Renal epithelioid angiomyolipoma: computed tomography manifestation and radiologic-pathologic correlation depending on different epithelioid component percentages

Abstract

Background: Renal epithelioid angiomyolipoma (EAML) is a rare and potentially malignant mesenchymal lesion mainly composed of epithelioid cells. Although some case reports or small case series have been published, the computed tomography (CT) manifestations and radiologic-pathologic correlation depending on different epithelioid component percentages have not been studied before.

Objective: To investigate the CT manifestation and radiologic-pathologic correlation between renal EAML and angiomyolipoma (AML) with epithelioid component.

Methods: The clinicopathologic and imaging data of 53 patients with an original diagnosis of EAML or AML with epithelioid component were retrospectively collected from three hospitals. All tissue specimens were re-sectioned and re-observed under the microscope. Samples were divided into an EAML group (≥ 80% epithelioid component, n = 25) and AML with epithelioid component group (5% ≤ epithelioid component < 80%, n = 28). Two radiologists reviewed the images in consensus, describing and comparing the CT manifestation, including the long diameter of the tumor, morphology, presence of necrosis or cystic change, hemorrhage, fat, calcification, enlarged blood vessels, and dynamic enhancement pattern according to the Hounsfield unit value of each CT phase between the two groups. The radiologic-pathologic correlation depending on the different percentages of epithelioid component were studied.

Results: The long diameter of the tumor, presence of necrosis or cystic change, fat, enhancement pattern, and tumor-to-cortex enhancement ratio of the cortical phase between the two groups were significantly different (z = - 2.932, P = 0.003; χ² = 18.020, P < 0.001; χ² = 16.377, P < 0.001; P = 0.020; and T = - 3.944, P < 0.001, respectively). In multivariate logistic regression analysis, the significant predictive factors of EAML included the presence of necrosis or cystic change [odds ratio (OR) 11.864, P = 0.001] and absence of fat (OR 0.095, P = 0.003). Correlation analysis found that the presence of necrosis or cystic change (r = 0.679, P < 0.001) and fat (r = - 0.603, P < 0.001) were both moderately related to the epithelioid component percentage. The combined model based on the presence of necrosis or cystic change and absence of fat yielded the best diagnostic performance in discriminating EAML and AML with epithelioid component with the highest area under the curve (0.887).

Conclusion: EAML has characteristic CT signs; these characteristic CT signs are closely related to the epithelioid component percentage. The presence of necrosis or cystic change and the absence of fat were independent predictors of EAML.

Keywords: Angiomyolipoma (AML) with epithelioid component; Renal epithelioid angiomyolipoma (EAML); Tomography; X-ray computer.