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《天津医科大学学报》[ISSN:1006-8147/CN:12-1259/R]

卷:

26卷

期数:

2020年02期

页码:

166-170,187

栏目:

临床医学

出版日期:

2020-04-30

文章信息/Info

Title:

Clinical features of renal epithelioid angiomyofipoma

文章编号:

1006-8147(2020)02-0171-04

作者:

方辉龙; 杨元兴; 李黎明

（天津医科大学总医院泌尿外科，天津 300052）

Author(s):

FANG Hui-long;  YANG Yuan-xing;  LI Li-ming

（Department of Urology, General Hospital of Tianjin Medical University, Tianjin 300052, China)

关键词:

肾脏上皮样血管平滑肌脂肪瘤; 病理; 治疗; 预后

Keywords:

renal epithelioid angiomyolipoma;  pathology;  treatment;  prognosis

分类号:

R692.9

DOI:

-

文献标志码:

A

摘要:

目的：探讨肾脏上皮样血管平滑肌脂肪瘤（EAML）的临床特点。方法:回顾性分析在2012年6月-2018年11月收治的10例EAML患者的临床资料，对其临床表现、影像特点、病理特征、治疗方法和预后进行分析。结果：10例患者中，女性9例，男性1例，平均年龄36岁，3例在体检时发现，7例有阳性体征。彩超可见囊实性或不均匀实性病灶；CT平扫呈低密度或稍高密度影，可见不同程度的强化；MRI在Ｔ2WI上表现为低信号，呈不均匀强化。8例行肾脏部分切除术，1例行根治性肾脏切除术，1例行肾脏切除术。术后病理8例诊断为EAML；２例病理诊断为肾脏血管平滑肌脂肪瘤（AML），部分呈EAML。免疫组化染色HMB-45（+）、Melan-A（+）、SMA（+）、CK（-）。术后随访时间7～50个月，平均随访时间28个月，暂未发现复发或远处转移。结论：EAML在临床上较少见，早期症状不明显，确定诊断依靠术后病理HE染色和免疫组化染色。EAML具有恶性潜能，治疗主要以手术切除为主，目前预后尚无统一认识，需要长时间密切随访。

Abstract:

Objective: To explore the clinical features of renal epithelioid angiomyolipoma（EAML）. Methods: The clinical data of 10 patients with EAML admitted from June 2012 to November 2018 were investigated, retrospectively. The clinical manifestations, imaging features, pathological features, treatment methods and prognosis were analyzed. Results: Among the 10 patients, 9 cases were female and 1 case was male, with an average age of 36 years. Three patients were found in physical examination, and 7 patients had positive signs. Cystic solid or heterogeneous lesions were scan by color ultrasound; CT plain scan showed low density or slightly high density shadow, and the CT enhancement scan showed different degrees enhancement; low signal and uneven enhancement showed on T2WI by MRI. Eight patients were performed partial nephrectomy, and 1 patient was treated with radical nephrectomy, and 1 patient was treated with nephrectomy. Postoperative pathology indicated that 8 patients were diagnosed as renal epithelioid angiomyolipoma, and 2 patients were diagnosed as renal angiomyolipoma which showed partial epithelioid angiomyolipoma features.Immunohistochemical staining results of the tumor showed that HMB-45（+）、Melan-A（+）、SMA（+）、CK（-）. Postoperative follow-up time was 7 to 50 months, with an average follow-up time of 28 months. No recurrence or distant metastasis cases were found. Conclusion: EAML is rare in clinical practice and early symptoms of which are not obvious. The definitive diagnosis of EAML depends on postoperative pathological HE staining and immunohistochemical staining. EAML has malignant potential, and the main treatment of which is surgical resection. There are no unified understanding of the prognosis until now, which requires long-term and close follow-up.

参考文献/References:

[1] Mai K T, Perkins D G, Collins J P. Epithelioid cell variant of renal angiomyolipoma[J]. Histopathology, 1996, 28(3): 277
[2] Lee W, Choi S Y, Lee C, et al. Does epithelioid angiomyolipoma have poorer prognosis, compared with classic angiomyolipoma?[J]. Investig Urol, 2018, 59(6): 357
[3] Faraji H, Nguyen B N, Mai K T. Renal epithelioid angiomyolipoma: a study of six cases and a meta-analytic study. Development of criteria for screening the entity with prognostic significance[J]. Histopathology, 2009, 55(5): 525
[4] Kato I, Inayama Y, Yamanaka S, et al. Epithelioid angiomyolipoma of the kidney[J]. Pathol Int, 2009, 59(1): 38
[5] Lee K H, Tsai H Y, Kao Y T, et al. Clinical behavior and management of three types of renal angiomyolipomas[J]. J Formos Med Assoc, 2019, 118(1 Pt 1): 162
[6] 富聪聪, 夏禹, 王传卓, 等. 肾上皮样血管平滑肌脂肪瘤的CT和超声表现与病理对照分析[J]. 中国医科大学学报, 2014, 43(8): 752
[7] 纪建松, 王祖飞, 赵中伟，等. 肾脏上皮样血管平滑肌脂肪瘤的CT诊断[J]. 中华放射学杂志, 2010, 44(3): 279
[8] Cui L, Zhang J G, Hu X Y, et al. C T imaging and histopathological features of renal epithelioid angiomyolipomas[J]. Clin Radiol, 2012, 67(12): e77
[9] 丁玉芹, 孙辉红, 何德明, 等. 肾脏上皮样血管平滑肌脂肪瘤的MDCT和MRI表现及其与病理的关系[J]. 放射学实践, 2012, 27(11): 1231
[10] Nese N, Martignoni G, Fletcher C D, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification[J]. Am J Surg Pathol, 2011, 35(2): 161
[11] Konosu-Fukaya S, Nakamura Y, Fujishima F, et al. Renal epithelioid angiomyolipoma with malignant features: Histological evaluation and novel immunohistochemical findings[J]. Pathol Int, 2014, 64(3): 133
[12] Li W, Guo L, Bi X, et al. Immunohistochemistry of p53 and Ki-67 and p53 mutation analysis in renal epithelioid angiomyolipoma[J]. Int J Clin Exp Pathol, 2015, 8(8): 9446
[13] Ooi S M, Vivian J B, Cohen R J. The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma[J]. Int Urol Nephrol, 2009, 41(3): 559
[14] Guo B, Song H, Yue J, et al. Malignant renal epithelioid angiomyolipoma: A case report and review of the literature[J]. Oncol Lett, 2016, 11(1): 95
[15] Espinosa M, Roldan-Romero J M, Duran I, et al. Advanced sporadic renal epithelioid angiomyolipoma: case report of an extraordinary response to sirolimus linked to TSC2 mutation[J]. BMC Cancer, 2018, 18(1): 561
[16] Shitara K, Yatabe Y, Mizota A, et al. Dramatic tumor response to everolimus for malignant epithelioid angiomyolipoma[J]. Jpn J Clin Oncol, 2011, 41(6): 814-816
[17] 石泓哲, 李长岭, 寿建忠，等. 肾上皮样血管平滑肌脂肪瘤的诊治[J]. 中国癌症杂志, 2013, 23(3): 207
[18] Brimo F, Robinson B, Guo C, et al. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy[J]. Am J Surg Pathol, 2010, 34(5): 715
[19] Zhan R, Li Y Q, Chen C Y, et al. Primary kidney malignant epithelioid angiomyolipoma: Two cases report and review of literature[J]. Medicine (Baltimore), 2018, 97(32): e11805
[20] Wen J, Li H Z, Ji Z G, et al. Renal epithelioid angiomyolipoma without obvious local progress in 10 years: a case report and literature review[J]. Ir J Med Sci, 2011, 180(2): 557
[21] Aydin H, Magi-Galluzzi C, Lane B R, et al. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association[J]. Am J Surg Pathol, 2009, 33(2): 289

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备注/Memo

备注/Memo:

作者简介 方辉龙（1987-），男，硕士在读，研究方向：泌尿外科学；
通信作者:李黎明，E-mail:13920420809@163.com。

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更新日期/Last Update: 2020-06-02