A 2 year old child with Beckwith-Wiedemann syndrome is brought to you with history of an abdominal mass in the right hypochondrium. It was also associated with thrombocytosis and anemia. A CT showed enlarged pulmonary hilar nodes. What is the most probable diagnosis? Note that viral markers are -ve in this patient.
Correct Answer: Hepatoblastoma
Description: This is a case of hepatoblastoma. It is associated with familial adenomatous polyposis, Beckwith-wiedemann syndrome and low bih weight. It most commonly occurs in children below the age of 3 years. Metastasis visually occurs to lung nodes. Hepatocellular Ca is seen in older children (adolescents) or adults. It is associated with hepatitis B or C infection or conditions like hereditary tyrosinemia, glycogen storage disease, a - antitrypsin deficiency and biliary cirrhosis. Hepatic hemangioma shows no metastasis and is associated with anemia and thrombocytopenia. Ref: Nelson, Edition 17, Page 1726
Category:
Pediatrics
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