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子宫血管周上皮样细胞肿瘤的临床病理分析
作者:杨雅洁  刘霞  孙艳花  许美权 
单位:深圳市第二人民医院 病理科, 广东 深圳 518035
关键词:子宫 血管周上皮样细胞肿瘤 病理学 
分类号:R737.33
出版年·卷·期(页码):2019·47·第五期(596-599)
摘要:

目的:探讨子宫血管周上皮样细胞肿瘤(PEComa)的临床病理学特征、治疗及预后。方法:分析2例子宫血管周上皮样细胞肿瘤的临床特点、病理形态及免疫组化表现,并结合相关文献进行复习。结果:患者均为老年女性,肿瘤均位于宫体,光镜下肿瘤细胞呈上皮样或梭形,围绕血管生长,免疫组化HMB45(+)、Melan-A(-)、SMA(+),随访至今无复发或远处转移。结论:子宫血管周上皮样细胞肿瘤是一种少见的间叶性肿瘤,具有比较特征性的组织学形态及免疫组化特点,目前首选治疗为全子宫+双附件切除,肿瘤生物学行为不明确,应当定期随访。

Objective:To analyze the clinicopathologic features,treatments and prognosis of perivascular epithelioid cell tumor (PEComa). Methods:Two cases of uterine PEComa were investigated of clinical characteristics,histopathology and immunohistochemistry. Related literatures were reviewed. Results:Two patients were elderly women, the tumors were located in the uterine body. The tumor cells were epithelioid or fusiform under light microscope and grew around the blood vessels. Immunohistochemical HMB45 (+), Melan-A (-), SMA (+), follow up showed no recurrence or distant metastasis. Conclusion:Uterine PEComa is a rare mesenchymal tumor with characteristic histopathological and immunohistochemical features. The preferred treatment is uterectomy and bilateral salping-oophenrectomy. It should be followed-up for its unclear biological behavior.

参考文献:

[1] BONETTI F,PEA M,MARTIGNONI G,et al.PEC and sugar[J]. Am J Surg Pathol,1992,16(3):307-308.
[2] ZAMBONI G,PEA M,MARTIGNONI G,et al. Clear cell "sugar" tumor of the pancreas. A novel member of the famil of lesions characterized by the presence of perivascular epithelioid cells[J]. Am J Surg Pathol,1996,20(6):722-730.
[3] VANG R,KEMPSON R L. Perivascular epithelioid cell tumor(‘PEComa’) of the uterus[J].Am J Surg Pathol,2002,26(1):1-13.
[4] JEON I S,LEE S M.Multimodal treatment using surgery,radiotherapy,and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus[J].J Pediatr Hemotol Oncol,2005,27(12):681-684.
[5] CELIK H,KEFELI M,CETINKAYA M,et al.Perivascular epithelioid cell tumor(PEComa)of the uterine cervix in a patient with tuberous sclerosis complex:a literature review[J].Turk Patoloji Dergisi,2014,11:1-5.
[6] FUKUNAGA M. Perivascular epithelioid cell tumor of the uterus:report of four cases[J].Int J Gynecol Pathol,2005,24:341-346.
[7] FOLPE A L,MENTZEL T,LEHR H A,et a1.Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin:a clinicopathologic study of 26 cases and review of the literature[J].Am J Surg Pathol,2005,29(12):1558-1575.
[8] PAN C C,JONG Y J,CHAI C Y,et al.Comparative genomic hybridization study of perivascular epithelioid cell tumor:molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm[J].Hum Patho1,2006,37(5):606-612.
[9] SCHOOLMEESTER J K,DAO L N,SUKOV W R,et al.TFE3 translocation associated perivascular epithelioid cell neoplasm(PEComa)of the gynecologic tract:morphology,immunophenotype,differential diagnosis[J].Am J Surg Pathol,2015,39(3):394-404.
[10] 刘飞飞,饶秋,张仁亚,等.伴有TFE3扩增的宫颈血管周上皮样细胞肿瘤[J].临床与实验病理学杂志,2013,29(12):1361-1363.
[11] BLEEKER J S,QUEVEDO J F,FOLPE A L."Malignant" perivascular epithelioid cell neoplasm:risk stratification and treatment strategies[J].Sarcoma,2012,2012(2):1-12.
[12] VANG R,KEMPSON RL.Perivascular epithelioid cell tumor(‘PEComa’)of the uterus:a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors[J].Am J Surg Pathol,2002,26(1):1-13.
[13] BUNCH K,SUNDE J.Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor:a case report[J].Gynecologic Oncology Case Reports,2014,8:14-16.
[14] STARBUCK K D,DRAKE R D,BUDD G T,et al.Treatment of advanced malignant uterine perivascular epithelioid cell tumor with mTOR inhibitors:single-institution experience and review of the literature[J].Anticancer Res,2016,36(11):6161-6164.
[15] 伍健,李媛,贺玉洁.子宫体恶性血管周上皮细胞肿瘤1例并文献复习[J].临床与实验病理学杂志,2012,28(3):333-336.
[16] 牟田,王建六.子宫血管周上皮样细胞肿瘤二例及文献复习[J].中国妇产科临床杂志,2010,11(1):62-64.
[17] 崔恒. 子宫血管周上皮样细胞肿瘤[J].中华妇产科杂志,2011,46(4):298-300.
[18] 胡旦红,刘芳,于亚威. 恶性子宫血管周上皮样细胞肿瘤1例[J].实用妇产科杂志,2012,28(5):375-376.
[19] 李荣岗,陈兰花,黄秀芳.子宫颈恶性血管周上皮样细胞肿瘤二例[J].中华病理学杂志,2014,43(9):631-632.
[20] 唐灿,粟占三,刘保安,等. 子宫及阔韧带血管周上皮样细胞肿瘤6例临床病理分析[J].诊断病理学杂志,2014,21(5):286-289.
[21] 宫国良,戎锡云,陈静宁,等.子宫恶性血管周上皮样细胞肿瘤3例临床病理观察[J].诊断病理学杂志,2015,22(2):74-77.
[22] 张爱兵,李俊. 子宫恶性具有血管周上皮样细胞分化的肿瘤1例病理分析[J].华北理工大学学报(医学版),2016,18(6):451-453.

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