Childhood Cancer

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Childhood Cancer

Treatment

At diagnosis, many parents don’t know how to find experienced doctors and the best treatment for their child. State-of-the-art care is available from physicians who participate in the Children’s Oncology Group (COG). This study group, composed of pediatric surgeons and oncologists, neurologists, radiation oncologists, researchers, and nurses, provides the best current treatments for childhood cancers and conducts studies to discover better therapies and supportive care for children with cancer. You can learn more about COG and find a list of its member treatment centers at www.childrensoncologygroup.org.

After diagnosis and staging, the pediatric oncologist will discuss with you the bestknown treatment or a clinical trial (see Chapter 9, Choosing a Treatment) based on the following risk categories:

•  Low risk. Children with low-risk disease do not require aggressive treatment. They usually have surgery or biopsy, followed by observation. The following are included in the low-risk category:

– All children with INSS stage 1 disease

– Children with INSS stage 2 disease, except those older than age 1 at diagnosis with MYCN amplification and unfavorable pathology

– Infants with 4S disease with hyperdiploidy, favorable pathology, and non-amplified MYCN

•  Intermediate risk. Children with intermediate-risk disease generally require surgery and 12 to 24 weeks of chemotherapy. Radiation therapy is occasionally necessary. The following are included in the intermediate-risk category:

– Children younger than age 1 with INSS stage 3 disease with non-amplified MYCN

– Children older than age 1 with INSS stage 3 disease with non-amplified MYCN and favorable pathology

– Children younger than age 1 with INSS stage 4 disease with non-amplified MYCN

– Children younger than age 1 with stage 4S disease, non-amplified MYCN, diploidy, or unfavorable pathology

•  High risk. Children with high-risk neuroblastoma need aggressive treatment, which usually lasts 10 to 12 months and is divided into three phases: 1) an induction phase of chemotherapy and surgery; 2) a consolidation phase with peripheral stem cell transplantation and radiation and; 3) a maintenance phase that includes 6 months of isotretinoin and immunotherapy. The following are included in the high-risk category:

– Children older than 1 year with INSS stage 2 disease with amplified MYCN and unfavorable pathology

– Children younger than age 1 with INSS stage 3 disease with amplified MYCN

– Children older than 1 year with INSS stage 3 disease with non-amplified MYCN and unfavorable pathology or with amplified MYCN

– Children older than 1 year with INSS stage 4 disease

– Infants with stage 4 or 4S disease with amplified MYCN

The goal of treatment is to achieve a complete remission by killing all cancer cells as quickly as possible. Complete remission occurs when all signs and symptoms of neuroblastoma disappear and abnormal cells are no longer found by any standard evaluation (MRI/CT scan, bone scan, bone marrow aspiration, or biopsy). Newer methods are being evaluated that can detect a much smaller amount of tumor in the blood or marrow. This is called the detection of minimal residual disease (MRD).

Chemotherapy

They did a biopsy of the tumor, a bone marrow biopsy, mIBG scan, bone scan, a full staging. Once we had a diagnosis, they sat us down and said, “This is what’s going to happen,” and presented the stages of treatment. They added, “We’ll tell you what you can expect at each stage when we reach it.” I made the nurse practitioner sit down and explain everything to me, and told her, “I need you to tell me what is coming down the line.” I saw kids up there whose parents were really detached. They didn’t understand, and they didn’t want to understand. They didn’t need to know like I needed to know.

Chemotherapy is used to treat almost all children with intermediate- and high-risk neuroblastoma, and some children with low-risk disease. Response rates have improved considerably through the use of multi-drug regimens (treatments using more than one chemotherapy drug). The most commonly used chemotherapy drugs include carboplatin, cyclophosphamide, doxorubicin, and etoposide. Cisplatin, vincristine, and topotecan are also used for children with high-risk disease. For more information, see Chapter 15, Chemotherapy.

Tanner (13 months) went immediately into the pediatric intensive care unit [PICU] because the tumor was pushing on his lungs. His belly was huge and he was having trouble breathing. He was intubated, but he was awake and on a lot of pain meds. It took several days after the biopsy to get the results, then we got a treatment plan and he started chemotherapy in the PICU. We stayed in the PICU through the first two chemo treatments, and then he stayed on the regular oncology unit for the third one.

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Luke (2 years old) had side effects from his chemo protocol that were relatively minor compared to what other children experience. He did have nausea, but it usually consisted of one to three vomiting episodes over 1 or 2 days and was over quickly. Over the entire 8-course cycle, he did have numerous neutropenic bouts, with several visits to the emergency room for night-onset fevers resulting in short-term hospitalizations to get IV antibiotics. He did get two or three infections in his Broviac® that also required hospitalizations for antibiotics. However, he only needed one blood and one platelet transfusion during the entire protocol.

Generally, parents receive an outline of the chemotherapy treatment plan (called a protocol) that includes the drugs that will be used, the doses, and a treatment schedule.

Surgery

Almost all children with neuroblastoma need to have surgery. It is used to obtain tumor tissue for diagnosis, to stage the disease, to remove the tumor, and for second-look surgeries. If the tumor hasn’t spread and appears resectable, surgery is performed soon after diagnosis. However, this usually is not possible, and chemotherapy is used to shrink the tumor before surgery. Even after chemotherapy, surgical removal is often incomplete in children with stage 3 and 4 disease, and radiation is required to destroy remaining tumor cells.

Tanner’s tumor shrank quite a bit before the surgery. The surgery took 6 to 7 hours, and the surgeon who saw him in the beginning told me, “I will get this tumor out.” He had a great attitude. An MRI showed some tumor or necrotic [dead] tissue afterwards, so the second surgeon went in a couple of weeks after the first surgery to make sure no live tumor was left in. Tanner did fine. They bounce back really quickly when they’re little.

The goals of the initial surgery will depend on a variety of factors, including tumor location, resectability, proximity to major blood vessels and nerves, and your child’s prognosis. Lymph nodes near the tumor are usually sampled to determine whether the disease has spread. Sometimes a liver biopsy is done to determine whether an infant has stage 4S disease. However, in older children, the value of this practice is unclear if the liver appears normal on the scans and during surgery.

The surgery to remove the tumor took about 4 hours, and there were no major complications. Scott spent 3 hours in the recovery room before being ready to go to the ward. His first words after waking were, “I love you, Mom.” While in the recovery room, we realized that Scott’s stuffed Yoshi® toy, which also went to the operating room, returned with a neck bandage identical to Scott’s. Someone had a sense of humor!

Complications can occur during or after aggressive surgeries. Common complications are bleeding, adhesions after surgery (internal scars that make tissues or organs stick together), injuries to vessels leading to the kidneys, and neurological problems such as Horner’s syndrome. When surgery is done after chemotherapy has been given to shrink the tumors, fewer complications occur.

Scott has a minor complication from surgery called Horner syndrome. His left eyelid droops, and the pupil is smaller than that of the right eye. It doesn’t impact his vision, but it is clearly noticeable when you look at him.

Spinal cord compression, often called “dumbbell” extension, occurs when a tumor invades the spinal canal. Chemotherapy is often used to treat this condition, but in some cases, a surgical procedure called laminectomy is done instead. A laminectomy removes part of the bone covering the spinal canal, relieving symptoms caused by compression of the cord and nerve roots.

Radiation therapy

Neuroblastoma is very sensitive to radiation. Radiation therapy is used for some children with intermediate-risk disease when their tumors do not respond well to chemotherapy. It also is a standard part of the treatment protocol for children with high-risk disease, and is sometimes used to relieve pain. For more information, see Chapter 17, Radiation Therapy.

Stem cell transplantation

My son experienced very few side effects from the local radiation he received to his abdomen and chest. He was premedicated each time with Zofran® and only had a few bouts of vomiting over a 2-week course. Other than diarrhea, he tolerated it great and wasn’t even very fatigued.

Peripheral blood stem cell transplant (also referred to as hematopoietic stem cell transplant or HSCT) is often part of the protocols used to treat children with high-risk or relapsed neuroblastoma. The child’s own blood stem cells are removed and frozen early in treatment, then transplanted back during the consolidation phase of treatment. Some treatment centers are experimenting with doing tandem transplants (two transplants in a row) in an attempt to increase the likelihood of destroying all the neuroblastoma cells. Chemotherapy drugs such as melphalan, cisplatin, teniposide, and doxorubicin are used to prepare children for transplant. For more information, see Chapter 18, Stem Cell Transplantation.

Differentiation therapy and immunotherapy

Preparation for the transplant was harder on me than it was on 7-year-old Nelson. They had given him a month off from chemo so he was feeling really good, and they explained to him that he was going to get a whopping big dose of chemotherapy and he wasn’t going to feel too well. And he didn’t. We had some issues of him not wanting to eat since he completely lost his appetite. He was like a limp rag and felt really, really lousy. His counts bounced back really fast after the transplant, and he was only in the hospital 4.5 weeks before we got out of there. He’s a major fighter.

13-cis-retinoic acid (isotretinoin) is in a category of drugs called retinoids (relatives of Vitamin A), which are thought to cause normal cells to differentiate, or mature, into normal cells more rapidly. Thus, treatment with isotretinoin is sometimes called differentiation therapy. Isotretinoin is taken by mouth daily for 2 weeks (followed by a 2-week rest) for 6 months after a stem cell transplant to stop the growth and spread of neuroblastoma. This treatment only works when there is minimal residual disease (MRD), not when there’s a substantial amount of tumor left.

The monoclonal antibody ch14.18 is an important immunotherapy treatment used to treat children with high-risk neuroblastoma. It is given in five cycles along with two substances (GM-CSF and interleukin-2) that help the white blood cells use the antibody to more effectively destroy the neuroblastoma.

PrognosisInformation on standard treatments

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