Surgical Management and Outcome of a Bilateral Thalamic Pilocytic Astrocytoma: Case Report and Review of the Literature

Konar, Subhas; Shukla, Dhaval; Nandeesh, Bevinahalli N; Prabhuraj, A R; Devi, Bhagavatula Indira

doi:10.1159/000495990

Cited by 1 publication

(1 citation statement)

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“…In fact, the complete resection has been considered the curative procedure for PA. In cystic lesions, the resection of only the nodule is recommended, but not the cyst wall ( 48 ); nevertheless, an increased thickness of the cystic wall suggests en-bloc removal of the neoplastic lesion ( 48 , 49 ). In detail, for subtentorial localisation of PA, the lesion should be treated through a telovelar suboccipital approach.…”

Section: Treatment and Prognosismentioning

confidence: 99%

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

Pizzimenti,

Fiorentino,

Germanò

et al. 2024

Oncol Lett

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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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Section: Treatment and Prognosismentioning

confidence: 99%