Posterior Cranial Fossa Gangliogliomas

Abstract

Ganglioglioma (GG) is an uncommon primary lesion of the central nervous system that is typically located supratentorially. There are only a few reports of GG arising from the cerebellum. To the best of our knowledge this is the first case of a cerebellar GG with supratentorial extension and a longstanding history before its recognition. In fact, this 29-year-old male presented with an 11-year history of intermittent headaches. A cranial computerized tomography (CT) performed at the onset of his complaints failed to reveal the tumor. After a particularly longstanding cephalalgic episode, the patient underwent a new CT scan that was also negative. However, magnetic resonance (MR) imaging of the brain revealed a space-occupying lesion in the right cerebellar hemisphere with extension to the level of the superior colliculi and pineal recess. The tumor was partially removed through a midline suboccipital craniotomy and supracerebellar approach. Pathological examination of the tumor showed composition of atypical ganglion cells and astrocytes, indicating the diagnosis of cerebellar GG. At last follow-up, 24 months after surgery, the patient reported a marked improvement of his clinical condition with significant reduction of intensity and frequency of the headache. The present report illustrates how cerebellar GG may remain undetectable by CT and may therefore present with a longstanding history and nonspecific signs and symptoms. MR investigation can lead to the proper diagnosis. Even after partial removal the prognosis remains good and remission of the symptoms may be achieved. In this article, we review the literature and summarize the current understanding of infratentorial GGs.

Uncommon glial tumors

“…Alternatively, some gangliogliomas do not demonstrate gadolinium enhancement on MRI (Im et al 2002a). Less common locations in the optic nerve, chiasm and tract (Bergin et al 1988;Chilton et al 1990;Sugiyama et al 1992), hypothalamus (Liu et al 1996), trigeminal nerve (Athale et al 1999), brainstem (Garcia et al 1984;Davidson et al 1992), cerebellum (Mizuno et al 1987;Safavi-Abbasi et al 2007), thalamus (Johnson et al 2001), pineal gland (Hunt & Johnson 1989Johnson et al 1995) and spinal cord (Johannsson et al 1981) have been reported. In one report, for example, MRI was able to demonstrate leptomeningeal and subarachnoid spread along the middle cerebral artery and right Sylvian fissure in a patient with leptomeningeal metastasis (Tien et al 1992).…”

Hemorrhagic Ganglioglioma of the Posterior Fossa: Case Report

“…However, the enhancement pattern of the ganglioglioma may mimic a vascular malformation, and catheter angiography is required to exclude a vascular malformation. Intraoperatively, these tumors have in fact been found to be extremely vascular [35,37]. In this case, the numerous telangiectatic vessels (resembling an angioma) seen bordering the hematoma may have been responsible for the hemorrhage.…”

“…Interestingly, epilepsy of cerebellar origin has also been reported in patients with cerebellar gangliogliomas [3,12,18,19,20]. In general, cerebellar gangliogliomas are rare, with only 30 cases being reported in the literature [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,31,32,33,34], and they usually present with a short history (mean 1.6 years) [35]. To our knowledge, this is the first documented case of a cerebellar ganglioglioma presenting with hemorrhage.…”

Atypical Presentation of a Pediatric Cerebellar Ganglioglioma

“…Calcification and cystic components are inconsistently present. Histologically, GGs are characterized by both ganglion and glial cell components, with the latter determining how aggressive the tumor behaves [19] . These elements stain positive for the neural markers chromogranin, synaptophysin, and glial fibrillary acidic protein.…”