Hepatoblastoma in the neonatal period: An unusual presentation
A 10-day-old male baby was brought to the emergency room with complaints of jaundice and respiratory distress. The baby was born at term by normal vaginal delivery and birth weight was 3 kg. On clinical examination, chest was clear and an abdominal lump was palpable in the right upper quadrant with smooth surface, moving with respiration. At the upper part of the lump, the lower border of liver was palpable and appeared to be separate from the lump. Ultrasonography (USG) of abdomen suggested an adrenal mass lying closely to upper pole of the right kidney. There was unconjugated hyperbilirubinemia and prothrombin time was normal. Vanillylmandelic acid (VMA) was not elevated. Next, USG-guided fine needle aspiration cytology (FNAC) was attempted. The smear [] was highly cellular, composed of tumor cells arranged in trabeculae and acini. The cells had abundant cytoplasm with distinct borders. The nucleus was round with fine chromatin and some of the cells showed single central nucleoli. Some foci showed the trabeculae lined by endothelial cells on one side. Tumor cells did not show the presence of bile pigments. There was evidence of extramedullary hematopoiesis featured by the presence of erythroid precursors and megakaryocytes []. The cytomorphology suggested HBL of fetal type. AFP level was marginally elevated for age (9790.0 ng/ ml). Peripheral blood count (PBC) showed normal values. Laparotomy was done and a tumor was found projecting from undersurface of the liver, extending to cover the upper part of right kidney. PRETEXT staging (International Society of Pediatric Oncology) showed the tumor infiltrated the liver extensively with only the left lateral sector (segments II and III) free. The stage was III A I, E0 M0 P0 V0. A biopsy was taken including a margin of normal liver. The histopathology showed the tumor mass composed of cells resembling, but slightly smaller than normal hepatocytes and arranged in trabeculae. Cells mostly had clear cytoplasm, while some showed eosinophilic cytoplasm, resulting in alternate pink and white appearance. Nuclei were small and round with extramedullary hematopoiesis. No mitotic activity was found. Normal liver tissue was seen at the periphery. The histopathology thus confirmed the diagnosis of HBL of fetal type. The baby was put on chemotherapy regime of Cisplatin and Doxorubicin. Three cycles had been given at three weekly intervals. The baby is doing well and the mass has reduced in size as seen on computed tomography (CT) scan.