• Advances in pediatric surgery
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• v.5 no.1
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• pp.33-38
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• 1999

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1 years (range 7-13 years). Six patients presented with an abdominal mass or pain, and one with weight loss. Tumor size ranged from $8.0{\times}8.0$ cm to $15.0{\times}15.0$ cm. Four tumors were located in the right lobe, two in the left lobe and one in both. One patient died during chemotherapy. Initial complete resection was accomplished in three patients. Two patients underwent complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, and 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferntiated (embryonal) sarcoma of the liver is highly malignant, the combination therapy of surgery, chemotherapy and radiotherapy appears to result in a favorable prognosis.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.26 no.4
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• pp.407-411
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• 2022

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.