协和医学杂志

2012, (01) 51-55

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静脉内平滑肌瘤病的临床病理
Clinicopathological Features of Intravenous Leiomyomatosis

赵大春;田庄;梁智勇;刘彤华;
ZHAO DA-chun1,TIAN Zhuang2,LIANG Zhi-yong1,LIU Tong-hua1 1Department of Pathology,2Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Scienses & Peking Union Medical College,Beijing 100730,China

摘要(Abstract):

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。
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.

关键词(KeyWords): 静脉内平滑肌瘤病;免疫组化;下腔静脉
intravenous leiomyomatosis;immunohistochemistry;inferior vena cava

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作者(Author): 赵大春;田庄;梁智勇;刘彤华;
ZHAO DA-chun1,TIAN Zhuang2,LIANG Zhi-yong1,LIU Tong-hua1 1Department of Pathology,2Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Scienses & Peking Union Medical College,Beijing 100730,China

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