Molecular characterization of disseminated pilocytic astrocytomas

Gessi, Marco; Engels, Alexander; Lambert, Sally R.; Rothämel, Thomas; Hornstein, Stephan von; Collins, V. Peter; Denkhaus, Dorota; Gnekow, Astrid; Pietsch, Torsten

doi:10.1111/nan.12256

Cited by 8 publications

(5 citation statements)

References 12 publications

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“…5 Similar results have been found in disseminated LGG. 6 In our series, the one patient who had testing for the V600E mutation was negative. The BRAF V600E mutation is targetable with vemurafenib, which has been successfully used in pilomyxoid astrocytoma, suggesting that targeting of the downstream MAPK pathway may be a viable option going forward.…”

Section: Discussionmentioning

confidence: 60%

Long-term outcomes of primarily metastatic juvenile pilocytic astrocytoma in children

Fisher

Cheshier²,

Edwards³

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVEPrimarily metastatic juvenile pilocytic astrocytoma (JPA) is rare, likely representing 2%–3% of all cases of JPA. Due to the rarity of primarily metastatic JPA, there is currently no standard treatment paradigm and the long-term outcomes are not fully known. The goal of this case series was to add to the current understanding of this disease process.METHODSThe authors searched a comprehensive database of pediatric patients with brain and spinal cord tumors treated at Lucile Packard Children’s Hospital from 1997 to 2016 and identified 5 patients with primarily metastatic JPA. A retrospective chart review was performed and details of the patients’ treatment and clinical course were recorded for further analysis.RESULTSFor the 5 patients with primarily metastatic JPA, the mean follow-up period was 12.3 years. All patients in our series had biopsies or subtotal resections and upfront treatment. Three patients were treated with chemotherapy alone, one was treated with chemotherapy and radiotherapy, and one was treated with radiotherapy alone. Four patients had stable disease after initial treatment, and one patient had multiple episodes of progressive disease but underwent successful salvage therapy and has had stable disease for 19 years. One patient died of an intracerebral hemorrhage 10 years following initial radiation treatment believed to be secondary to radiation vasculopathy.CONCLUSIONSEvaluation of the entire neuraxis should be performed in all instances of initial JPA diagnosis to properly assess for primarily metastatic disease. Many patients with primarily metastatic JPA will have stable disease after upfront treatment, although the higher rate of stable disease found in this series relative to other reports is likely secondary to the small sample size.

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Section: Discussionmentioning

confidence: 60%

Long-term outcomes of primarily metastatic juvenile pilocytic astrocytoma in children

Fisher

Cheshier²,

Edwards³

et al. 2018

Journal of Neurosurgery: Pediatrics

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“…Circumscribed astrocytoma (CA) arises in the central nervous system 23 . This grade of astrocytoma often affects adolescents and young people and can develop anywhere in the central nervous system, specifically the cerebellum 24 .…”

Section: Classification Of Astrocytomamentioning

confidence: 99%

“…Circumscribed astrocytoma (CA) arises in the central nervous system. 23 This grade of astrocytoma often affects adolescents and young people and can develop anywhere in the central nervous system, specifically the cerebellum. 24 According to the grade I of the World Health Organization, it can also happen in the optic nerve and brain stem.…”

Section: Classification Of Astrocytomamentioning

confidence: 99%

Unraveling the signaling mechanism behind astrocytoma and possible therapeutics strategies: A comprehensive review

Nahar Metu,

Sutihar,

Sohel

et al. 2023

Cancer Reports

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BackgroundA form of cancer called astrocytoma can develop in the brain or spinal cord and sometimes causes death. A detailed overview of the precise signaling cascade underlying astrocytoma formation has not yet been revealed, although various factors have been investigated. Therefore, our objective was to unravel and summarize our current understanding of molecular genetics and associated signaling pathways with some possible therapeutic strategies for astrocytoma.Recent FindingsIn general, four different forms of astrocytoma have been identified in individuals, including circumscribed, diffuse, anaplastic, and multiforme glioblastoma, according to a recent literature review. All types of astrocytoma have a direct connection with some oncogenic signaling cascade. Common signaling is MAPK cascade, including Ras‐Raf‐ERK, up‐regulated with activating EGFR/AKT/PTEN/mTOR and PDGFR. Recent breakthrough studies found that BRAF mutations, including KIAA1549: BRAF and BRAF V600E are responsible for astrocytoma progression. Additionally, cancer progression is influenced by mutations in some tumor suppressor genes, such as the Tp53/ATRX and MGMT mutant. As synthetic medications must cross the blood–brain barrier (BBB), modulating signal systems such as miRNA is the primary option for treating patients with astrocytoma. However, available surgery, radiation therapy, and experimental therapies such as adjuvant therapy, anti‐angiogenic therapy, and EGFR‐targeting antibody drug are the usual treatment for most types of astrocytoma. Similar to conventional anticancer medications, some phytochemicals slow tumor growth by simultaneously controlling several cellular proteins, including those involved in cell cycle regulation, apoptosis, metastatic spread, tyrosine kinase, growth factor receptor, and antioxidant‐related proteins.ConclusionIn conclusion, cellular and molecular signaling is directly associated with the development of astrocytoma, and a combination of conventional and alternative therapies can improve the malignancy of cancer patients.

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“…Several studies found that clinical outcome is independent of BRAF status (59,64,65), whereas others have found that fusion is an independent prognostic marker for significantly improved 5-year progression-free survival (58). One study has reported that disseminated PAs possess genetic features similar to those of classic PA, with a similar incidence of KIAA1549-BRAF fusions and BRAF mutations (66).…”

Section: Molecular and Genetic Characteristicsmentioning

confidence: 99%

Recent Progress in the Pathology and Genetics of Pilocytic and Pilomyxoid Astrocytomas

Ding¹,

Tihan²

2019

Balkan Med J

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Pilocytic and Pilomyxoid Astrocytomas constitute one of the most common gliomas in children and young adults, and are considered indolent neoplasms with long overall survival probability. The genetic characteristics of these neoplasms have been well characterized, and the deeper understanding of molecular alterations has led to novel treatment strategies and approaches. Currently, we can account for some of the unusual behavior of these neoplasms such as oncogene-induced senescence and associated spontaneous regression, anaplastic transformation and cerebrospinal dissemination. However, there are still enigmatic issues with these tumors that are often considered "chronic diseases". We review the classical and less common clinical pathological and genetic features of these indolent gliomas.

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Molecular characterization of disseminated pilocytic astrocytomas

Cited by 8 publications

References 12 publications

Long-term outcomes of primarily metastatic juvenile pilocytic astrocytoma in children

Long-term outcomes of primarily metastatic juvenile pilocytic astrocytoma in children

Unraveling the signaling mechanism behind astrocytoma and possible therapeutics strategies: A comprehensive review

Recent Progress in the Pathology and Genetics of Pilocytic and Pilomyxoid Astrocytomas

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