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Clinicopathological characteristics and prognosis of central nervous system solitary fibrous tumor: An analysis of 271 cases.

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PMID42155214
JournalPathology, research and practice
Publication Date2026-05-12
Ingested2026-08-02 12:06 AM
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BACKGROUND: Intracranial solitary fibrous tumors (ISFTs) are rare central nervous system (CNS) mesenchymal neoplasms with heterogeneous clinical behavior. Using the 2021 WHO CNS classification, we aimed to explore the clinicopathological features and prognostic factors of ISFTs to optimize personalized management. METHODS: We retrospectively analyzed 271 primary ISFT patients treated between 2007 and 2024. Kaplan-Meier analysis was used to evaluate survival outcomes. Schoenfeld residual tests validated the proportional hazards (PH) assumption, and model-specific stratified Cox regression was applied to identify independent prognostic factors. RESULTS: The cohort had a median age of 48 years, with a male-to-female ratio of 1.316:1. After 2021 WHO reclassification, 48.34% were grade 1, 35.42% grade 2, and 16.24% grade 3. Gross total resection (GTR) was achieved in 79.34% of patients, and 62.36% received postoperative radiotherapy (PORT), predominantly in high-grade or STR cases. During a median 42-month follow-up, 49.08% developed recurrence, and 9.96% developed distant metastasis. PH testing revealed model-specific violations: surgical extent violated the assumption in the OS model, while 2021 WHO grade violated it in the PFS model. Stratified regression confirmed high WHO grade and STR as poor prognostic factors. Subgroup analysis showed PORT significantly improved PFS in high-risk patients (grade 2/3 tumors) but conferred no benefit in low-risk grade 1 patients. CD34 negativity was associated with worse survival in univariate analysis but not in multivariate analysis. CONCLUSION: The 2021 WHO grading system provides excellent risk stratification for ISFTs. GTR is the primary treatment, while PORT only benefits high-risk patients, supporting a risk-adapted treatment strategy for ISFT patients.

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Clinicopathological characteristics and prognosis of central nervous system solitary fibrous tumor: An analysis of 271 cases.

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