Primary Intracranial Alveolar Soft Part Sarcoma: Systematic Review.

Background and Objective: Alveolar soft part sarcoma is a rare malignancy of unknown histiogenesis. Rarely, primary intracranial alveolar soft part sarcoma has been reported. To review the clinical manifestations, progression and management of this neoplasm was the prime objective of this systematic review. Methodology: A Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant review of PubMed Central, Google Scholar, and ScienceDirect identified 18 primary intracranial alveolar soft-part sarcoma cases (eight reports, three series; 2000-2024). Case reports and series providing data on clinical presentation, radiological features, surgical management, histopathological findings, and patient outcomes were included. Results: Patients were predominantly female 61.1% (11), with a mean age of 23.3 ± 14.2 years. Headache in 50% (9) and papilledema in 33.3% (6) were common presentations. Frontal lobe involvement was most common in 33.3% (6). Magnetic resonance imaging showed iso+hypointense T1 and iso+hyperintense T2 signals in 22.2% (4) each. Meningioma was the leading differential in 27.8% (5). Gross total resection was achieved in 77.8% (14). Transcription Factor E3 immunopositivity occurred in 55.6% (10) of tumors. Average follow-up was 19.9 months. Conclusion: Surgical resection remains the primary treatment. Transcription Factor E3 immunoreactivity supports diagnosis. Occult extracranial disease should be excluded. Long-term monitoring is essential due to the indolent course of primary intracranial alveolar soft-part sarcoma.