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Neuroblastoma
Dr Ira Shah
M.D, DNB, DCH(Gold Medalist), FCPS

Neuroblastoma is one of the most common cancers affecting children today, forming 8%-10% of the total seen from birth through age 14 years. Neuroblastoma occurs with slightly more frequency in males than in females. The median age at diagnosis is 2 years. It develops from the tissues that form the sympathetic nervous system. Neuroblastoma typically begins in the abdominal area either in the adrenal gland (located just above the kidney) or around the spinal cord in the neck, chest, or pelvis. Although neuroblastoma often is present at birth, it generally is not detected until the tumor begins to grow and compress the surrounding organs. Cancer cells can metastasize (spread) quickly to other areas of the body, such as lymph nodes, liver, lungs, bones, the central nervous system and bone marrow. Close to 70 percent of children diagnosed with neuroblastoma will have metastatic disease. It has a wide range of "virulence"; children with localized neuroblastoma can be cured by surgery alone but those with bone metastases usually have a fatal outcome (CANCER, 59:1853-1859, 1987).

The most common symptoms include the following:
An abdominal mass, either felt during an examination or seen as a swollen abdomen .
Uncontrolled eye movement caused by the tumor
Swelling and bruising of the area around the eyes, caused by metastases (tumor spread)
Compression of kidney or bladder by the tumor may cause changes in urination
Pain, limping or weakness may be present from bone involvement
Anemia or bruising may be present if there is bone marrow involvement
Paralysis and weakness may be present if there is spinal cord involvement
Diarrhea caused by a substance produced by the tumor (vasoactive intestinal peptide or VIP) may be present
Fever

Diagnosis

Determination of urine catecholamine excretion levels - These levels are usually higher than normal due to tumor production
MIBG Scans - MIBG is a substance selectively taken up by neuroblastoma cells. This scan highlights the location of the tumors in a patient's body.
Bone marrow biopsy and / or aspiration
Biospy of primary tumorand/ormetastatic lesions

Treatment

Treatment includes a wide range of approaches. Depending on individual circumstances, these approaches are used either alone or in combination:

Surgery. This is done to remove the primary tumor and stage the patient in order to assess metastases.
Chemotherapy
Radiation Therapy
Blood and marrow transplant

References
  1. Nelson textbook of pediatrics, 17th edition, page 1709-1711
  2. Rudolph Pediatrics, 21st edition, page 1617-1620
  3. Nathan & Oski. Hematology of infancy &childhood, page 1376-1382
Last Updated on 11-08-2007

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