Annals of Hepato-Biliary-Pancreatic Surgery (한국간담췌외과학회지)

The Korean Association of Hepato-Biliary-Pancreatic Surgery (한국간담췌외과학회)
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Progression of a persisting mesenchymal hamartoma to intrahepatic cholangiocarcinoma 24 years after the initial diagnosis: A case report

  • Sujin Gang (Department of Surgery, Seoul National University Hospital) ;
  • YoungRok Choi (Department of Surgery, Seoul National University Hospital) ;
  • Sola Lee (Department of Surgery, Seoul National University Hospital) ;
  • Su young Hong (Department of Surgery, Seoul National University Hospital) ;
  • Sanggyun Suh (Department of Surgery, Seoul National University Hospital) ;
  • Eui Soo Han (Department of Surgery, Seoul National University Hospital) ;
  • Suk Kyun Hong (Department of Surgery, Seoul National University Hospital) ;
  • Nam-Joon Yi (Department of Surgery, Seoul National University Hospital) ;
  • Kwang-Woong Lee (Department of Surgery, Seoul National University Hospital) ;
  • Kyung-Suk Suh (Department of Surgery, Seoul National University Hospital)
  • Received : 2022.04.21
  • Accepted : 2022.05.18
  • Published : 2022.11.30
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Abstract

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.

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References

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