Comparison of the clinical features and treatment outcomes of pilocytic astrocytoma in pediatric and adult patients

Kim, Joo Whan; Kim, Seung-Ki; Lee, Joo Ho; Park, Sung Sup; Won, Jae Kyung; Choi, Jung Yoon; Kang, Hyoung Jin; Park, Shin Young

doi:10.1007/s00381-023-05839-x

Cited by 6 publications

(3 citation statements)

References 28 publications

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“…T2-weighted MRI sequences can show isointense and hyperintense lesions containing features suggestive of calcification and mildly contrast-enhancing cysts of variable sizes [ 5 , 6 ]. Gross surgical resection is curative in most patients [ 7 , 8 ]. Although others indicate that PA may show aggressive behavior macroscopically, PAs typically appear as well-circumscribed, cyst-like tumors with a discrete mural nodule [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Calcified Pilocytic Astrocytomas and Calcifying Pseudoneoplasms of the Neuraxis: A Diagnostic Challenge

Hernández-Reséndiz,

Villanueva-Castro,

Mateo-Nouel

et al. 2024

Cureus

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Pilocytic astrocytoma (PA), recognized as the most prevalent central nervous system (CNS) tumor, has long been associated with calcifications, a characteristic often attributed to benign or indolent growth patterns. In this study, we explored the calcified attributes in these tumors that beckon a deeper understanding. This is a retrospective study, on a set of seven cases, with a histopathological diagnosis of pilocytic astrocytoma with calcifications and psammoma bodies (PB). Despite an encouraging overall survival outcome, the recurrence in four cases cast some doubt on the conventional classification. The histological study of these cases revealed a spectrum of calcifications, varying in size and morphology, all of which exhibited positive reactivity to glial fibrillary acidic protein (GFAP), osteoconduction, and osteopontin. Notably, the immunohistochemistry showed hyaline bodies displaying an atypical immune profile, strikingly negative for vimentin and GFAP, and a robust positivity for epidermal growth factor receptors (EGFR), tumor necrosis factor-alpha (TNF-α), and interleukin 1 beta (IL-1β). These results stimulated speculation that the identity of these calcified tumors may have extended and potentially embraced the realm of calcifying pseudoneoplasms of the neuraxis (CAPNON), underscored by intense pilot gliosis. This study transcends mere anatomical exploration; it delves into the intricacies of calcified tumors, casting a spotlight on the dynamic interplay between PA and CAPNON. As we traverse the frontiers of neuro-oncology, these findings pave the way for innovative avenues in the diagnostics and therapeutics of these tumors.

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Section: Introductionmentioning

confidence: 99%

Calcified Pilocytic Astrocytomas and Calcifying Pseudoneoplasms of the Neuraxis: A Diagnostic Challenge

Hernández-Reséndiz,

Villanueva-Castro,

Mateo-Nouel

et al. 2024

Cureus

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“…B-Raf aberrant kinase is utilized for targeted treatment in the BRAF-mutated PA forms: inhibitors such as vemurafenib and dabrafenib have shown promise in preclinical studies and clinical trials on PA [7,8]. The study by Kim et al (2023), comparing clinical features and treatment outcomes in pediatric and adult patients with PA, offers valuable insights into age-related differences [9]. However, some childhood PAs exhibit clinical variability, recurrence, and poor progression-free survival.…”

Section: Introductionmentioning

confidence: 99%

Proteomic Profiling of Cerebrospinal Fluid and Its Extracellular Vesicles from Extraventricular Drainage in Pediatric Pilocytic Astrocytoma, towards Precision Oncology

Spinelli,

Kajana,

Garbarino

et al. 2024

Cancers

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Pediatric pilocytic astrocytoma (PA) is the most common brain tumor in children. Complete resection provides a favorable prognosis, except for unresectable PA forms. There is an incomplete understanding of the molecular and cellular pathogenesis of PA. Potential biomarkers for PA patients, especially the non-BRAF-mutated ones are needed. Cerebrospinal fluid (CSF) is a valuable source of brain tumor biomarkers. Extracellular vesicles (EVs), circulating in CSF, express valuable disease targets. These can be isolated from CSF from waste extraventricular drainage (EVD). We analyzed the proteome of EVD CSF from PA, congenital hydrocephalus (CH, non-tumor control), or medulloblastoma (MB, unrelated tumoral control) patients. A total of 3072 proteins were identified, 47.1%, 65.6%, and 86.2% of which were expressed in the unprocessed total and in its large-EV (LEV), and small-EV (SEV) fractions. Bioinformatics identified 50 statistically significant proteins in the comparison between PA and HC, and PA and MB patients, in the same fractions. Kinase enrichment analysis predicted five enriched kinases involved in signaling. Among these, only Cyclin-dependent kinase 2 (CDK2) kinase was overexpressed in PA samples. PLS-DA highlighted the inactive carboxypeptidase-like protein X2 (CPXM2) and aquaporin-4 (AQP4) as statistically significant in all the comparisons, with CPXM2 being overexpressed (validated by ELISA and Western blot) and AQP4 downregulated in PA. These proteins were considered the most promising potential biomarkers for discriminating among pilocytic astrocytoma and unrelated tumoral (MB) or non-tumoral conditions in all the fractions examined, and are proposed to be prospectively validated in the plasma for translational medicine applications.

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“…Several molecular profiling studies have been conducted that aimed to stratify PA patients based on large-scale gene expression and/or DNA methylation profiling data [ 1 , 18 , 20 , 38 , 48 ]. In particular, studies have analyzed epigenetic data to define molecular groups [ 20 , 38 ].…”

Section: Introductionmentioning

confidence: 99%

Integrative multi-omics reveals two biologically distinct groups of pilocytic astrocytoma

Picard,

Felsberg,

Langini

et al. 2023

Acta Neuropathol

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Pilocytic astrocytoma (PA), the most common pediatric brain tumor, is driven by aberrant mitogen-activated protein kinase signaling most commonly caused by BRAF gene fusions or activating mutations. While 5-year overall survival rates exceed 95%, tumor recurrence or progression constitutes a major clinical challenge in incompletely resected tumors. Here, we used similarity network fusion (SNF) analysis in an integrative multi-omics approach employing RNA transcriptomic and mass spectrometry-based proteomic profiling to molecularly characterize PA tissue samples from 62 patients. Thereby, we uncovered that PAs segregated into two molecularly distinct groups, namely, Group 1 and Group 2, which were validated in three non-overlapping cohorts. Patients with Group 1 tumors were significantly younger and showed worse progression-free survival compared to patients with group 2 tumors. Ingenuity pathways analysis (IPA) and gene set enrichment analysis (GSEA) revealed that Group 1 tumors were enriched for immune response pathways, such as interferon signaling, while Group 2 tumors showed enrichment for action potential and neurotransmitter signaling pathways. Analysis of immune cell-related gene signatures showed an enrichment of infiltrating T Cells in Group 1 versus Group 2 tumors. Taken together, integrative multi-omics of PA identified biologically distinct and prognostically relevant tumor groups that may improve risk stratification of this single pathway driven tumor type.

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Comparison of the clinical features and treatment outcomes of pilocytic astrocytoma in pediatric and adult patients

Cited by 6 publications

References 28 publications

Calcified Pilocytic Astrocytomas and Calcifying Pseudoneoplasms of the Neuraxis: A Diagnostic Challenge

Calcified Pilocytic Astrocytomas and Calcifying Pseudoneoplasms of the Neuraxis: A Diagnostic Challenge

Proteomic Profiling of Cerebrospinal Fluid and Its Extracellular Vesicles from Extraventricular Drainage in Pediatric Pilocytic Astrocytoma, towards Precision Oncology

Integrative multi-omics reveals two biologically distinct groups of pilocytic astrocytoma

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