Desmoplastic infantile ganglioglioma with calcification

Abstract

Desmoplastic infantile gangliogliomas (DIGs) are rare intracranial, cystic tumors, usually detected within the first 2 years of life. Histologically, these tumors are characterized by intense desmoplasia and a divergent astrocytic and neuronal differentiation. Less than 60 well-documented cases of this extremely rare tumor are reported in the literature. We present the case of a 10-month-old male child presenting with a large, cystic, intracranial lesion and having the characteristic histological features of a DIG. In addition, the lesion also showed focal areas of calcification within the tumor, not usually considered a feature of this entity. The present case extends the reported spectrum of this rare entity and describes the unusual occurrence of calcification within the lesion.

Atypical cystic meningioma overexpressing AQP1 in early infancy: case report with literature review

“…DIGs are rare intracranial supratentorial cystic tumours usually occurring in the first 2 years of life, characterized by desmoplasia and different neuronal and astrocytic differentiation. So far, less than 60 well‐documented cases are reported in the literature (20), also with atypical features, such as calcifications (20) or high Ki‐67 proliferation index (21). DIGs are classified as benign WHO grade I tumours of infancy, generally associated with epilepsy and with good prognosis, with reports of recurrence‐free intervals of up to 14 years (22).…”

Congenital Brain Tumors

“…115 Recently, a case with a prenatal diagnosis of DIG has also been reported. 114 They account for 0.1 to 1% of intracranial tumors in children 110,112,116,117 while representing approximately 16% of all infantile intracranial tumors 118,119 with a slight male predominance. 111,120 The most common initial symptom is macrocephaly secondary to hydrocephalus (40%), followed by seizures (20%), bulging fontanels, bony bossing, visual disturbance (setting-sun eyes), and paresis.…”

“…111,120 The most common initial symptom is macrocephaly secondary to hydrocephalus (40%), followed by seizures (20%), bulging fontanels, bony bossing, visual disturbance (setting-sun eyes), and paresis. [114][115][116][117] DITs mainly affect the frontal and parietal 10,20 or even temporal lobes, but in more than half of cases they involve multiple lobes. 20,112,121 DITs appear as large hemispheric uni-or multilocular cystic masses of deep localization, with a small solid mural nodule at the periphery 10,20,122 reflecting the initial location of DIT in the subarachnoid space, with subsequent invasion of the brain parenchyma along the Virchow-Robin spaces.…”

Purely Cystic Desmoplastic Ganglioglioma: An Incidental Finding in a Giant Cyst

“…Now this tumor is recognized as a distinct entity and is included in the World Health Organization (WHO) classification under the category of neural and mixed glioneuronal tumors, and is in the grade I of the WHO classification. 3 Until 2013, approximately 70 cases of DIG reported in literature. 4 We are presenting a 12-month-old male infant, with developmental delay and a giant cyst on the right side of supratentorial compartment, which showed histologic features of desmoplastic ganglioglioma.…”