Ganglioneuroblastoma

Definition 
Ganglioneuroblastoma is a tumor arising from nerve tissue which is intermediate 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 has a yearly occurrence of less than 5 per million children.

Tumors of the nervous system vary in the degree of differentiation, which determines how they 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 widespread, but 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.

Signs and tests 

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

Treatment 

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

Depending on the specific nature of the tumor, treatment may 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.

Expectations (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.

Complications 

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

Calling your health care provider
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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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