exome sequencing identifies multiple, complex etiologies in an idiopathic hereditary pancreatitis kindred. JOP. 2012;13(3):258.
  7. Howes N, Lerch MM, Greenhalf W, et al. Clinical and genetic characteristics of hereditary pancreatitis in Europe. Clin Gastroenterol Hepatol. 2004;2(3):252.
  8. Lowenfels AB, Maisonneuve P, Whitcomb DC, Lerch MM, DiMagno EP. Cigarette smoking as a risk factor for pancreatic cancer in patients with hereditary pancreatitis. JAMA. 2001;286(2):169.
  9. Ulrich CD; Consensus Committees of the European Registry of Hereditary Pancreatic Diseases, Midwest Multi-Center Pancreatic Study Group, International Association of Pancreatology. Pancreatic cancer in hereditary pancreatitis: consensus guidelines for prevention, screening and treatment. Pancreatology. 2001;1(5):416.
  10. Brand RE, Lerch MM, Rubinstein WS, et al. Advances in counselling and surveillance of patients at risk for pancreatic cancer. Gut. 2007;56(10):1460-9.

Topics:

Endocrine Cancer Pancreatic Cancer