Causes

This rare tumor has a yearly occurrence of less than 5 per 1,000,000 children.

Tumors of the nervous system vary in their degree of differentiation. The degree of differentiation determines how the tumors appear under the microscope and whether or not they are likely to spread.

Benign tumors are less likely to spread. Malignant tumors are aggressive, grow quickly, and often spread. A ganglioneuroma  is a benign tumor, while a neuroblastoma  (occurring in children more than a year old) is generally malignant.

A ganglioneuroblastoma may be localized to one area or it may be widespread, but it is usually less aggressive than a neuroblastoma. The cause is unknown.

Symptoms

Most commonly, a mass can be felt in the abdomen, but this condition may also occur in other parts of the body.

Exams and Tests

• Bone marrow aspiration and biopsy may be necessary.
• Bone scan may be necessary.
• CT scan or MRI scan of the affected area
• MIBG scan may be necessary.
• Specialized blood and urine tests
• Surgical biopsy to confirm diagnosis

Treatment

Because these tumors are rare, they should be treated in a specialized center by experts who have experience with them.

Depending on the specific nature of the tumor, treatment can consist of surgery, and possibly chemotherapy and radiation therapy.

Support Groups

The stress of illness can often be helped by joining a support group where members share common experiences and problems. See cancer - support group.

Outlook (Prognosis)

The prognosis depends on the extent of the tumor and whether or not some areas of the tumor contain the more aggressive cells of a neuroblastoma.

Possible Complications

• Invasion of the tumor into surrounding areas (spread of the tumor)
• Complications of surgery, radiation, or chemotherapy

When to Contact a Medical Professional

Call your health care provider if you feel a mass or growth on your child's body. Make sure children receive routine examinations as part of their well child care.

References

Sovak MA, Aisner SC, Aisner J. Tumors of the pleura and mediastinum. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Abeloff’s Clinical Oncology. 4th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 77.

Kim S, Chung DH. Pediatric solid malignancies: neuroblastoma and Wilms' tumor. Surg Clin North Am. 2006;86(2):469-487.

Park JR, Eggert A, Caron H. Neuroblastoma: biology, prognosis, and treatment. Pediatr Clin North Am. 2008;55(1):97-120.

Update Date: 3/2/2012

Reviewed by: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital.