J.Jpn. Surg. Soc.. 119(2): 180-185, 2018

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Feature topic

GENETIC ANALYSIS FOR SURGERY IN THE HEPATOBILIARY-PANCREATIC FIELD

Familial pancreatic cancer patients are defined as those with more than one pair of first-degree relatives diagnosed with pancreatic cancer. Although recent technical advances in genome sequencing have allowed analysis of numerous mutations in the human genome, most of the genetic mutations in familial pancreatic cancer patients are still unknown. In Japan, the Familial Pancreatic Cancer Registration System organized by the Japan Pancreatic Society began in 2013, and data have been accumulating. In pancreatic neuroendocrine tumors (NETs), the familial and hereditary types are in almost the same category due to high permeability, and it is becoming clear that treatment requires surgical strategies different from those used for the treatment of sporadic-type NETs. In some genetic diseases, liver transplantation is appropriate as it is the only surgical treatment option, among which biliary atresia accounts for 50％ of liver transplantations in Japan. Liver transplantation is also performed in Alagille syndrome, Byler syndrome, Wilson disease, Crigler-Najjar syndrome, and urea cycle disorder, which occur due to a single gene mutation, and satisfactory results have been reported. The prognosis of liver transplantation depends on the presence of comorbidities, and appropriate assessment is important.

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