5-aminolevulinic acid-guided surgery for focal pediatric brainstem gliomas: A preliminary study

Abstract

Background: There is a growing body of literature supporting the use of 5-aminolevulinic acid (5-ALA) in the pediatric population, however, its use is still considered “off label” in this setting. In this retrospective study, we report our experience using 5-ALA in pediatric patients with focal brainstem gliomas (BSGs). Methods: Patients younger than 16 years presenting with a newly diagnosed BSG that was focal in nature were considered suitable for treatment with 5-ALA-assisted surgery. Exclusion criteria included MRI features suggestive of a diffuse intrinsic pontine glioma. A single dose of 5-ALA was administered preoperatively. Intraoperative fluorescence was recorded as “solid,” “vague,” or “none.” The effectiveness of the fluorescence was graded as “helpful” or “unhelpful.” Results: Eight patients underwent 5-ALA-assisted surgery. There were four tumors located in the pons, two midbrain tumors, and two cervicomedullary tumors. Histological analysis demonstrated three diffuse astrocytomas, three pilocytic astrocytomas, and two anaplastic astrocytomas. Solid fluorescence was found in three of the eight cases, vague fluorescence was found in two cases, and no fluorescence was found in three cases. Fluorescence was useful in 3 (37%) cases. No patients experienced any complications attributable to the administration of the 5-ALA. Conclusion: With a total fluorescence rate of 62.5% but a subjectively assessed “usefulness” rate of only 37.5%, the role of 5-ALA in BSG surgery is limited. Given the toxicological safety, however, of the agent, caution is perhaps needed before dismissing the use of 5-ALA entirely.

Comprehensive metabolomics study on the pathogenesis of anaplastic astrocytoma via UPLC-Q/TOF-MS

“…[55] Porphyrin metabolism perturbed with changes in several metabolites was also observed in AA in this study. Emerging evidence shows that protoporphyrin IX, [56,57] as an intermediate compound of the heme-biosynthesis cascade, can produce fluorescence and is used as a standard for surgical resection of glioma, [58] since it accumulates within the tumor tissues. [59] Bilirubin has been demonstrated to activate endoplasmic reticulum stress-induced autophagy of neuronal cells, which is a critical method of apoptosis of tumor cells.…”

5-ALA fluorescence in randomly selected pediatric brain tumors assessed by spectroscopy and surgical microscope

“…In contrast, Labuschagne recently reported two series of 23 fluorescing infratentorial (cerebellar and brain stem) tumors out of 27 [23,24]. Among these 16 displayed "strong" fluorescence and fluorescence was considered helpful in 15 of the 27 operations (56%).…”

Cervicomedullary Gliomas in Pediatric Age: A Systematic Review of the Literature and Tertiary Care Center Experience

“…The first description of a CMG was reported by Epstein et al [11], although a group of brainstem gliomas characterized by a more benign and indolent course had already been identified (by Hoffmann et al [2]). Reviewing the literature, a quite different number of articles were published in the two periods, each spanning about 20 years: 11 publications in the years 1981-2000 [4,7,[12][13][14][15][16][17][18][19][20], and 19 in 2001-2021 [8][9][10][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] (Tables 1, 2). The total number of cases described in the various studies was 127.…”