Desmoplastic infantile ganglioglioma: A questionably benign tumour

Abstract

Desmoplastic infantile ganglioglioma is a rare intracranial tumour of childhood that involves the cerebral cortex and the leptomeninges. We report two patients with desmoplastic infantile gangliogliomas and multiple cerebrospinal metastases. To our knowledge, only two similar cases have been reported in the published literature. Pathologically, this rare intracranial tumour shows glial and ganglionic differentiation, accompanied by an extreme desmoplastic reaction. These are low-grade neoplasms that are questionably benign.

Novel suprasellar location of desmoplastic infantile astrocytoma and ganglioglioma: a single institution’s experience

“…2–4,8,13,23,36,39 The tumor nodules tend to be homogeneously enhancing, hypodense on T2-weighted imaging, and are not associated with significant vasogenic edema. 3 Although there are several studies demonstrating multifocal disease, progression, metastasis, and/or malignant transformation to glioblastoma, 7,8,16,21,24,32,37 these more aggressive forms of DIA/DIGs tend to be exceptions rather than the rule. 17 Generally, DIA/DIGs are considered benign and are often cured by resection alone.…”

“…14,39 Classically, patients with DIA/DIG present with rapidly enlarging head circumference. 17,32,34,36 On MRI, DIA/DIGs are typically described as large supratentorial cysts with a cortical-based enhancing tumor nodule with a dural attachment. 1,5,34 Resection is curative, and chemotherapy and/or radiation therapy is unnecessary in the majority of cases.…”

“…1,3,26,34,35 Indeed, DIA/DIGs are now categorized together in the 2016 WHO classification, are designated grade I, and generally have a good prognosis with long disease-free survival. 1,5,15,26,32,34,35,39 How- ever, there have been a few case reports of DIA/DIGs with atypical presentations, including primary vermian localization, 1 primary spinal localization, 25 metastatic spread, 32 multiple cerebral lesions, 1,18,28,32 and even occurrence in patients older than 6 years. 9,25,26 Based on these reports, patients with atypical presentations of DIA/DIGs may have markedly different clinical courses and long-term outcomes than patients with more classic presentations.…”

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.…”