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Ganglioneuroblastoma

Ganglioneuroblastoma is an intermediate tumor that arises from nerve tissues. 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

Ganglioneuroblastoma mostly occurs in children ages 2 to 4 years. The tumor affects boys and girls equally. It occurs rarely in adults. Tumors of the nervous system have different degrees of differentiation. This 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 less malignant in nature. 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 with tenderness. 

This tumor may also occur at other sites, including:

  • Chest cavity
  • Neck
  • Legs

Exams and Tests

The health care provider may do the following tests:

  • Fine-needle aspiration of the tumor
  • Bone marrow aspiration and biopsy
  • Bone scan
  • CT scan or MRI scan of the affected area
  • PET scan
  • Metaiodobenzylguanidine (MIBG) scan
  • Special blood and urine tests
  • Surgical biopsy to confirm diagnosis

Treatment

Depending on the type of tumor, treatment can involve surgery, and possibly chemotherapy and radiation therapy.

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

Support Groups

Organizations that provide support and additional information:

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

Complications that may result include:

When to Contact a Medical Professional

Call your 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

Asa SL, Fischer SE. Adrenal gland. In: Gattuso P, Reddy VB, David O, Spitz DJ, Haber MH, eds. Differential Diagnosis in Surgical Pathology. 3rd ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 9.

Myers JL. Mediastinum. In: Goldblum JR, Lamps LW, McKenney JK, Myers JL, eds. Rosai and Ackerman's Surgical Pathology. 11th ed. Philadelphia, PA: Elsevier; 2018:chap 12.

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Review Date: 10/21/2017

Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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