Primary Sarcomas of the Liver

Primary hepatic sarcomas are uncommon, representing less than 1% of the primary malignant lesions of the liver. Three patients underwent resection for primary hepatic sarcoma at the Department of Surgery, Inje University Paik Hospital, Seoul: a 6 year-old girl with malignant mesenchmoma, a 74 year-old man with malignant fibrous histiocytoma and a 53 year-old man with rhabdomyosarcoma. Abdominal mass, fever with chills, and abdominal pain were the presenting symptoms, respectively. The patient with rhabdomyosarcoma was positive for HBsAg and had chronic active hepatitis. AFP level was elevated in this patient. CA 19-9 level was elevated in the patient with malignant fibrous histiocytoma. CEA levels were normal in all cases. All these tumors were hypodense on computed tomography. Malignant mesenchymoma was hypovascular and rhabdomyosarcoma was hypervascular on angiography. Immunohistochemical stains of the tumors were positive for vimentin but negative for epithelial markers, differentiating these lesions from other hepatic tumors. Electron microscopic examination was helpful in the diagnosis of the specific type of primary hepatic sarcoma. None had postoperative adjuvant chemotherapy or radiotherapy. The patient with malignant mesenchymoma, who underwent right hepatic trisegmentectomy, was disease free at 32 months. The patient with malignant fibrous histiocytoma, who underwent non-curative excision, died of the tumor at 2 months. The patient with rhabdomyosarcoma, who underwent posterior segmentectomy, was alive at 4 months. Resection of primary hepatic sarcoma should be executed if feasible, with potential survival measured in years, in view of the lack of other effective treatment modalities.

Peripheral Cholangicoarcinoma

Peripheral cholangiocarcinoma (PCC) is defined as a malignancy of intrahepatic bile duct presenting as a focal liver mass, specifically, those located peripheral to the 2nd-order branchings of the bile duct. During the study period of 11 years from Jan. 1985 through Dec. 1995, a total of 12 patients underwent liver resection for PCC at the Department of Surgery, Inje University Paik Hospital, Seoul. This figure accounts for 8.3% of primary liver cancer. There were 9 men and 3 women with a ratio of 3:1, the mean age was 53.3 years with a range of 39 to 67 years. Twenty-five per cent of the patients had concomitant intrahepatic stone (IHS). HBsAg was positive in one case (8.3%) who had chronic hepatitis. No patient had liver cirrhosis. The accurate preoperative diagnosis of PCC in 3 cases was difficult because of the association with IHS. These 3 patients underwent surgery under the diagnosis of chronic cholangitis rather than PCC. CEA and CA 19-9 were elevated in 70% and 50% of the patients, respectively. In most cases concomitant measurement of CEA and AFP facilitated the differentiation between PCC and hepatocellular carcinoma. As to the tumor characteristics, 83% of the patients had tumors greater than 5 cm in diameter, indicating the advanced stage of the tumor at the time of resection. The site of origin of tumor mass was distributed more or less evenly between the hepatic lobes. The patients with mass-forming, expanding type of tumors made up 58% of the cases, infiltrating type 25%, and the remainder being the combination of both. A significant number of patients, 27%, had nodal metastasis. Two thirds of the patients underwent major hepatic resections. Segmentectomies here include the resection of lateral segment done as a treatment for IHS, which later proved to be cancer on histologic examination. There was no operative death among the 12 patients. The overall median survival time was 12 months. The overall survival rates at 1, 3, and 5 years were 42.4%, 42.4%, and 42.4%, respectively. Only the radicality of the resection was the statistically significant risk factor for survival by univariate analysis. The 5-year survival rate was 100% in 6 patients who had curative resection (no lymph node invasion, no peritoneal seeding, clear resection margin). The prognosis of patients with advanced PCC is unfavorable unless the tumor is entirely removed. In view of the association of IHS in a significant portion of the cases, the possible coexistence of the carcinoma should always be borne in mind when dealing with patients with IHS.