Desmoplastic infantile ganglioglioma/astrocytoma

Desmoplastic infantile gangliogliomas are a form of desmoplastic infantile tumours, and despite their aggressive appearances tend to have a good prognosis. 

Epidemiology

The vast majority occur in children with less than one year of age, and males are more commonly affected with a M:F ratio of approximately 2:1 ¹.

Clinical presentation

A rapidly increasing head circumference is the most common presentation, with symptoms usually presenting in a short time (5 days to 3 months) ¹.

Seizure activity is uncommon.

Radiographic features

Desmoplastic infantile ganglioglioma manifests as an exceptionally large cerebral hemispheric mass composed of both cystic and solid portions. The frontal and parietal lobes are the most common sites.

CT

The solid portion of these large masses is typically slightly hyperattenuating and usually located along the cortical margin of the mass. Following administration of contrast, these masses usually enhance intensely, and may demonstrate a dural tail. 

Calcification is not a feature of desmoplastic infantile ganglioglioma.

MRI

The solid portions typically have the following signal intensity:

• T1: isointense to brain parenchyma
• T2: isointense to brain parenchyma
• T1 C+ (Gd)
  • intense enhancement
  • a dural tail may be seen

Treatment and prognosis

Surgical resection is the treatment of choice; however, because of the large size of these lesions and the firm attachment to the dura, complete resection is difficult.

In cases of partial resection, adjunctive chemotherapy may be considered and have been reported to produced some reduction in tumour volume ¹. 

Overall prognosis is good for most patients.