Gaucher´s Disease vs. Hepatoblastoma

Gaucher´s Disease vs. Hepatoblastoma

Left picture: In this infant the cause of the upper belly mass is an infantile type of Gaucher´s disease, which is the most frequently encountered type of inherited lipoidoses. The former total splenectomy may become unnecessary due to the present available treatment with bone marrow transplantation. Right picture: This infant has a hepatoblastoma which is the most frequent malignant liver tumor in the first three years of life. Abdominal mass, distension and/or pain is observed in more than two thirds of the children. As seen in the picture at the top there is usually a bulky solitary mass surrounded by a pseudocapsule. The second picture from the top shows the bulky but typically intrahepatic site of the hepatoblastoma in a 11-month-old boy; therefore the clinical presentation may be a less localized mass as it would be present in the picture at the top and clinically similar to the third picture from the top.

Two infants with a prominent upper belly. Left picture: As shown in the picture this 1.1-year-old boy has a large mass which includes both sides in the upper belly; the visible part has the size of the relatively small thoracic cavity. Right picture: In this 9-month-old boy mainly the left upper belly is prominent, which is explained by the exophytic mass hold by the surgeon after a left subcostal incision. Left picture: The skin is rather pale and the mass in the upper belly corresponds to a impressive hepatosplenomegaly with its lower border at and below the level of the navel. Right picture: The mass is in connection with the left lobe and has a pseudocapsule.