泌尿生殖系统血管周上皮样细胞瘤(PEComa)的临床特征分析

doi:10.16150/j.1671-2870.2023.05.007

摘要/Abstract

摘要：

目的：探讨泌尿生殖系统血管周上皮样细胞瘤（perivascular epithelioid cell tumor, PEComa）的影像学表现及病理和免疫组织化学（免疫组化）特征，以提高临床对该病的认识。方法：回顾性分析经手术病理证实的5例泌尿生殖系统PEComa患者的临床、病理及影像学资料。结果：年龄为 9~63 岁，平均年龄为 40.8 岁；男性 3 例，女性 2 例，男：女为3:2。5例患者中，4例病变位于肾脏，1例病变位于阴道；病灶直径为0.9~19.0 cm。2例均行CT平扫及增强、MRI平扫及增强检查，3例仅行CT平扫及增强检查。影像学检查结果中，2例患者的CT平扫图像可见较小病灶,大小分别为0.9 cm×0.9 cm×1.0 cm、2.1 cm×1.7 cm×2.0 cm，密度均匀，增强扫描呈均匀明显强化；MRI平扫信号均匀，增强扫描呈均匀强化。另3例患者的病灶较大，最大径约19 cm，CT平扫提示病灶密度不均匀；CT增强扫描较大病灶则呈不均匀明显强化，可见液化坏死区，病灶内或周围出现粗大血管影。术后大体标本显示肿瘤切面呈灰白、灰红色，可见有出血，质韧，有包膜。镜下可以见肿瘤形态为上皮样、梭形，胞浆丰富，嗜酸性、颗粒状，空泡状，其核仁明显，核分裂象少，其内可见大片坏死。免疫组化检查示，PEComa组织中黑色素瘤抗体[Melanoma marker (human) monoclonal antibody, HMB45]呈阳性表达，Melan-A、肌源性抗体（Myogenic antibody, SMA）、CD34和S-100呈部分阳性表达。结论：泌尿生殖系统PEComa的影像学表现有一定特点，病灶较小（最大径<2 cm）时在CT图像密度均匀，CT增强呈均匀明显强化，强化程度低于正常肾实质；在MRI图像上信号均匀，MRI增强呈均匀明显强化。病灶较大时，在CT图像上密度不均，可出现液化坏死区，CT增强扫描呈不均匀强化，实性部分强化明显，可见粗大血管供血，内部坏死区未见明显强化。

关键词: 泌尿生殖系统, 血管周上皮样细胞肿瘤, 体层摄影术, 螺旋计算机, 磁共振成像

Abstract:

Objective: To investigate the imaging features and clinicopathological features of perivascular epithelioid cell tumor (PEComa) of the genitourinary system, and to improve the understanding of PEComa. Methods: The clinical, pathological and imaging data of 5 patients with PEComa confirmed by surgical pathology were retrospectively analyzed. Results: The age of the five patients ranged from 9 to 63 years, with a mean age of 40.8 years Among the 5 patients, 4 lesions were located in the kidney and 1 lesion in the vagina. The diameter of the lesion is 0.9~19 cm. Postoperative gross specimens showed that section of the tumor was gray and gry-red, with bleeding, toughness and envelope. Microscopically, the tumor was epithelioid and fusiform, with abundant cytoplasm, eosinophilic, granular, vacular, obvious nucleolus, few mitotic images, and large necrosis. CT plain scan and enhanced examination and MRI plain scan and enhanced examination were performed in 2 cases, only CT plain scan and enhanced examination in 3 cases. Homogeneous enhancement was observed in 2 cases, and uneven and obvious enhancement was observed in 3 cases of larger lesions, with liquefaction necrotic area. There are large vascular shadows in or around the lesion. Immunohistochemistry showed positive expression of HMB45, and partial positive expression of MELAN-A, SMA CD-31, CD34 and S-100. Conclusions: The imaging findings of perivascular epithelioid cell tumor of genitourinary system have certain characteristics, The CT image density is uniform and the enhancement is uniform and obvious, and the enhancement degree is lower than with of normal renal parenchyma. MRI image signal is uniform, the enhancement is uniform and obvious. When the lesion is large, the CT image density is uneven, liquefaction necrotic area may appear, the enhancement scan shows uneven enhancement, the solid part of the enhancement is obvious, and the blood supply of large vessels is visible, and the internal necrotic area does not show obvious enhancement.

Key words: Urogenital system, Perivascular epithelioid cell tumor, Computed tomography, Magnetic resonance imaging

中图分类号:

R737
R445.4

冯丽, 任刚, 蔡嵘, 汪心韵, 王辉, 祝明洁. 泌尿生殖系统血管周上皮样细胞瘤(PEComa)的临床特征分析[J]. 诊断学理论与实践, 2023, 22(05): 460-465.

FENG Li, REN Gang, CAI Rong, WANG Xinyun, WANG Hui, ZHU Mingjie. Clinical features study of perivascular epithelioid cell tumor (PEComa) in genitourinary system[J]. Journal of Diagnostics Concepts & Practice, 2023, 22(05): 460-465.

图/表 5

表1

图1

图2

图3

表2

参考文献 8

[1]	PEA M, BONETTI F, ZAMBONI G, et al. Clear cell tumor and angiomyolipoma[J]. Am J Surg Pathol, 1991, 15(2):199-202. pmid: 2025321
[2]	BONETTI F, PEA M, MARTIGNONI G, et al. Clear cell("sugar") tumor of the lung is a lesion strictly related to angiomyolipoma--the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC)[J]. Pathology, 1994, 26(3):230-236. doi: 10.1080/00313029400169561 URL
[3]	MARTIGNONI G, PEA M, ZAMPINI C, et al. PEComas of the kidney and of the genitourinary tract[J]. Semin Diagn Pathol, 2015, 32(2):140-159. doi: 10.1053/j.semdp.2015.02.006 pmid: 25804448
[4]	ZAMBONI G, PEA M, MARTIGNONI G, et al. Clear cell "sugar" tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells[J]. Am J Surg Pathol, 1996, 20(6):722-730. doi: 10.1097/00000478-199606000-00010 pmid: 8651352
[5]	FOLPE A L, GOODMAN Z D, ISHAK K G, et al. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults[J]. Am J Surg Pathol, 2000, 24(9):1239-1246. doi: 10.1097/00000478-200009000-00007 pmid: 10976698
[6]	THWAY K, FISHER C. PEComa: morphology and gene-tics of a complex tumor family[J]. Ann Diagn Pathol, 2015, 19(5)359-368. doi: 10.1016/j.anndiagpath.2015.06.003 URL
[7]	MARTIGNONI G, PEA M, REGHELLIN D, et al. PEComas: the past, the present and the future[J]. Virchows Arch, 2008, 452(2):119-132. doi: 10.1007/s00428-007-0509-1 pmid: 18080139
[8]	吴小鹏, 陈静, 张力峰, 等. 肾血管周上皮样细胞肿瘤一例报告[J]. 中华泌尿外科杂志, 2013, 34(8):607.
	WU X P, CHEN J, ZHANG L F, et al. Renal perivascular epithelioid cell tumor a case report[J]. Chin J Urol, 2013, 34(8)607.

Serial numb-er	Gen-der	Age （years old）	Symptom	NSE	Location	Shape	Volume（mm）	Density	Necrosis	Enhancement type	Enhanced degree	Small blood vessels travel after enhanc-ement	Renal venous cancer thromb-us	Distant metastasis of tumor
No.1	male	sixty	No symptoms	high	Right kidney	Round	9×7×8	Homog-eneous	-	Fast in and out	Obvious	-	-	-
No.2	male	nine	Abdominal mass	high	Left kidney	Irregular	150×160×190	Heterog-eneous	+	Fast in and slow out	Medium	+	-	-
No.3	fema-le	sixty-one	No symptom	Norm-al	Right kidney	Oval	100×161×190	Heterog-eneous	-	Fast in and slow out	Medium	-	-	-
No.4	male	sixty-three	Ventosity	Norm-al	Left kidney	Irregular	50×60×25	Heterog-eneous	+	Fast in and slow out	Medium	+	-	-
No.5	fema-le	nine	Vaginal fluid	high	Vagina	Round	21×17×25	Homog-eneous	-	Enhancement appearances	Obvious	-	-	-

Serial number	HMB45	MelanA	SMA	CD31	CD34	S-100
NO.1	+	-	-	-	+	-
NO.2	+	+	-	-	-	+
NO.3	+	-	+	-	-	-
NO.4	+	-	-	-	+	+
NO.5	+	-	-	-	-	-

[1]	李笑石, 秦越. 影像学技术在痛风诊断及疾病监测中的应用研究进展[J]. 诊断学理论与实践, 2023, 22(03): 311-318.
[2]	陈乾, 林慧敏, 严福华. 磁共振成像评估肝功能储备的研究进展[J]. 诊断学理论与实践, 2023, 22(02): 190-196.
[3]	李卫侠, 徐学勤, 朱晓雷, 陈克敏. 39例肾上腺皮质癌患者的CT、MRI影像特点及其诊断价值[J]. 诊断学理论与实践, 2023, 22(02): 134-140.
[4]	黄娟, 朱晓雷, 李晓, 陈克敏, 严福华, 徐学勤. 血氧水平依赖磁共振成像评估早期慢性肾病肾缺氧的研究[J]. 诊断学理论与实践, 2022, 21(03): 385-389.
[5]	朱乃懿, 姜奕歆, 柴丽, 柴维敏. 磁共振对超声阴性而乳腺X线检出BI-RADS4类以上钙化灶的诊断价值分析[J]. 诊断学理论与实践, 2021, 20(05): 439-444.
[6]	张雪坤, 李彦, 严福华, 赵洪飞, 宋琦. 基于光梭成像的新型加速技术在颅脑MRI中的应用价值研究[J]. 诊断学理论与实践, 2021, 20(04): 378-383.
[7]	孙甜甜, 叶宝英, 杨钰, 牛建梅. 彩色多普勒超声与磁共振成像在凶险型前置胎盘及合并胎盘植入产前诊断中的应用及漏诊分析[J]. 诊断学理论与实践, 2021, 20(02): 173-177.
[8]	吴霜, 解骞, 管雪妮, 张素芳, 高信芳, 梁宗辉. 磁共振体素内不相干运动扩散加权成像诊断活动期克罗恩病的价值及效能分析[J]. 诊断学理论与实践, 2020, 19(02): 157-161.
[9]	常蕊, 杨琰昭, 孔德艳, 徐嘉旭, 曹琪琪, 杨文洁, 严福华, 董海鹏. 不同管电压、管电流扫描方案联合KARL迭代重建在新型冠状病毒肺炎疫情期间胸部CT筛查中的应用研究[J]. 诊断学理论与实践, 2020, 19(02): 182-187.
[10]	曹烨, 刘晓晟, 葛晓乾, 周斌. 运用动态增强磁共振成像评估颈动脉粥样斑块稳定性的初步研究[J]. 诊断学理论与实践, 2019, 18(04): 436-441.
[11]	朱晓雷, 陈璐, 陆文丽, 刘燕, 严福华, 王伟, 董治亚. 474例中枢性性早熟女童不同年龄段垂体MRI影像学异常比例分析[J]. 诊断学理论与实践, 2019, 18(03): 286-290.
[12]	李云峰, 江泓, 李宁, 孙青芳. 核磁共振成像诊断三叉神经痛的价值分析与研究[J]. 诊断学理论与实践, 2018, 17(05): 562-565.
[13]	许晶晶, 张敏鸣. 人工智能机器学习方法在阿尔茨海默病中的应用现状[J]. 诊断学理论与实践, 2018, 17(04): 466-470.
[14]	赵华丽, 徐文鹏, 梁宗辉. 创伤性臂丛神经损伤的磁共振成像3D-FIESTA-C、IDEAL序列特征及诊断价值[J]. 诊断学理论与实践, 2018, 17(02): 197-201.
[15]	黎鑫乐, 柴维敏, 朱樱. 乳腺乳头状肿瘤的影像学诊断进展[J]. 诊断学理论与实践, 2018, 17(02): 225-230.