Renal epithelioid angiomyolipoma: magnetic resonance imaging characteristics

Abstract

Objective: The aim of the study was to analyze MR imaging features of renal epithelioid angiomyolipoma (EAML).

Methods: This study included 17 patients with histopathologically confirmed renal EAML who underwent renal MRI scanning before radical or partial nephrectomy. MR images were retrospectively reviewed and correlated with pathological findings.

Result: Fifteen lesions (88.2%) appeared as round or oval. The tumor-kidney interface was round in 14 lesions (82.4%). Fifteen tumors (88.2%) presented mainly isointensity on T1WI, and eleven tumors (64.7%) presented mainly hypointensity on T2WI. Twelve lesions (70.6%) showed restricted diffusion on DWI, and the mean ADC value was 1.23 ± 0.28 × 10^-3mm²/s. Minimal fat component was identified as loss of signal intensity on opposed-phase MR images in 6 cases (35.3%). Sixteen lesions (100%) demonstrated inhomogeneous enhancement, and 7 of 16 masses (43.8%) showed reticular enhancement. Rapid wash-in and wash-out enhancement was seen in 13 masses (81.3%). In the corticomedullary phase, the mass showed markedly enhancement in 14 cases (87.5%). The irregular vessels and hemorrhage were detected in 4 cases (23.5%) and 7 cases (41.2%), respectively. One patient (5.9%) had a lymph node involvement at initial diagnosis, and showed distant metastasis after operation. In the immunohistochemical analysis, 15 tumors (88.2%) were positive for melanocytic marker (HMB45 or Melan-A), and all cases (100%) were negative for epithelial-associated markers (CK or AE1/AE3).

Conclusion: The presence of hypointensity on T2WI, restricted diffusion on DWI, round tumor-kidney interface, reticular, and marked enhancement (rapid wash-in and wash-out) should further raise suspicion for renal EAML. The diagnosis may be confirmed by pathological analysis.

Keywords: Angiomyolipoma; Epithelioid angiomyolipoma; Magnetic resonance imaging; Perivascular epithelioid cell neoplasms; Renal neoplasms.