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Diagnosis, Staging & Treatment

Diagnosing neuroblastoma involves staging and classifying the disease which determines treatment options and prognosis.

In addition to a complete medical and physical examination, diagnostic procedures for neuroblastoma may include the following:

  • blood tests  -  including a complete blood count, blood chemistries, kidney and liver function tests, and a urinalysis.
  • multiple imaging studies  -  to evaluate primary tumor and determine extent/location of any metastases, including:
  • computed tomography scan (Also called a CT or CAT scan.)  -  a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
  • magnetic resonance imaging (MRI)  -  a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
  • x-ray  -  a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
  • ultrasound (Also called sonography.)  -  a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
  • bone scans  -  pictures or x-rays taken of the bone after a dye has been injected that is absorbed by bone tissue. These are used to detect tumors and bone abnormalities.
  • bone marrow aspiration and/or biopsy  -  a procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells.
  • spinal tap/lumbar puncture  -  a special needle is placed into the lower back, into the spinal canal. This is the area around the spinal cord. The pressure in the spinal canal and brain can then be measured. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing to determine if there is an infection or other problems. CSF is the fluid that bathes your child's brain and spinal cord.
  • biopsy of primary tumor and/or metastatic lesions
  • MIBG Scan - A MIBG (iodine meta-iodobenzylguanidine) scan is a test used to find tumors of a specific origin. Special pictures are taken after a medicine is injected into a vein. The medicine is called a radiopharmaceutical (a tiny amount of a radioactive liquid). The pictures show the medicine in the tumors.

 ** The most common procedures you will have if NB is suspected is a blood panel, the CT scan, MIGB scan, and the bone marrow biopsy **

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Staging

Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging systems that can be used for neuroblastoma. Always consult your child's physician for information on staging. One method of staging neuroblastoma is the following:

  • stage 1  -  involves a tumor that does not cross the midline of the body, is completely resectable, and has not spread to other areas of the body. The lymph nodes on the same side of the body as the tumor do not have cancer cells present.
  • stage 2A  -  involves a tumor that does not cross the midline of the body, but is not completely resectable. This stage of tumor has not spread to other areas of the body, and lymph nodes on the same side as the tumor do not have tumor cells present.
  • stage 2B  -  involves a tumor that may or may not be completely resectable, has not spread to other areas of the body, but lymph nodes on the same side of the tumor have tumor cells present. Lymph nodes on the opposite side of the tumor must be negative for tumor cells in this stage of disease.
  • stage 3  -  involves a tumor that crosses the midline of the body, is not completely resectable, and lymph nodes are positive for tumor cells. This stage also includes a tumor that does not cross the midline, but the lymph nodes on the opposite side also contain tumor cells.
  • stage 4  -  involves a tumor that has metastasized to distant lymph nodes, bone marrow, liver, skin, and/or other organs (except as defined in stage 4S).
  • stage 4S  -  a child is younger than 12 months and has a tumor that may have metastasized (spread) to liver, skin, and/or bone marrow (includes minimal bone marrow involvement; more extensive bone marrow involvement should be classified as stage 4). The tumor is on one side of the body and is localized. It may have spread to the lymph nodes on the same side of the body. 
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Treatment for Neuroblastoma

Specific treatment for neuroblastoma will be determined by your child's physician based on:

  • your child's age, overall health, and medical history
  • extent of the disease
  • your child's tolerance for specific medications, procedures, or therapies
  • expectations for the course of the disease
  • your opinion or preference

Treatment may include (alone or in combination):

  • surgery (for tumor and/or metastatic resection, and removal of lymph nodes involved)
  • chemotherapy
  • radiation therapy
  • bone marrow transplant and peripheral blood stem cell transplantation
  • retinoid therapy
  • supportive care (for the side effects of treatment)
  • antibiotics (to prevent/treat infections)
  • continuous follow-up care (to determine response to treatment, recurrent disease, and late effects of treatment)

Treatment options should always be discussed with your child's physician/oncologist. 

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Prognosis 

Approximately 70% of patients with neuroblastoma have metastatic disease at diagnosis. The prognosis for patients with neuroblastoma is related to their age at diagnosis, clinical stage of disease, site of the primary tumor, tumor histology, and, in patients older than 1 year, regional lymph node involvement.

With treatment, many children with neuroblastoma have a good chance of surviving. In general, neuroblastoma has a more favorable outcome if the cancer hasn't spread or if the child is younger than 1 year old when it's diagnosed.

High-risk neuroblastoma is harder to cure and is more likely to become resistant to standard therapies or come back (recur) after initially successful treatment.

Amplification of the MYCN gene is associated not only with deletion of chromosome 1p, but also gain of the long arm of chromosome 17 (17q), the latter of which independently predicts a poor prognosis. In contrast to MYCN gene amplification, the degree of expression of the MYCN gene in the tumor does not predict prognosis. However, high overall MYCN-dependent gene expression and low expression of sympathetic neuron late differentiation genes both predict a poor outcome of neuroblastomas otherwise considered to be at low or intermediate risk of recurrence. 

Gene expression profiling may prove useful for prognosis prediction.

 


  Neuroblastoma behaves differently in each child and each case is unique. Just because one child had a certain outcome does not mean all children will have that same outcome.

It is our hope that one day, the prognosis statistics for this disease will be greatly improved and we will be able to fight this disease with better ammunition.


 

 

The medical information provided on this page was gathered from the Childrens Hospital of Philadelphia (CHOP) website on Neuroblastoma. Comments in orange are from the founders of Daxton's Fish, Inc and their personal experience.