Familial pancreatic cancer

  1. Familial pancreatic cancer is an established genetic tumor syndrome, accounting for about 3% of all pancreatic cancers, and is generally considered to be autosomal dominant due to the vertical relationship between its occurrence.  The clinical diagnostic criteria for familial pancreatic cancer have been controversial, and most experts define it as the occurrence of pancreatic cancer with pathological basis in two or more family members in the absence of other malignant hereditary tumors in a family. Familial pancreatic cancer was first reported as a familial aggregation of pancreatic cancer in 1973 by MacDermott et al. In 1991, Ghadirian et al. in collaboration with the International Agency for Research on Cancer found that 7.8% of patients with pancreatic cancer had a family history, which was 13 times higher than that of controls.  A positive family history of pancreatic cancer, like smoking, is one of the high-risk factors for the development of pancreatic cancer, which increases significantly in pancreatic cancer family lines as the number of first-degree relatives with pancreatic cancer increases, for example, the risk of developing pancreatic cancer in one first-degree relative is 4.6 times that of the general population, and the risk of developing pancreatic cancer in two first-degree relatives will be 6.4 times that of the general population, while if three or more If three or more first-degree relatives have pancreatic cancer, the risk of developing the disease will be 32 times higher.  In addition, patients with a positive family history of pancreatic cancer in first-degree relatives have an increased risk of developing malignancies at other sites (such as ovarian, breast, lymphoma and colon cancer). In addition, familial pancreatic cancer is also inherited early, that is, the younger the generation, the earlier the age of onset, the more severe the symptoms and the worse the prognosis.  2. Family members of familial pancreatic cancer are the high risk group of pancreatic cancer. After a relative is diagnosed with pancreatic cancer, family members must not be too nervous, over and too frequent checkups, and just do regular follow-up monitoring.  For family lines with 2 or more pancreatic cancer patients, you can register a report at the pancreatic surgery side of the oncology hospital, and specialists will work out a reasonable screening measure and time according to the actual situation.  In general, screening should start at the age of about 50 years, but for those who have family members with pancreatic cancer at the age of onset less than 50 years, it should be 10 years below the youngest age of pancreatic cancer onset (for example, if the youngest patient is 45 years old, other members of the family should start screening at about 35 years).  Screening methods include ultrasound gastroscopy (EUS), thin spiral CT, MRI, MRCP, transendoscopic retrograde cholangiopancreatography (ERCP), and the tumor marker CA199. Foreign studies found that 6.8% of relatives of patients with familial pancreatic cancer had pancreatic cancer detected during regular EUS screening, and a proportion of patients with IPMN (intraductal papillary mucinous neoplasm, precancerous lesion) and PanIN (pancreatic intraepithelial neoplasia, precancerous lesion) were also detected.  Therefore, by monitoring people with a high prevalence of pancreatic cancer through regular physical examinations, pancreatic cancer or precancerous lesions can be detected earlier and appropriate interventions can be taken to achieve the best outcome.