Pleomorphic Xanthoastrocytoma Treatment [Neurosurgery Wiki]

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====== Pleomorphic Xanthoastrocytoma Treatment ======

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Standard Treatment Approach
1. Surgical Resection
Goal: Maximal safe resection.

Gross total resection (GTR) is associated with significantly improved prognosis.

In cases where GTR is not feasible (e.g., due to proximity to eloquent brain areas), subtotal resection is performed.

2. Histological and Molecular Analysis
Confirm diagnosis with neuropathology.

Test for BRAF V600E mutation – present in ~60-70% of PXAs.

Evaluate for anaplastic features: high mitotic activity, necrosis, and/or high Ki-67 index. These suggest Anaplastic PXA (WHO Grade III), which has a more aggressive course.

3. Adjuvant Therapy (case-dependent)
For WHO Grade II PXA after GTR:

Often no additional therapy is needed initially.

Close surveillance with MRI every 3–6 months for the first 2 years, then annually.

For subtotal resection or recurrence:

Radiotherapy may be considered.

Chemotherapy options include temozolomide, especially if there is progression or recurrence.

For Anaplastic PXA or progressive/recurrent disease:

Adjuvant radiotherapy and/or chemotherapy are often recommended.

Targeted therapy with BRAF inhibitors (e.g., dabrafenib, vemurafenib) ± MEK inhibitors (e.g., trametinib) has shown promising results in BRAF-mutant cases.

4. Clinical Trials
Consider for recurrent or anaplastic PXA, particularly those that are BRAF wild-type or resistant to standard therapies.

Follow-Up
Long-term imaging surveillance is critical due to risk of recurrence.

Neurological exams and neurocognitive assessments may be part of regular follow-up, especially in pediatric cases.
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The discovery of [[BRAF]] mutations within a substantial percentage of [[Pleomorphic Xanthoastrocytoma]] fosters a clearer understanding of the pathophysiology of these tumors with clear prognostic and therapeutic implications. These findings are expected to provide insight into the spectrum of clinical behavior observed in PXA, ranging from cure with surgery to diffuse dissemination throughout the neuraxis
((Shaikh N, Brahmbhatt N, Kruser TJ, Kam KL, Appin CL, Wadhwani N, Chandler J, Kumthekar P, Lukas RV. Pleomorphic xanthoastrocytoma: a brief review. CNS Oncol. 2019 Nov 1;8(3):CNS39. doi: 10.2217/cns-2019-0009. Epub 2019 Sep 19. PMID: 31535562; PMCID: PMC6880293.)).

===== Surgery =====

Conflicting reports exist on the importance of extent of resection (EOR)

===== Radiotherapy =====

[[Pleomorphic Xanthoastrocytoma Radiotherapy]].