A Girl With Sotos Syndrome and Hepatoblastoma

Abstract

A three-year-old girl with Sotos syndrome was found to have liver enzyme deviations when she was evaluated for a fever of unknown origin. She was admitted to the Pediatric Surgery Department where contrast-enhanced computed tomography revealed a tumor in the S8 area of the liver and bordering the diaphragm. Histopathological evaluation of a tumor biopsy specimen led to the diagnosis of fetal-type hepatoblastoma. Subsequent tumor stage evaluation confirmed the absence of lymph node or distant organ metastasis and the tumor was classified into group I hepatoblastoma by a PRE-Treatment EXTent of disease system classification. Cis-platin (CDDP) (80 mg/m²) was administered as preoperative chemotherapy to decrease the tumor size before performing a right lobectomy. Two postsurgery courses of CDDP (80 mg/m²) completed the treatment. The patient showed good progress without recurrence of hepatoblastoma.