Desmoplastic Infantile Ganglioglioma

Elise's Story Journal Articles on DIG I wish I had known...

The Desmoplastic Infantile Ganglioglioma website has been created by Robin Britton to help people who have been affected by this very rare pediatric (childhood) cancer. If you are seeking support, please visit Robin on our Rare Cancer Forums.

What is Desmoplastic Infantile Ganglioglioma (DIG)?

Desmoplastic Infantile Ganglioglioma (DIG) is characterized by a large tumor which tends to be primarily on a single side of the brain. It was first described as a new type of rare tumor in 1987. This brain tumor occurs in children and is usually found in the first year of life. It is exceptionally rare and there is not much information in the literature about it. It was originally classified as a low grade, slow growing tumor that tends to be massive and benign. There is a movement in the current medical community to reclassify this tumor as malignant or fast growing.

It is a Mixed Type cancer which means there are different type of cells that make up the tumor mass. In the case of DIG there are three types of cells:

Cysts - fluid filled sacks
Hard nodule like type of cell
Generally soft tissue cancer cell

There are two other types of brain tumors that are similar to DIG, desmoplastic infantile astrocytoma, and pleomorphic xanthoastrocytoma. All of these tumors are thought to be embryonal, meaning they begin before the baby is even born.

Medical Description of Desmoplastic Infantile Ganglioglioma

From the first abstract describing desmoplastic infantile ganglioglioma, author Vandenburg (DIG research godfather):

Abstract: The tumors were characterized by their voluminous size, their intense desmoplasia, and the frequent presence of divergent astrocytic and ganglionic differentiation as demonstrated by special neurohistological and immunohisto- and immunocytochemical techniques. All the tumors presented in subjects below the age of 18 months, usually within the first 4 months of life. They most often involved the frontal and parietal regions and were composed predominantly of a dense desmoplastic tissue superficially resembling a moderately cellular fibroma. The fibroblastic elements were admixed with variable numbers of pleomorphic neuroepithelial cells. Divergent astrocytic and neuronal differentiation was demonstrable in nine of the 11 tumors. All showed astrocytic differentiation. The study of one example by electron microscopy, immunocytochemistry, and tissue culture disclosed that the astrocytic tumor cells were partly invested by a pericytoplasmic basal lamina. Successful total or near-total surgical resection has been followed by a favorable postoperative course extending in some cases over many years of tumor-free survival. The name "desmoplastic infantile ganglioglioma" is proposed for this apparently distinct clinicopathological entity, whose massive size is indicative of a pre- or perinatal origin. Its identification can be achieved by careful histological analysis and is of obvious prognostic significance.