Tumor weighing 2 kg removed from the liver of a 10-month-old girl

When it was removed, the tumor mass amounted to almost 2 kg, about a quarter of her body weight: it happened to a 10-month-old and about 8 kg girl who had most of her liver removed due to a mesenchymal hamartoma. The surgery was successfully performed by the professor’s team Marco Spadahead of Hepato-Bilio-Pancreatic Surgery and liver and kidney transplants at the Bambino Gesù Pediatric Hospital. Today the baby, who recently turned one, is doing well, eating without problems and has started to grow regularly again.

A troubled twin

It was the alarmed parents who took the little girl to the Child Jesus Emergency Department. Compared to her twin brother, she showed a swelling of the abdomen and lack of appetite. The ultrasound immediately performed showed a large lesion with liquid content in the liver. The child underwent a multidisciplinary evaluation involving hepatologists, anesthesiologists-resuscitators, radiologists, oncologists and pathologists. The outcome was the confirmation of the presence of a liver neoplasm, more than 13 cm in diameter, which entirely occupied the right and central part of the liver, compressing the left portion.

The tumor squeezed and forced the other surrounding organs (stomach, pancreas, intestine, right kidney) into a dislocation from their original site. The radiological characteristics of the lesion led to the suspicion that it was a mesenchymal hamartoma.

What is mesenchymal hamartoma

Mesenchymal hamartoma is a benign tumor resulting from the abnormal growth of liver cells of mesenchymal origin. The term hamartoma comes from the Greek word meaning “mistake”. The cells that make up hamartoma are normal, but grow in a disorganized way.

Although rare, it is the second most frequent tumor that can develop in the liver in children, especially in the first two years of life. In consideration of the dimensions, the localization and its prevailing liquid component, a biopsy of the lesion would not have allowed in safety and with certainty to confirm the benign nature of the tumor and to exclude that the neoplasm was not instead an undifferentiated embryonic sarcoma, a malignant tumor which it can always present itself in childhood with radiological characteristics similar to those of mesenchymal hamartoma. For this reason it was necessary to proceed with its surgical removal.

The therapeutic strategy

Using sophisticated CT image processing software, a three-dimensional model of the liver was built to evaluate the size and relationship of the tumor with the healthy part of the organ and its blood vessels. The model made it clear that the removal of the tumor would have left an insufficient amount of healthy liver which, despite the regeneration capacity of the liver cells, would not have guaranteed the proper functioning of the organ after the operation.

Professor Spada’s team therefore decided to adopt a strategy that allows for an increase in the volume of the healthy liver in a few weeks, which will remain after the tumor is removed. This method of endovascular radiology, called portal embolizationwhich is more often used in adult patients, consists of blocking the blood supply to the part of the liver occupied by the tumor and diverting all blood flow from the portal vein to the healthy part of the liver.

A recent meta-analysis conducted by the University of Heidelberg has documented that up to now only 8 cases of portal embolization performed in pediatric patients have been described in the literature. Only two of these were as small as the patient of the Child Jesus.

The surgery

Four weeks after the portal embolization, performed by the interventional radiologists of the Holy See Hospital, a new CT scan with 3D reconstruction confirmed that the healthy liver had doubled, going from an estimated volume of 80 to 120 ml, favoring the conditions to carry out the intervention.

The child underwent tumor resection, which involved the removal of most of the liver, except for the healthy left lateral portion. In practice, 6 of the 8 segments (portions) that make up the liver have been removed. The weight of the mass removed was almost 2 kg, compared to the 8.5 kg of the girl’s weight. The operation lasted over 6 hours and once it was finished the little girl was transferred to intensive care.

“Such extensive liver resections – explains Professor Spada – are not frequent in young children and require high skills not only in hepatobiliary surgery, but also in anaesthesiological, intensive care, radiological, hepatological, oncological, anatomopathological and pediatric nursing, such as those present in our hospital Only in this way can children with liver tumors be treated correctly and safely.”

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The postoperative course was uneventful and the girl was discharged 9 days later. Histological examination confirmed that the tumor was a mesenchymal hamartoma. “At the checks carried out after her discharge – adds Spada – we verified that the child is very well, she has resumed eating and growing regularly. The “remaining” liver has already started the regeneration process which will ensure that its volume returns to normal within a few weeks, guaranteeing the child a completely normal life”.