Childhood Cancer

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Childhood Cancer

Hepatoblastoma

Hepatoblastoma is the type of liver cancer most often diagnosed in young children. Almost all (95%) children with hepatoblastoma are diagnosed before they are 3 years old. The incidence of hepatoblastoma has doubled in the last 20 years. It is thought that this rise in incidence may be related to the increase in rates of survival of very low birth weight, premature infants who are known to have a higher risk of developing hepatoblastoma than children who were not born prematurely.

Children with certain syndromes (e.g., Beckwith-Wiedemann syndrome, familial adenomatous polyposis, and Aicardi syndrome) are at higher risk of developing this cancer. Children with a condition called hemihypertrophy (when one side of the body grows more quickly than the other side) are also at increased risk of developing hepatoblastoma. However, most children with these conditions do not develop hepatoblastoma.

Staging

Wyatt was diagnosed when he was 9 months old. He wasn’t sitting up very well, so I asked the pediatrician about it and he said Wyatt had hypotonia (low muscle tone) and referred us to a physical therapist. She was checking the muscles in Wyatt’s abdomen and she felt a lump. I took him to the pediatrician that afternoon, and he thought it might be an enlarged liver. He sent us to the closest children’s hospital, and they found a huge tumor—6 x 7 cm. He had imaging, was admitted, then the next day they did a biopsy and placed a central line. His tumor was Stage III because it was in three lobes. He gets 5-FU, vincristine, and cisplatin and it’s shrunk by 50%, but he’s on the transplant list because it’s only 2 mm away from a major blood vessel. So, they can’t resect it and leave clean margins.

Once hepatoblastoma has been diagnosed, the pediatric oncologist will order more tests and scans to determine the extent of the disease. Imaging studies, such as a CT, MRI, or bone scan, should be performed to check for metastases (spread of the cancer to other parts of the body).

Staging is needed to choose the best treatment. In the United States, staging is based on whether the tumor can be completely removed surgically and whether it has metastasized.

•  Stage I. The tumor was completely removed.

•  Stage II. The tumor was removed surgically, but microscopic traces of disease were still present. Children with tumors that rupture during surgery are classified as having stage II disease.

•  Stage III. The tumor cannot be removed surgically or is partially removed, and disease was found in the lymph nodes.

•  Stage IV. The disease has spread to distant areas in the body. Ten percent of children with hepatoblastoma have metastatic disease in the lungs at diagnosis. Rare areas of spread can include the central nervous system (brain and spinal cord) and the abdomen.

Logan was 19 months old when he was diagnosed with hepatoblastoma. At diagnosis, the tumor was the size of a grapefruit and had spread to his lymph nodes and also to the right atrium of his heart.

After surgery, approximately 20 to 30 percent of children are found to have stage I or II hepatoblastoma, 50 to 70 percent have stage III disease, and 10 to 20 percent have stage IV disease.

An alternative staging system (called PRETEXT), widely used in Europe, is based on the number of liver lobes that contain cancer. The amount of tumor in each lobe is determined by MRI, and sometimes through CT or ultrasound. This staging system is used before surgery.

•  PRETEXT stage 1: Tumor is in only one lobe.

•  PRETEXT stage 2: Tumor is in two adjoining lobes.

•  PRETEXT stage 3: Tumor is in three adjoining lobes or two non-adjoining lobes.

•  PRETEXT stage 4: Tumor is in all four lobes. Tests are conducted to determine whether cancer has spread outside of the liver.

In international treatment protocols, all children with hepatoblastoma are treated with chemotherapy prior to surgery.

Treatment

To choose the best treatment for children with hepatoblastoma, pediatric oncologists consider the child’s stage of disease, characteristics of cancer cells (called histology), and the level of AFP in the blood. An initial AFP level of less than 100 ng/ml, regardless of stage, puts children in a higher risk category.

My infant daughter was diagnosed with stage III hepatoblastoma. It was in two lobes but couldn’t be removed because it was so close to the blood vessels in the liver. There is another spot on her liver, not near the tumor, that they don’t know if it’s more tumor or a bile duct. So, we don’t know if the surgeon is going to take the spot and the tumor, or just the tumor. I hope they can take it all so we’ll never have to worry about the mystery spot. Her AFP has gone way down, and they said that the scans show that the tumor might be dead and won’t shrink any more. They are going to do the surgery next week, then two more rounds of chemo after that. We know the surgeon—he did her biopsy, both Hickmans (had to have the first one removed due to a blood infection), and also put in her G-tube.

All children with hepatoblastoma need surgery. Because of the relationship between the quality of the surgery and children’s outcomes, surgery should be done by pediatric surgeons who have experience removing tumors in the liver and who have access to liver transplant programs (see section later in this chapter called “Liver transplantation”).

Logan’s chemotherapy was hung and dripping within 2 hours of the pathology report. Logan received cisplatin, vincristine, and 5-FU [fluorouracil]. He had chemotherapy every 3 weeks that usually took an average of 2 days. After Logan received the first round, there was a small decrease in size of the tumor. After the second, there was a little more. Since his tumor was responding, we decided to stay on the protocol.

Just 10 days after his second birthday, Logan had a 5-hour operation to remove the entire right lobe of his liver and his gallbladder. During surgery he lost quite a lot of blood and was given five units while in the operating room. He was transferred to ICU [intensive care unit] and was given another unit of blood and one of platelets. He remained there for only 36 hours and was transferred to the pediatric oncology floor.

By the third day, he was walking around. He had very little pain and he never complained. By the fourth day, he was running up and down the hall feeling wonderful. You would never guess that he had just had a major operation. On the fifth day, we were able to take him home. Two weeks after the operation, he went back to have one more round of chemotherapy and then we were done.

The most common treatment plans, based on stage of disease and the kind of cancer cells, are described below.

Stages I and II: Treatment of stages I and II hepatoblastoma of pure fetal histology (when the cancer cells look exactly like fetal liver cells under a microscope) usually involves surgery to completely remove the tumor. This is followed by regular monitoring of AFP levels and periodic imaging studies to see whether the cancer returns (called watchful waiting). Chemotherapy is generally not given. However, care must be taken to ensure that the histology diagnosis was accurate, because sometimes pockets of other types of hepatoblastoma cells are found in the tumor (called non-pure fetal histology). Children with these mixed types of cancer cells need more aggressive treatment, usually involving four courses of three chemotherapy drugs (cisplatin, vincristine, and fluorouracil) after surgical removal of the tumor. The chemotherapy is given to ensure any tumor cells that may have spread outside the liver are killed.

Stage III: Children with stage III disease are first treated with chemotherapy to try to shrink the tumor so it can be completely removed. The most commonly used combination of chemotherapy drugs includes cisplatin, vincristine, and fluorouracil. If the tumor shrinks enough, it is removed surgically. If the tumor cannot be completely removed, liver transplantation is sometimes recommended. Occasionally, a two-step treatment called transarterial chemoembolization is used. This involves giving chemotherapy directly to the tumor via a catheter and then cutting off the blood supply to the tumor by plugging the artery with material (e.g., metal coils or gelatin sponges).

A clinical trial ended right before Wyatt was diagnosed, so he is on standard treatment for his stage III tumor. He takes 5-FU, cisplatin, vincristine, doxorubicin, and also Zinecard® to protect his heart from the doxorubicin. Our go-to drugs are Zofran® and Benadryl® for the nausea and vomiting. After the fifth cycle, he vomited for 5 days despite the antinausea drugs, and he got really dehydrated and was hospitalized. They added Ativan® and Reglan® for the 2 weeks he was in. We just had a 3-week break and he gained a pound a week. I’m grateful that he seems to be luckier than some of the other kids we’ve met—he just hasn’t been as sick.

Stage IV: The standard treatment for stage IV hepatoblastoma is four courses of cisplatin, vincristine, and fluorouracil, followed by surgery to remove the primary tumor and any tumors in the lungs. If the primary tumor is completely removed, the tumors in other parts of the body can no longer be seen on scans, and the AFP returns to normal, then two more courses of the same three chemotherapy drugs are given. If the cancer in the liver cannot be removed through surgery and there are no signs of cancer in other parts of the body, the treatment is generally a liver transplant. If a liver transplant is not possible, treatment may include additional chemotherapy (e.g., ifosfamide, cisplatin, and doxorubicin), radiation therapy, or transarterial chemoembolization—giving chemotherapy directly to the tumor via a catheter and then cutting off the blood supply to the tumor by plugging the artery with material (e.g., metal coils or gelatin sponges).

To find out how to learn about the most current treatments available, see the section at the end of this chapter called “Information on standard treatments.”

DiagnosisHepatocellular carcinoma

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