|Amy Feldman, MD|
|Abramson Cancer Center of the University of Pennsylvania|
Neuroblastoma is a tumor that arises from developing sympathetic nerve tissue (the part of the nervous system that allows the body to respond to stress by adjusting heart rate, blood pressure, hormone levels, and digestion). This special type of nervous tissue is found next to the spinal cord, along the paths of nerve fibers, and in the adrenal glands (two organs which sit above each kidney and produce various hormones needed by the body). Neuroblastoma is a cancer that occurs most frequently in infants and young children and is rarely seen in children older than ten years of age. It is the leading form of cancer in infants and is the most common type of solid extra-cranial (outside the head) tumor in children. There are over 800 cases of neuroblastoma diagnosed each year in the United States. Overall prognosis depends on age (infants do better), stage (spread of disease), and the absence/presence of molecular and genetic markers in tumor cells.
Most patients with neuroblastoma present with signs and symptoms that are related to primary and metastatic tumor growth. Tumors located in the abdomen can cause abdominal pain, constipation, bladder dysfunction and abdominal swelling. Tumors located in the thorax (area around the lungs) can cause breathing difficulties, facial swelling, and droopy eyes due to the Superior Vena Cava Syndrome and Horner's Syndrome, respectively. Tumors located along the spinal cord or along various nerve fibers can present with any type of neurologic dysfunction including weakness, loss of sensation, pain, bladder and bowel incontinence, or paralysis. Spinal tumors can also cause scoliosis (curvature of the spine). Other symptoms that can be seen in association with neuroblastoma include bulging eyes or dark circles around the eyes (from metastases into the orbital sockets that hold the eyes), the appearance of two different colored eyes, p ainless bluish lumps under the skin in infants, complaints of back pain or limb pain (from spinal or bone metastases), watery diarrhea (from elevated levels of the substance "VIP"), jerky body movements or uncontrolled eye movements (from autoimmune antibodies), swelling of various body parts (from lymphatic blockage), hypertension (high blood pressure), or fatigue, bruising and bleeding (due to decreased amounts of blood cells when cancer overtakes the bone marrow).
A physician will perform a detailed physical examination, schedule imaging tests, and order laboratory tests to help determine if a child has neuroblastoma and to aid in staging and treatment.
A tissue diagnosis is needed for definite diagnosis of neuroblastoma and to aid in staging and prognosis. Biopsies (tissue samples) will be taken from the tumor site and bone marrow will be aspirated from both the right and left hip bones. These cell samples will be viewed carefully under a microscope to look for specific molecular and genetic tumor findings.
Neuroblastoma is a clinically diverse disease. There are several factors that affect prognosis and response to therapy.
Treatment of neuroblastoma depends on stage. In low and intermediate risk tumors, surgical removal of the primary tumor is the initial treatment and combinations of various chemotherapy drugs are added when needed. In high risk tumors where the long term probabilities of survival are less than 15%, treatment involves intensive combinations of chemotherapy agents, surgery, bone marrow transplantation, and radiation therapy. When these conventional modalities fail and disease recurs, novel therapies are attempted including retinoic acid, topotecan, immunologic agents, anti-angiogenic agents, and MIBG radionuclide therapy.
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