协和医学杂志

2012, (01)

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静脉内平滑肌瘤病临床病理分析
Clinicopathologic Analysis of Intravenous Leiomyomatosis

赵大春

摘要(Abstract):

【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,对手术标本进行HE和免疫组织化学染色(EnVisionTM)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。大体表现其中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,CD34有4例阳性,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 clinicopathologic features, immunophenotype and differential diagnosis of Intravenous Leiomyomatosis. Methods Clinical manifestation, morphologic features , HE and immunohistochemical staining were studied in 34 cases of IVL. Results Patients were 24 to 61 years of age (mean 44.8). Patients presented with edema of low extremity,abdominal distention ,hypermenorrhea and other pelvic discomfort . The symptoms included chest tightness,palpitation and dyspnea when tumors involved in inferior vena cava and heart. 14cases located in uterine, 6 involved in pelvic veins. Inferior vena cava(IVC) were involved in 14 patients, and 9 of their tumors extended through IVC into right heart chamber. Microscopically, the tumors were composed of spindle-shaped cells with rare mitotic figure. The tumors were positive for SMA, ER, PR, Desmin, and negative for CD10, HMB45. 4 cases were CD34 positive, others negative. Ki-67 index 0.3-2.7%. Conclusion IVL is rare disease before the onset of menopause, microscopic benign smooth-muscle tumor but invasive growth pattern. It have characteristic features of IHC and macrograph.

关键词(KeyWords): |静脉内平滑肌瘤病|免疫组化|下腔静脉||
|Intravenous Leiomyomatosis| IHC| inferior vena cava|

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作者(Author): 赵大春

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