Malignant ganglioglioma: case report and review of literature

Abstract

We report a case of de novo malignant ganglioglioma. A 61-year-old male presented with a 12-day history of headaches and general malaise. Pre-operative magnetic resonance imaging revealed an irregular enhancing mass in the left temporal lobe with associated dural enhancement and subacute subdural hematoma. The findings at surgery were of a vascular tumor intimately involving the surrounding white matter, with central necrosis. Histological and immunohistochemical studies showed an anaplastic ganglioglioma with World Health Organization grade IV characteristics. Gangliogliomas of the central nervous system are rare and are typified by a pediatric predilection and indolent behavior. A review of the de novo anaplastic and malignant gangliogliomas is presented.

Pediatric Primary Anaplastic Ganglioglioma: A Case Report and Review of the Literature

“…The diagnosis can be confirmed with the use of immunohistochemical markers: the glial component is positive for glial fibrillary acidic protein, S-100 [9] and vimentin [5], whereas the presence of the neuronal component can be confirmed by immunoreactivity to synaptophysin, neurofilament protein, neuron-specific enolase [9] and chromogranin A [15]. Although the neurons may be positive for CD34, a stem-cell epitope not expressed in a normal brain, the reactivity to this neuronal stem-cell marker is less common in AGGs than in GGs [3,7].…”

“…Although the pure forms are more frequent, some variants have been described, such as GG with associated anaplastic oligodendroglioma; combinations with meningioma or sarcoma may also occur [5]. …”

“…Less than 30 cases of primary pure AGGs have been described in the scientific literature [5,12,15,16]. The age range of the patients was 6 months to 77 years, with only 19 cases diagnosed at a pediatric age (i.e.…”

“…On imaging, AGGs generally show irregular contrast enhancement and are characterized by mixed solid and cystic portions [5,15]. On MRI, the lesions appear isointense or hypointense on T1-weighted images and hyperintense on T2-weighted images.…”

“…The aggressive subtype is called anaplastic ganglioglioma (AGG). AGGs account for 1–8% [3,4,5] of GGs. These tumors are generally located in the supratentorial compartment, without a strong predilection for the temporal lobe [6,7], but they can be found anywhere throughout the central nervous system.…”