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静脉内平滑肌瘤病的临床病理

赵大春,  田庄,  梁智勇,  刘彤华

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文章导航 > 协和医学杂志  > 2012 >  3(1): 51-55
赵大春, 田庄, 梁智勇, 刘彤华. 静脉内平滑肌瘤病的临床病理[J]. 协和医学杂志, 2012, 3(1): 51-55. doi: 10.3969/j.issn.1674-9081.2012.01.012
引用本文: 赵大春, 田庄, 梁智勇, 刘彤华. 静脉内平滑肌瘤病的临床病理[J]. 协和医学杂志, 2012, 3(1): 51-55. doi: 10.3969/j.issn.1674-9081.2012.01.012
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DA-chun ZHAO, Zhuang TIAN, Zhi-yong LIANG, Tong-hua LIU. Clinicopathological Features of Intravenous Leiomyomatosis[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 51-55. doi: 10.3969/j.issn.1674-9081.2012.01.012
Citation: DA-chun ZHAO, Zhuang TIAN, Zhi-yong LIANG, Tong-hua LIU. Clinicopathological Features of Intravenous Leiomyomatosis[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 51-55. doi: 10.3969/j.issn.1674-9081.2012.01.012
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静脉内平滑肌瘤病的临床病理

doi: 10.3969/j.issn.1674-9081.2012.01.012
  • 1.

    中国医学科学院 北京协和医学院 北京协和医院 病理科, 北京 100730

  • 2.

    中国医学科学院 北京协和医学院 北京协和医院 心内科, 北京 100730

详细信息
    通讯作者:

    刘彤华 电话:010-65295523, E-mail:liuthp-umch@yahoo.com.cn

  • 中图分类号: R732.2+2;R446.8

Clinicopathological Features of Intravenous Leiomyomatosis

  • 1.

    Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Scienses & Peking Union Medical College, Beijing 100730, China

  • 2.

    Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Scienses & Peking Union Medical College, Beijing 100730, China

More Information

    摘要
  • 摘要:   目的  探讨静脉内平滑肌瘤病(intravenous leiomyomatosis, IVL)的临床病理学特征, 诊断特点及鉴别诊断。  方法  分析34例IVL患者的临床表现, 对其手术标本进行HE和免疫组织化学染色(EnVision)观察。  结果  患者年龄24~61岁, 平均年龄44.8岁。临床表现为下肢肿胀、腹胀、月经异常等盆腔相关症状, 累及下腔静脉及心脏患者可有呼吸困难、心悸、胸闷等心血管症状。病变大体表现:14例位于子宫内, 6例累及盆腔静脉, 5例累及下腔静脉, 9例生长入心房内; 镜下表现为梭行平滑肌细胞构成, 核分裂罕见; 免疫组化染色SMA、ER、PR、Desmin阳性, HMB45、CD10阴性, 4例CD34阳性, 余均为阴性, Ki-67阳性指数0.3%~2.7%。  结论  静脉内平滑肌瘤病为育龄期妇女少见疾病, 组织学表现良性但生物学行为恶性, 具有特定的大体表现和免疫组化特点。
    Abstract:   Objective  To investigate the clinicopathological features, immunophenotype, and differential diagnosis of intravenous leiomyomatosis (IVL).  Methods  Clinical manifestations, morphologic features, and immunohistochemical staining results from 34 patients with IVL were retrospectively analyzed.  Results  Patients were aged 24 to 61 years (mean 44.8 years), with the clinical manifestations included edema of low extremity, abdominal distention, hypermenorrhea, and other pelvic discomfort. In patients whose inferior vena cava and heart were also involved, the symptoms also included chest tightness, palpitation, and dyspnea. The morphologic changes were observed in uterine (n=14), pelvic veins (n=6), and inferior vena cava (IVC) (n=14); in 9 patients, tumors extended into right heart chamber through IVC. Microscopically, the tumors were composed of spindle-shaped cells with rare mitotic figure. The tumors were positive for SMA, ER, PR, and Desmin and negative for CD10 and HMB45. CD34 was positive in 4 patients and negative in the remaining patients. Ki-67 index ranged 0.3% -2.7%.  Conclusion  IVL is a rare disease among women of child-bearing age. It can be histologically begign but biologically malignant, with specific morphologic manifestations and immunohistochemical features.
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  • 图  1  子宫静脉内平滑肌瘤病, 肿瘤从宫旁血管中膨胀性生长, 挤压血管腔, 肿瘤中心黏液样变

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    图  2  下腔静脉内取出肿瘤, 呈条索样, 表面光滑, 局部呈结节状结构

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    图  3  梭形细胞构成的肿瘤在血管腔内生长, 可见肿瘤与血管壁间裂隙状分隔, 肿瘤细胞生长较均匀、致密(HE, ×150)

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    图  4  免疫组化染色示SMA弥漫阳性(×150)

    下载: 全尺寸图片 幻灯片

    图  5  免疫组化染色示CD10阴性(×150)

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    图  6  免疫组化染色示肿瘤旁血管内皮及肿瘤中新生小血管内皮CD34阳性(×150)

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    • 参考文献(12)
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    [3] Galajda Z, Copotoiu C, Suciu H, et al. The diagnosis, morphological particularities, and surgical technique in a case of intravascular leiomyoma extended to the right heart chambers[J]. J Vasc Surg, 2010, 51:1000-1002. doi:  10.1016/j.jvs.2009.09.061
    [4] Lam PM, Keith WK, Mei Yu, et al. Intravenous leiomyomatosis:two cases with different routes of tumor extension[J]. J Vasc Surg, 2004, 39:465-469. doi:  10.1016/j.jvs.2003.08.012
    [5] Wu CK, Luo JL, Yang CY, et al. Intravenous leiomyomatosis with intracardiac extension[J]. Intern, 2009, 48:997-1001.
    [6] 彭娴婧, 金征宇.静脉内平滑肌瘤病的临床表现与影像学评估[J].中国医学科学院学报, 2010, 32:179-184. http://www.wanfangdata.com.cn/details/detail.do?_type=perio&id=zgyxkxyxb201002011
    [7] Andrade LA, Torresan RZ, Sales JF Jr, et al. Intravenous leiomyomatosis of the uterus. A report of three cases[J]. Pathol Oncol Res, 1998, 4:44-47. doi:  10.1007/BF02904695
    [8] Clement PB. Pure mesenchymal tumors[M]//Clement PB, Young RH. Tumors and tumor like lesions of the uterine corpus and cervix. New York: Churchill Livingstone Inc, 1993: 285-328.
    [9] Kir G, Kir M, Gurbuz A. Estrogen and progesterone expression of vessel walls with intravascular leiomyomatosis; discussion of histogenesis[J]. Eur J Gynaecol Oncol, 2004, 25:362-366. http://europepmc.org/abstract/MED/15171320
    [10] Lo KW, Lau TK. Intracardiac leiomyomatosis. Case report and literature review[J]. Arch Gynecol Obste, 2001, 264:209-210. doi:  10.1007/s004040000115
    [11] Fukuyama A, Yokoyama Y, Futagami M, et al. A case of uterine leiomyoma with intravenous leiomyomatosis——histological investigation of the pathological condition[J]. Pathol Oncol Res, 2011, 17:171-174. doi:  10.1007/s12253-010-9265-7
    [12] Du J, Zhao X, Guo D, et al. Intravenous leiomyomatosis of the uterus:a clinicopathologic study of 18 cases, with emphasis on early diagnosis and appropriate treatment strategies[J]. Hum Pathol, 2011, 42:1240-1246. doi:  10.1016/j.humpath.2010.10.015
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  • 收稿日期:  2011-10-28
  • 刊出日期:  2012-01-30

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