Childhood Neuroblastoma: Staging
When a child is diagnosed with most types of childhood cancer, including neuroblastoma, a critical part of the diagnosis is the “stage” of the disease. The stage of the disease indicates how far the disease has spread or progressed from the site of the original tumor. In the case of neuroblastoma, there are currently two methodologies in use to determine staging.
The International Neuroblastoma Risk Group Staging System (INRGSS) relies upon the results of imaging tests such CT, MRI, or MIBG scans. Using this method, the stage can be determined in advance of a specific treatment protocol. This system utilizes a four-stage classification:
- L1: the tumor remains confined to one area of the body and has not spread
- L2: the tumor has only slightly spread but has at least one image-defined risk factor (IDRF)
- M: the tumor has spread to a distant area of the body
- MS: the tumor has spread only to the skin, liver, and/or bone marrow in a child who is younger than 18 months. To fall into this category, scans must demonstrate that no more than 10% of marrow cells are cancerous and the cancer has not spread to the bones.
Increasingly, many oncologists are moving towards this methodology because it enables them to develop a stage and risk assessment before deciding upon an appropriate treatment, especially for children for whom surgery is not appropriate or not immediately appropriate.
The International Neuroblastoma Staging System (INSS) assesses the stage of the disease after surgery to remove all or most of the tumor. This is the more traditional staging system, and is still the system utilized by the COG to determine risk groupings (see below). The stages in this system are:
- Stage 1: the tumor has not spread and can be entirely removed by surgery.
- Stage 2A: the tumor has not spread but cannot be removed entirely by surgery.
- Stage 2B: the tumor has not spread but cannot be entirely removed by surgery. Nearby lymph nodes contain some neuroblastoma cells.
- Stage 3: the tumor has begun to spread and (1) the cancer cannot be removed by surgery and has crossed to the other side of the body; (2) the cancer has not crossed the midline to the other side of the body except in the lymph nodes; and/or (3) the tumor is in the middle of the body and is growing out on both sides, either directly or through the lymph nodes, and cannot be removed entirely by surgery.
- Stage 4: the tumor has spread to distant parts of the body such as lymph nodes, bone, bone marrow, skin, liver, and/or other organs.
- Stage 4S: only applicable in children under 1 year of age. The cancer has potentially spread to lymph nodes, liver, skin, and/or bone marrow but only on one side of the body. Scans must show that no more than 10% of marrow cells are tumorous and the cancer has not spread to the bones.
Neuroblastoma Prognosis Factors
In addition to the stage of the disease, oncologists also determine so-called “prognosis factors”, which can help indicate how a specific neuroblastoma may respond to treatment. These prognosis factors may also impact risk assessment and long-term prognosis. For neuroblastoma, oncologists will look at:
- Tumor histology
- DNA ploidy
- MYCN gene amplifications
- Chromosome changes
- Neurotrophin (nerve growth factor) receptors
Childhood Neuroblastoma: Prognosis Statistics
For childhood neuroblastoma, statistics relating to prognosis and five-year survival rates are currently maintained by the Children’s Oncology Group (COG) and therefore utilize COG risk-groupings: low risk, intermediate risk, and high risk. These risk group classifications are determined by the disease’s stage and prognosis factors as described above.
As always when discussing five-year survival rates, it is important to note that these statistics provide only a statistical average for each classification; your child’s prognosis will depend on his or her specific diagnosis, health, response to treatment, etc.
Low Risk: “Low risk” neuroblastoma patients have a five-year survival rate that is greater than 95%. The low risk group includes neuroblastomas within the following categories:
- Any Stage 1 diagnosis
- Stage 2A or 2B; under 1 year of age
- Stage 2A or 2B; older than 1 year of age; with no extra copies of the MYCN gene
- Stage 2A or 2B; older than 1 year; with extra copies of the MYCN gene; with a favorable histology
- Stage 4S with favorable histology, hyperdiploid, and 0 extra copies of the MYCN gene
Intermediate Risk: “Intermediate risk” neuroblastoma have a five-year survival rate of about 90-95%. The intermediate risk group incorporates diagnoses within these specific categories:
- Stage 3; under 1 year of age; 0 extra copies of the MYCN gene
- Stage 3; older than 1 year; 0 extra copies of the MYCN gene; with a favorable histology
- Stage 4; less than 1 year of age; no copies of the MYCN gene
- Stage 4S; 0 extra copies of the MYCN gene; with normal DNA ploidy and/or an unfavorable histology
High Risk: “High risk” neuroblastoma have a five-year survival rate of about 40-50%. A high risk diagnosis involves the following classifications:
- Stage 2A or 2B, older than 1 year of age; extra copies of the MYCN gene; with an unfavorable histology
- Stage 3 (regardless of age) with extra copies of the MYCN gene
- Stage 3; over 18 months old; an unfavorable histology
- Stage 4 with extra copies of the MYCN gene
- Stage 4 and over 18 months old
- Stage 4; between 12 and 18 months old; with extra copies of the MYCN gene, unfavorable histology, and/or a DNA index of 1
- Stage 4S with extra copies of the MYCN gene
More about Childhood Neuroblastoma Cancers:
- About Childhood Neuroblastoma Cancer – Detection and Diagnosis
- Causes, Risk Factors, and Prevention of Childhood Neuroblastoma Cancer
- What are the signs and symptoms of Childhood Neuroblastoma Cancer?
- Childhood Neuroblastoma Cancer Treatment
- Childhood Neuroblastoma Cancer – Stages and Prognosis
- What is the expected life span of Childhood Neuroblastoma Cancer?
- After Treatment – Living as a Childhood Neuroblastoma Cancer Survivor
Learn More About the Different Types of Childhood Cancers:
- Childhood Brain Tumor Cancer (Brain Stem Tumors)
- Spinal Cord Tumor Cancer
- Childhood Neuroblastoma Cancers
- Childhood Hodgkin Lymphoma Cancers
- Non-Hodgkin Lymphoma Cancers
- Wilms tumor (Kidney Tumors)
- Bone cancer (including osteosarcoma and Ewing sarcoma)
- Leukemia Cancers: Acute lymphocytic (lymphoblastic) leukemia (ALL) Acute myelogenous leukemia (AML); Juvenile myelomonocytic leukemia (JMML)
- Hepatoblastoma (Liver Cancer)
- Rhabdoid Tumors
About American Childhood Cancer Organization
American Childhood Cancer Organization (ACCO) is a non-profit charity dedicated to helping kids with cancer and their families navigate the difficult journey from cancer diagnosis through survivorship. Internationally, ACCO is the sole U.S. member of Childhood Cancer International (CCI), the largest patient-support organization for childhood cancer in the world. Here in the United States, ACCO promotes the critical importance of ensuring continued funding into new and better treatment protocols for childhood cancer. And most importantly, ACCO is focused on the children: developing and providing educational tools for children fighting cancer and their families, empowering them in their understanding of childhood cancer and the medical decisions they must make during this difficult journey. All of ACCO’s resources are available free of charge for families coping with childhood cancer.
For additional information about childhood cancer or on ACCO, or to order resources for you or your child, please visit our website at www.acco.org.