A Four-Year Follow-up Study of Renal Epithelioid Angiomyolipoma: A Multi-Center Experience and Literature Review

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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.

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