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Neuroblastoma

Classifying & Staging

Neuroblastoma presents uniquely in each child, and its unpredictable behavior requires close coordination by pediatric oncologists, radiotherapists and surgeons. Treatment recommendations for neuroblastoma are based on the following factors:

• age at diagnosis

• tumor stage – describes the size of the primary tumor and whether or not the tumor has spread. The international neuroblastoma staging system is as follows:

• Stage 1: Localized tumor that is completely surgically removed at diagnosis.
• Stage 2: Localized tumor that cannot be completely surgically removed at diagnosis. Lymph nodes on the same side of the tumor may have tumor cells present.
• Stage 3: Large tumor that has spread across the middle of the body that cannot be surgically removed at diagnosis.
• Stage 4: Tumor of any size that has metastasized (spread) to distant lymph nodes, bone marrow, bone or liver.
• Stage 4S: Special stage that applies only to a child less than one year old. Small, localized tumor that has metastasized (spread) to liver, skin, and/or bone marrow.

Tests on the tumor specimen including:

• Tumor histology: Describes the appearance of the tumor cells under the microscope. A tumor is described as having either "favorable" or "unfavorable" histology depending on how active the cells look. Tumors with unfavorable histology tend to behave more aggressively.
• Tumor MYCN status: MYCN is a gene that regulates tumor cell growth. Tumor cells can have a single copy or multiple copies of this gene (called MYCN amplification). Tumors with MYCN amplification tend to behave more aggressively.
• Tumor DNA index (also called ploidy): Describes the number of chromosomes in the tumor cells, compared to the number in normal cells. Sometimes a DNA index tells us that a child needs more chemotherapy, especially in infants.

After taking all of these factors into account, neuroblastoma is classified into three risk groups. The risk groups are called "low," "intermediate" and "high" risk. These risk groups are used to determine treatment.

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