Abstract
In this study, we systematically explored the clinical manifestations, diagnosis, treatment, and prognosis of renal epithelioid angiomyolipoma (EAML) retrospectively by analyzing data of 52 patients diagnosed with EAML at four centers. Our results showed that the onset of EAML was usually inconspicuous, and so no obvious symptoms or signs had occurred in most patients at diagnosis. Its diagnoses always depended on postoperative pathological examination. The immunohistochemical (IHC) results [HMB45 ( + ), cytokeratin (-), and S100 (-)] could be used to differentiate EAML from other malignancies such as renal cell cancer (RCC) and sarcomas. For treatment, surgery resulted in satisfactory short-term prognosis. The long-term prognosis of patients with EAML was poor, particularly when a large size, a high percentage of epithelioid component, tumor thrombus formation, and necrosis were present. In conclusion, EAML is a tumor with malignant potential. Once diagnosed, integrated approaches, including surgery, chemotherapy, and targeted therapy, should be considered; a close follow-up regimen is necessary for cases that met: 1) tumor size >9 cm, 2) tumor thrombus formation in the vein, 3) epithelioid cells >70% or atypia cells >60%, and 4) necrosis.
Highlights
Renal angiomyolipoma (AML) is one of the most common renal benign tumors, and originates from mesenchymal tissue
The compare of pathologic features between progressors and non-progressors EAML was shown at Table 2
AML specimens from 43 patients were stratified into 3 groups based on the percentage of epithelioid/atypia epithelioid cells
Summary
Renal angiomyolipoma (AML) is one of the most common renal benign tumors, and originates from mesenchymal tissue. According to the classification of tumors by the WHO in 20041 there are two types of renal AML: classical and epithelioid. The former is a benign tumor that is composed of different proportions of mature abnormal thick-walled blood vessels, fusiform or epithelioid smooth muscle cells, and adipose tissue. EAML, or monotypic EAML, especially for those with atypia epithelioid cells[2] has malignant potential, and is mainly composed of a large number of hyperplastic epithelioid cells arranged in sheets; the proportion of mature fat cells tends to be
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