EPITHELIAL HEPATOBLASTOMA IN A MALE INFANT WITH BECKWITH-WIEDEMANN SYNDROME: A CHALLENGING CASE REPORT

Abstract

Hepatoblastoma (HB) is among the most common pediatric primary liver tumor and constitutes almost 90% of tumors in children, aged 5 years, or younger. Hepatoblastomas are classified based on histological subtypes, which describe the types of cells and tissues present in the tumor. The primary classification divides them into epithelial and mixed epithelial and mesenchymal types. A two-month-old male infant was admitted at the Department of Pediatric Surgery-Clinical Hospital Acibadem Sistina, via referral from a pediatrician, with chief medical complaints of macroglossia, left-sided longitudinal hemi-hypertrophy and facial dysmorphic stigmatic features, for further clinical evaluation. After the initial physical examination, clinical investigations were obtained by protocol. Laboratory results showed elevated levels of lymphocytes, thrombocytes, RDW-SV, and hyperglycemia as well. The levels of alpha-fetoprotein (AFP) were >1000.0 (0-28 kiU/L). Imaging CT scan showed two tumefactions located between the 5^th and 6/7^th liver segments, clearly demarcated with dimensions of 57 mm (larger lesion) and 33 mm (smaller lesion), while the other intra-abdominal organs appeared normal. An urgent indication for surgical treatment was established, and a right-sided laparotomy was made. Intraoperative findings were two large liver masses located in the right lobe. Cholecystectomy and right partial hepatectomy were performed.  Histopathological analysis revealed epithelial hepatoblastoma, embryonal subtype. Histopathology was performed using the PRETEXT (Pretreatment Extent) staging system developed by the International Society of Pediatric Oncology (SIOPEL). After the procedure, chemotherapy was initiated according to the standard protocol.