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Ganglioneuroblastoma

Definition:

Ganglioneuroblastoma is an intermediate tumor that grows nerve tissue. An intermediate tumor is one that is between benign (slow-growing and unlikely to spread) and malignant (fast-growing, aggressive, and likely to spread).



Causes, incidence, and risk factors:

This rare tumor occurs in less than 5 out of every 1,000,000 children each year.

Tumors of the nervous system have different degrees of differentiation. The degree of differentiation is based on how the tumor cells look under the microscope. It can predict 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. A neuroblastoma (occurring in children over 1 year old) is usually malignant.

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



Symptoms:

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



Signs and tests:
  • Bone marrow aspiration and biopsy
  • Bone scan
  • CT scan or MRI scan of the affected area
  • MIBG scan
  • Special 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 type of tumor, treatment can involve surgery, and possibly chemotherapy and radiation therapy.



Support Groups:

You can often help the stress of illness by joining a support group where members share common experiences and problems. See: Cancer - support group



Expectations (prognosis):

The outlook depends on how far the tumor has spread, and whether some areas of the tumor contain more aggressive cancer cells.



Complications:

Calling your health care provider:

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



Prevention:



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.

Zage PE, Ater JL. Neuroblastoma. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 492.




Review Date: 2/7/2012
Reviewed By: Linda J. Vorvick, MD, Medical Director and Director of Didactic Curriculum, MEDEX Northwest Division of Physician Assistant Studies, Department of Family Medicine, UW Medicine, School of Medicine, University of Washington; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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