Primary Hepatic Tumors in Children: Clinical Experience in a Single Institution / 대한소아혈액종양학회지

ABSTRACT

PURPOSE: The frequency and clinical characteristics of primary childhood hepatic tumors diagnosed in a single institution were reviewed. Method: From January 1991 to June 2000, 33 patients were diagnosed with primary hepatic tumor. Retrospective analysis of the medical records of the various primary hepatic tumors was performed. RESULTS: Among the total of 33 patients with primary hepatic tumor, twenty five (76%) were malignant and eight (24%) were benign. The mean age of patients with hepatoblastoma (HB) was 32 months (12 of 16 patients were less than 3 years), 12.8 years in hepatocellular carcinoma (HCC) (all of them were older than 11 years), 2 months in hemangioendothelioma (HE), and 3 months in hamartoma. The ratio of male to female was 11 in HB, and 41 in HCC. The presence of hepatitis B infection was detected in 80% (4/5) of HCC and in 13% (2/16) of HB. The common presenting symptoms at diagnosis were the palpable mass, abdominal pain and jaundice. Many tumors were found incidentally without any presenting symptoms. Of 33 patients, 22 had involvement at right lobe. HCC had more necrosis and hemorrhage within the tumor than HB on imaging study. HE had a distinct feature of contrast enhancement increasing from the peripheral portion of the mass to central portion. Hamartoma showed a feature with multiple cysts and septums within the mass. To confirm the diagnosis, a tissue biopsy was done in 23 patients (70%). A significantly elevated level of serum alpha-fetoprotein (AFP) is found in 88% (mean 386,000 ng/mL) of HB cases and 100% (mean 369,000 ng/mL) of HCC. The mean AFP level in HB patients was 391,000 ng/mL at diagnosis, 52 ng/mL after the preoperative chemotherapy, 8.3 ng/mL at postoperative follow-up, and 2.8 ng/mL at completion of chemotherapy. In HCC, the AFP level decreased temporarily after chemotherapy or chemo- embolization, but increasesd at recurrence in most case. Patients resectable at the time of diagnosis or after preoperative chemotherapy were 12 (75%) in HB, and 10 of them are were alive with median the follow-up of 34 months (5 mo.~8 yrs 7 mo.). All the HCC patients were unresectable or had metastatic lesion at diagnosis, and all of them were assumed dead following discharge. Conclusions: Among the primary hepatic tumors in children, malignant tumors are more frequent than benign. Age of onset, AFP level and imaging studies all play an important role in diagnosis, and biopsy is usually confirmative. HCC has an extremely poor prognosis in childhood. HB patients with complete surgical resection enjoy a relatively high long term survival.