Awake Surgery for Angiocentric Glioma in the Eloquent Area in an Adolescent: A Case Report

Abstract

Angiocentric glioma (AG) is an extremely rare tumor that often develops in adolescents. Awake surgery for AG occurring in the eloquent area has not been reported to date. We report a case involving a right-handed 15-year-old boy with AG. He presented with a first-time generalized tonic-clonic seizure and was rushed to the local hospital. CT of the head indicated a left frontal low-density mass with no calcification. He was subsequently referred to our hospital. Comparison with a CT scan obtained two years prior due to mild head trauma indicated that the lesion showed a trend toward enlargement. The lesion was located in the anterior and lateral portions of the primary motor cortex, and MRI showed homogenous hypointensity on T1-weighted and hyperintensity on T2-weighted images. Contrast-enhanced MRI showed a linear contrast effect. The patient underwent awake surgery with successful intraoperative brain mapping and total resection, and brain function was preserved. Pathological analysis revealed AG. He returned to his normal life and has shown no recurrence without additional treatment for 2 years. Thus, awake surgery for complete tumor resection while preserving brain function is effective and safe even in adolescents with AGs.

Left-Parietal Angiocentric Glioma: Our Experience and a Review of the Literature

“…Although the literature agrees that this tumor does not enhance after gadolinium administration, in some studies, a slight enhancement with rather mixed signals has been reported [4,8,[19][20][21]28,33,35]. Inside the lesion, it is also possible to observe areas of cystic degeneration [51] (as in our case) and/or calcifications [39,41]. Magnetic resonance spectroscopy also demonstrates increased choline/phosphocreatine and choline/N-acetyl ratios, as well as increased levels of glycine and myoinositol [56].…”

“…Biopsy was performed in eight cases in total, four being in the brainstem and one being in the thalamus. In four cases, with a follow-up between 5 and 24 months, no recurrence or progression was described [31,35,39,53]. In one case, after the biopsy, the patient made a full recovery and a month later there was no worsening [9].…”

“…The above-mentioned characteristics must therefore direct us to a differential diagnosis that is equally compatible with lowgrade astrocytomas, ependymomas, DNETs, and cortical malformations [25,31,57]. A particular criterion has been described by some authors who report the presence, in MRI, of "stalk-like white matter abnormality perpendicular to the ventricular wall", low in T1WI and high in T2WI [1,39]. Our case also presents a peculiarity, already mentioned by Pokharel et al [38], which reinforces the hypothesis of a long time interval between the tumor genesis and the onset of symptoms: a thinning of the internal cortex of the bone overlying the lesion, a sign of long-lasting compression during its development.…”

“…The author concludes by saying that the spinal lesion, confirmed as an angiocentric glioma, is the possible metastatic evolution of an IDH-wildtype astrocytoma (WHO grade II, ATRX-wildtype, 1p19q intact) of the right-frontal lesion, operated 10 months earlier with stereotactic biopsy and then treated with post-operative radiotherapy (54 Gy in 30 fractions). Typically, AG is characterized by a low-intensity signal in T1WI scans, or rarely as a hyperintense signal in T1WI [16,35,39,41,47], or more finely as intrinsic "rim-like" hyperintensity [1]. It always behaves as hyperintense on T2WI and FLAIR scans.…”

“…According to histology, AGs are cellular tumors made up of monomorphic spindle- Typically, AG is characterized by a low-intensity signal in T1WI scans, or rarely as a hyperintense signal in T1WI [16,35,39,41,47], or more finely as intrinsic "rim-like" hyper-intensity [1]. It always behaves as hyperintense on T2WI and FLAIR scans.…”