MULTIPLE HEPATOBLASTOMAS ASSOCIATED WITH TRISOMY-18 IN A 3-YEAR-OLD GIRL

A very rare case of full trisomy 18 associated with multiple hepatobla stomas is reported. The patient also had ventricular septal defect and patent ductus arteriosus which were repaired at 6 months of age. Afte r the cardiac surgery, she was noted to have an abdominal mass and an elevated serum alpha-fetoprotein level. A Initial hepatic lobectomy wa s performed at 7 months of age, and the resected tumor was diagnosed a s a fetal-type hepatoblastoma. At 2 years and 4 months of age, a chest radiograph disclosed an elevated left diaphragm, and abdominal ultras onography demonstrated a tumor in the left hepatic lobe. The resected tumor was also diagnosed as a fetal-type hepatoblastoma. Chromosomal a nalysis demonstrated that the karyotypes of peripheral blood and hepat ic tumor cells obtained on two occasions were both 47,XX, +18. She has no evidence of recurrence at 3 years of age without specific therapy.