Gaucherīs Disease vs. Hepatoblastoma

Gaucherīs Disease vs. Hepatoblastoma

Left picture: In this infant the cause of the upper belly mass is an infantile type of Gaucherīs 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.