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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Desmoplastic infantile ganglioglioma


Other Names for this Disease
  • DIG
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Overview



What is desmoplastic infantile gangliomas (DIGs)?

What signs and symptoms are associated with desmoplastic infantile gangliomas?

What treatment is available for desmoplastic infantile gangliomas?


What is desmoplastic infantile gangliomas (DIGs)?

Desmoplastic infantile gangliomas (DIGs) are rare brain tumors that are normally located in the frontal or parietal lobes of the brain. They are usually diagnosed before 18 months of age with most infants presenting with a short duration of symptoms. Although seizures are not commonly observed, a bulging fontanelle, rapid head growth, vomiting, and a sunset sign are usually noted. The standard treatment for DIGs is surgical resection (surgical procedure in which the portion of the brain with the tumor is removed).[1]
Last updated: 7/30/2013

What signs and symptoms are associated with desmoplastic infantile gangliomas?

Most infants with DIGs do not have seizures; however, they usually have a bulging fontanelle, rapid head growth, sunset sign, and vomiting.[1]
Last updated: 7/30/2013

What treatment is available for desmoplastic infantile gangliomas?

Surgical resection (removal of the area of the brain with the tumor) has been the standard treatment reported in the medical literature. The size of the resection is probably based on the size of the tumor, although the extent of the resection is not documented for all cases reported in the medical literature. Adjuvant therapy is generally not performed when a gross total resection can be performed. When total resection is not possible, some of suggested chemotherapy, as the effects of radiation on extremely young children may be harmful.[1]
Last updated: 7/30/2013

References
  1. Smith SH. Uncommon Pediatric Brain Tumors. In: Raghavan D, Brecher ML, Johnson DH, Meropol NJ, Moots PL, Rose PG. Textbook of Uncommon Cancer. 3rd ed. England: John Wiley & Sons Ltd; 2006;