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

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.

Exams 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

Outlook (Prognosis)

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

Possible Complications

When to Contact a Medical Professional

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.

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.
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