Epithelioid renal angiomyolipoma: Clinical characteristics, treatment strategies, and oncological outcomes-An individual patient data meta-analysis of 259 cases.

Epithelioid angiomyolipoma (eAML) is a rare malignant renal neoplasm distinct from benign classic angiomyolipoma. Evidence-based management recommendations are lacking due to its rarity. This systematic review aimed to analyze clinical characteristics, treatment strategies, and oncological outcomes. We systematically searched MEDLINE through December 2025 (PROSPERO: CRD42024548957), identifying case reports and series reporting individual patient data. Extracted data included demographics, clinical presentation, radiological and histopathological features, treatment modalities, and survival outcomes. Univariable logistic and Cox regression analyses identified factors associated with recurrence and eAML-related mortality. Analysis included 259 patients from 137 studies (59% female, median age 43 years [IQR 33-54]). Most patients (56%) presented symptomatically with abdominal pain (42%) or macrohematuria (13%). Radical nephrectomy was performed in 71%, partial nephrectomy in 16%. At median follow-up of 21 months (IQR 8-54), recurrence occurred in 39% at median 12 months (IQR 6-36), predominantly as distant metastases (liver 14% and lung 10%). Five-year overall survival, cancer-specific survival, and recurrence-free survival were 84%, 85%, and 55%, respectively. Significant risk factors for recurrence included positive hilar lymph nodes (OR 15.6), vascular invasion (OR 6.4), necrosis (OR 10.5), and high mitotic index (OR 7.2), all P < 0.001. Limitations include publication bias and heterogeneous reporting. eAML exhibits aggressive behavior with 39% recurrence and 11% disease-related mortality. Complete surgical resection remains the cornerstone of initial management and structured cross-sectional imaging follow-up should be considered given high recurrence risk. For recurrent disease, mTOR inhibitors, particularly everolimus, represent effective first-line systemic therapy. High-risk histopathological features may guide future risk-stratified follow-up strategies.