Ganglioneuroblastoma - Symptoms,Causes
Ganglioneuroblastoma is an intermediate tumor arising from 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 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 ganglioneuromaganglioneuroma 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 scanMIBG 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.
Update Date: Updated 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.
Ganglioneuroblastoma - Symptoms,Causes
