Clinicopathological features and diagnostic challenges of hepatic epithelioid angiomyolipoma: a retrospective analysis of 27 cases

Background: Hepatic epithelioid angiomyolipoma, a rare mesenchymal tumor, poses significant diagnostic challenges due to its rarity and non-specific presentation. Existing literature is dominated by case reports, limiting a systematic understanding of its clinicopathological profile. Therefore, further in-depth research is of great significance for elucidating the nature and clinical features of this disease. Objective: To define the clinicopathological characteristics, diagnostic features, and management outcomes of Hepatic epithelioid angiomyolipoma by analyzing a single-center cohort. Methods: We retrospectively analyzed 27 patients diagnosed with hepatic epithelioid angiomyolipoma at a single institution between April 2019 and November 2024. We collected and examined data on clinical presentation, laboratory findings, imaging results from ultrasound, computed tomography, and magnetic resonance imaging, as well as histopathological and immunohistochemical features. Results: The cohort was predominantly female (88.9%; 24/27), with a mean age of 44.2 years, and most patients (81.5%; 22/27) were asymptomatic. Preoperative imaging frequently mimicked other liver tumors, resulting in a correct preoperative diagnosis in only 3.7% of cases. Pathologically, all tumors tested were positive for HMB-45 (100%) and Melan-A (100%). Surgical resection was the primary treatment (96.3%). Tissue biopsy combined with microwave ablation was performed in 3.7% (1/27) of the patients. 27 cases were diagnosed as typical hepatic epithelioid angiomyolipoma. Conclusion: Hepatic epithelioid angiomyolipoma presents with non-specific clinical and radiological features, making preoperative diagnosis challenging and often leading to misdiagnosis as other common liver tumors. Definitive diagnosis depends on histopathological and immunohistochemical analysis. Surgical resection remains the primary treatment due to the tumor's malignant potential. A high index of suspicion and reliance on pathological confirmation are crucial for accurate management of this rare tumor.