Intrahepatic Cholangiocarcinoma and Acute Intermittent Porphyria: A Case Report

Patients suffering from different forms of acute hepatic porphyria present a high risk of primary liver cancer, specifically hepatocellular carcinoma and cholangiocarcinoma, determined by the activity of the disease even though an exact mechanism of carcinogenesis has not been recognized yet. Here, we present the clinical case of a 72-year-old woman who, approximately 29 years after the diagnosis of acute intermittent porphyria, presented with intrahepatic cholangiocarcinoma with a histological diagnosis of adenocarcinoma starting from the biliary-pancreatic ducts, which was diagnosed during the clinical and anatomopathological evaluation of a pathological fracture of the femur.

Automated summary

Patients with acute hepatic porphyria are at a high risk of developing primary liver cancer, particularly hepatocellular carcinoma and cholangiocarcinoma, due to the active nature of the disease, although the exact mechanism of carcinogenesis is still unknown. This case report presents the clinical case of a 72-year-old woman who developed intrahepatic cholangiocarcinoma approximately 29 years after being diagnosed with acute intermittent porphyria. The tumor was histologically diagnosed as adenocarcinoma originating from the biliary-pancreatic ducts, and was discovered during the evaluation of a pathological femur fracture.