A Tale of Two Children with Liver Tumors
(...in the same City!)

A three-year-old boy was admitted to a major hospital in a large city in Saudi Arabia with upper abdominal swelling. Imaging revealed a mass in the liver and a blood test showed thrombocytosis and an extremely high serum level of alphafetoprotein (a-FP). There were no lung metastases. A needle biopsy of the mass confirmed the clinical diagnosis of "hepatoblastoma". After the biopsy, the child deteriorated with increasing abdominal girth, pleural effusions and hypoalbuminaemia and was transferred to the Intensive Care Unit (ICU) where he continued to deteriorate, despite supportive care including fresh frozen plasma, diuretics and parenteral nutrition. He was judged to be "too ill for chemotherapy," despite advice to the contrary from a paediatric oncologist. He died six weeks after admission, having had no cancer treatment whatsoever.

A 10-month-old girl was admitted at around the same time to a different hospital in the same city also with a large upper abdominal mass and a very high serum a-FP level. Biopsy confirmed hepatoblastoma and a CT scan of the lungs showed no metastases. Within a few days she was treated with cisplatin and doxorubin ("PLADO") chemotherapy at doses and on a schedule similar to that of the International Society of Pediatric Oncology’s first collaborative liver tumor study (SIOPEL-1). The patient’s condition stabilized and then steadily improved, with reduction of abdominal girth and a steep fall in serum a-FP levels. After four courses of PLADO given at 3-4 weekly intervals and with only one complication—an episode of febrile neutropenia, which rapidly resolved after broad-spectrum intravenous antibiotic treatment—she was referred to a tertiary pediatric surgical center for removal of the primary tumor. Near-complete excision was successfully achieved though a small amount of tumor, immediately adjacent to the hepatic artery and vein, was so closely adherent to these vessels that the risk of removal was deemed unacceptable. Histopathology examination confirmed that there was major tumor necrosis due to the chemotherapy with only around 10% residual viable tumor in the resected mass, most of which comprised scar tissue. After 48 hours in the ICU the baby—now 13 months old—returned to the children’s ward. She left hospital on the fifth post operative day and completed her treatment with two more courses of PLADO, via a Portacath which had been placed during her stay in the tertiary care center. At the end of treatment she showed no evidence of tumor in the liver, which had already regenerated to a virtually normal volume for her age and also normal cardiac, auditory, renal and hepatic function and a normal serum a-FP level. Some four months after the end of all treatment she remains well, is developing normally and is on the fiftieth percentiles for both height and weight.

Comment

Recent clinical trials have convincingly demonstrated remarkable improvement in the prognosis of children with liver tumors, especially hepatoblastoma. In the SIOPEL 1 study, for instance, relapse-free survival—with a median follow-up of four to five years—was 70-80%. Even children with metastases in SIOPEL 1 had a 30-40% chance of cure. Comparable figures as recently as the late 1970s were 20-30% and <10%, respectively. An article describing the SIOPEL studies—past and present—will appear in a future edition of Network. Suffice it to say, for the moment, that the chemotherapy regimes are relatively straightforward and, so long as there is a central venous access device and appropriate monitoring, well tolerated. Radiotherapy is hardly ever needed. The timing of surgery and the choice of surgical center are crucial. These themes will be developed further in the upcoming SIOPEL trials review—so watch this space!

-- Jon Pritchard Jeddah, Saudi Arabia