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Pilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review)

  • Authors:
    • Cristina Pizzimenti
    • Vincenzo Fiorentino
    • Antonino Germanò
    • Maurizio Martini
    • Antonio Ieni
    • Giovanni Tuccari
  • View Affiliations

  • Published online on: February 8, 2024     https://doi.org/10.3892/ol.2024.14279
  • Article Number: 146
  • Copyright: © Pizzimenti et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Among low‑grade gliomas, representing 10‑20% of all primary brain tumours, the paradigmatic entity is constituted by pilocytic astrocytoma (PA), considered a grade 1 tumour by the World Health Organization. Generally, this tumour requires surgical treatment with an infrequent progression towards malignant gliomas. The present review focuses on clinicopathological characteristics, and reports imaging, neurosurgical and molecular features using a multidisciplinary approach. Macroscopically, PA is a slow‑growing soft grey tissue, characteristically presenting in association with a cyst and forming a small mural nodule, typically located in the cerebellum, but sometimes occurring in the spinal cord, basal ganglia or cerebral hemisphere. Microscopically, it may appear as densely fibrillated areas composed of elongated pilocytic cells with bipolar ‘hairlike’ processes or densely fibrillated areas composed of elongated pilocytic cells with Rosenthal fibres alternating with loosely fibrillated areas with a varied degree of myxoid component. A wide range of molecular alterations have been encountered in PA, mostly affecting the MAPK signalling pathway. In detail, the most frequent alteration is a rearrangement of the BRAF gene, although other alterations include neurofibromatosis type‑1 mutations, BRAFV600E mutations, KRAS mutations, fibroblast growth factor receptor‑1 mutations of fusions, neurotrophic receptor tyrosine kinase family receptor tyrosine kinase fusions and RAF1 gene fusions. The gold standard of PA treatment is surgical excision with complete margin resection, achieving minimal neurological damage. Conventional radiotherapy is not required; the more appropriate treatment appears to be serial follow‑up. Chemotherapy should only be applied in younger children to avoid the risk of long‑term growth and developmental issues associated with radiation. Finally, if PA recurs, a new surgical approach should be performed. At present, novel therapy involving agents targeting MAPK signalling pathway dysregulation is in development, defining BRAF and MEK inhibitors as target therapeutical agents.
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April-2024
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Spandidos Publications style
Pizzimenti C, Fiorentino V, Germanò A, Martini M, Ieni A and Tuccari G: Pilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review). Oncol Lett 27: 146, 2024
APA
Pizzimenti, C., Fiorentino, V., Germanò, A., Martini, M., Ieni, A., & Tuccari, G. (2024). Pilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review). Oncology Letters, 27, 146. https://doi.org/10.3892/ol.2024.14279
MLA
Pizzimenti, C., Fiorentino, V., Germanò, A., Martini, M., Ieni, A., Tuccari, G."Pilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review)". Oncology Letters 27.4 (2024): 146.
Chicago
Pizzimenti, C., Fiorentino, V., Germanò, A., Martini, M., Ieni, A., Tuccari, G."Pilocytic astrocytoma: The paradigmatic entity in low‑grade gliomas (Review)". Oncology Letters 27, no. 4 (2024): 146. https://doi.org/10.3892/ol.2024.14279