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肾上皮样血管平滑肌脂肪瘤:磁共振成像特征。

Renal epithelioid angiomyolipoma: magnetic resonance imaging characteristics.

机构信息

National Cancer Center/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China.

China Rehabilitation Research Center, Beijing, 100068, China.

出版信息

Abdom Radiol (NY). 2018 Oct;43(10):2756-2763. doi: 10.1007/s00261-018-1548-6.

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 × 10mm/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.

摘要

目的

本研究旨在分析肾脏上皮样血管平滑肌脂肪瘤(EAML)的磁共振成像(MRI)特征。

方法

本研究纳入了 17 例经组织病理学证实为肾脏 EAML 的患者,这些患者在接受根治性或部分肾切除术之前均进行了肾脏 MRI 扫描。回顾性分析 MRI 图像,并将其与病理结果相关联。

结果

15 个病灶(88.2%)呈圆形或椭圆形。14 个病灶(82.4%)的肿瘤-肾界面呈圆形。15 个肿瘤(88.2%)在 T1WI 上主要呈等信号,11 个肿瘤(64.7%)在 T2WI 上主要呈低信号。12 个病灶(70.6%)在 DWI 上表现为弥散受限,平均 ADC 值为 1.23±0.28×10mm/s。6 例(35.3%)在反相位 MR 图像上可见微小脂肪成分信号丢失。16 个病灶(100%)呈不均匀强化,16 个肿块中有 7 个(43.8%)呈网状强化。13 个肿块(81.3%)呈快速强化。皮质期 14 例(87.5%)肿块明显强化。4 例(23.5%)可见不规则血管,7 例(41.2%)可见出血。1 例(5.9%)患者初诊时淋巴结受累,术后发生远处转移。免疫组化分析显示,15 个肿瘤(88.2%)黑色素细胞标志物(HMB45 或 Melan-A)阳性,所有病例(100%)上皮相关标志物(CK 或 AE1/AE3)均阴性。

结论

T2WI 低信号、DWI 弥散受限、肿瘤-肾界面圆形、网状及明显强化(快速强化和快速廓清)应进一步提高对肾脏 EAML 的怀疑。通过病理分析可明确诊断。

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