Cerebellar astrocytoma

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By Roger J Packer MD

Cerebellar astrocytoma is also known as or subsumes Classical juvenile pilocytic astrocytoma, Cystic cerebellar astrocytoma, Diffuse astrocytoma, Juvenile astrocytoma, Juvenile pilocytic astrocytoma, and Juvenile pilomyxoid astrocytoma. -ed.

Cerebellar astrocytomas are the most common benign brain tumor of childhood. Most tumors are pilocytic astrocytomas and are amenable to gross total resections. After such treatment, 95% or more of patients will be cured of disease. For those tumors that cannot be grossly totally resected, including occasional midline pilocytic astrocytomas and other variants of low-grade glioma, attempts at re-resection or, in infrequent situations, radiation therapy or chemotherapy may be indicated. New biological insights suggest that molecular-targeted therapy may be available soon to treat subtotally resected and/or progressive lesions. This clinical article updates the reader in the new concepts of the molecular pathogenesis of the tumor and the possible important role of BRAF fusion abnormalities in prognosis of subtotally resected pilocytic astrocytomas. The RAS intracellular signaling pathway is an intriguing target, especially utilizing agents that target BRAF and MEK. Increasing information concerning other molecular targets is included.

Key Points

  • Cerebellar astrocytomas are the most common form of childhood low-grade primary intracranial tumors.
  • After total resection, over 95% of children with cerebellar astrocytomas are cured.
  • Post-surgical adjuvant chemotherapy or radiotherapy is not needed for the vast majority of patients with cerebellar astrocytomas.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited