Dr. Mahak Bhasin, Dr Sindhu, Dr. Jayakumar, Department of Pediatrics, AIMS, Kochi
Six month old male ba born to G3P1L1 mother, GDM with Polyhydramnios with antenatal scans showing increasing abdominal circumference in the ba delivered vaginally ,induced at 35 weeks and 4 days of gestation. Birth weight was 3.380kgs at birth and ba was treated in the NICU for neonatal jaundice.
After birth, ba was found to be feeding well and growing well, however, a progressive increase in abdominal size with a hard mass per abdomen was felt the parents who were initially reassured local Pediatricians. Ba was developing normally, gaining weight and immunised for age but i/v/o the above concerns, he was evaluated at outside hospital.
Family history of colorectal carcinoma in paternal grandfather present.
On examination, child was active and playful. He had a hard mass about 12×15cm non tender palpable per abdomen with dilated veins visible per abdomen.
Blood investigations done showed neutrophilic leukocytosis with thrombocytosis and Hb 6.6g/dL MCV 58.5 AFP checked was 429386ng/mL. Preop serology done was negative.
Differentials
Hepatoblatoma
Nephrotic blastoma
Neuro blastoma
Hydronephrosis
Teratoma
Omental cyst
Choledocjsk cysts
Intestinal duplication cyst
USG abdomen done showed 12×12×10 cm lesion in the right hypochondrium and lumbar regions compressing the right lobe of the liver. CT Abdomen done showed a large heterogeneously enhancing mass with cystic areas and calcific foci in the intraperitoneal cavity measuring 10.2×13× 11.8 cm–likely a large exophytic hepatoblastoma arising from the left libe of the liver and non-opacification of the infrarenal IVC-?compressed.
USG Guided Liver Biospy thus done showed mixed epithelial and mesenchymal hepatoblastoma.
Hepatoblastoma predominantly occurs in children below 3 years of age. Their occurrence is associated with Familial Adenomatous Polyposis and with syndromes like Beckwith Weideman Syndrome and other somatic overgrowth syndromes. Prematurity and low birth weight are also recognised as risk factors.
Histologically, these are classified as Whole Epiyhelial Types, containing fetal or embryonal malignant cells and Mixed Types, containing both epithelial and mesenchymal elements.
These usually present as large asymtomatic abdominal masses with clinical symptoms becoming more apparent as the disease progresses.
Biopsy of the liver mass is crucial for establishing diagnosis and AFP levels are used in both establishing the diagnosis as well as monitoring of the tumor. Anemia is common and thrombocytosis is seen in 30% patients.
Treatment is based on surgery and systemic chemotherapy using cisplatin with vincristine and 5-fluorouracil. Unresectable tumors with or without metastatic disease respond to chemotherapy and resection of the primary tumor and extrahepatic disease ought to be undertaken once feasible. Orthotopic liver transplants also result in good long term survival rates for most patients with unresectable tumors.
While the prognosis for low stage resectable tumors can be as good as 90%, for unresectable tumors, survival rates of approximately 60% can be obtained.