Childhood Cancer

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Childhood Cancer

Staging

Once a diagnosis of neuroblastoma has been made, the pediatric oncologist will order additional tests and scans to determine the stage (extent of the disease). Using the International Neuroblastoma Staging System (INSS), neuroblastoma is categorized into one of the following stages:

•  Stage 1. The tumor is limited to the site of origin and can be completely removed (resected), with or without microscopic residual disease (extremely small amounts that are visible only with the aid of a microscope). Lymph nodes on both sides of the abdomen or chest are negative, which means that no cancer is found when the lymph node tissue is examined under a microscope.

•  Stage 2A. The tumor affects only one side of the body and is limited to the site of origin; the cancer cannot be completely removed (visible amounts of disease remains); and lymph nodes on both sides of the body are negative microscopically.

•  Stage 2B. The tumor affects only one side of the body and is limited to the site of origin; complete removal may or may not be possible; lymph nodes on the same side of the body near the tumor are positive (contain neuroblastoma cells); and lymph nodes on the opposite side of the body are negative microscopically.

•  Stage 3. The tumor has grown across the midline and cannot be easily removed; regional lymph nodes may or may not be positive; the tumor affects only one side of the body with lymph node involvement on the opposite side of the body; or the tumor begins in the midline with lymph node or tumor involvement on both sides of the body.

•  Stage 4. The tumor has spread to distant lymph nodes, bone, bone marrow, liver, and/or other organs (except as defined for stage 4S).

•  Stage 4S. This stage includes only infants younger than 1 year of age. The tumor is confined to the site of origin described in stages 1 or 2, with spread limited to the liver, skin, and/or bone marrow.

When Adam was 3 weeks old, we took him to the pediatrician because his abdomen was swollen. We were shocked to learn that it was cancer—stage 4S neuroblastoma. He didn’t receive any treatment for his disease at that time, because his doctor felt he didn’t need it. Instead, we just watched him very closely. His tumors eventually calcified, and he was fine until 5 years later when he suffered a relapse and required chemotherapy. He has done very well, and recently we celebrated his twelfth birthday.

The INSS system has been used for the past 25 years, but staging can vary depending on how skilled or aggressive the surgeon is. The medical community is now evaluating a new staging system (called the INRG staging system) that is based on image-defined risk factors, rather than the extent of surgical removal.

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