Progression of a persisting mesenchymal hamartoma to intrahepatic cholangiocarcinoma 24 years after the initial diagnosis: A case report

Mesenchymal hamartoma of the liver (MHL) is a rare benign tumor that often presents in early childhood, and it rarely occurs in adulthood. Aberrant development of the portal tract is a known cause of MHL. Although limited information is available on the natural course of MHL, malignant transformation has been reported in a few cases. Here, we report a case of a 26-year-old female with intrahepatic cholangiocarcinoma secondary to unresected MHL. The patient underwent resection of the hepatic mass, which was diagnosed as MHL at 2 years of age, due to an increase in mass size and a suspicion of malignant transformation during work-up. Histopathology confirmed intrahepatic adenosquamous carcinoma in the background of MHL, with a T2N0M0 pathological stage (stage II). The surgical margin was free from tumor cells. The patient fully recovered postoperatively and started receiving adjuvant chemotherapy. Previous case reports have only reported about the development of undifferentiated embryonal sarcoma or angiosarcoma as malignant transformation of MHL. Cases of other malignancies have not been published; however, it is difficult to rule out the occurrence of various malignancies related to the portal tract when considering the pathogenesis of the disease. To the best of our knowledge, this is the first case report of adenocarcinoma of bile duct origin secondary to MHL. This case report suggests that aggressive surgical management should be considered after the initial diagnosis of MHL.