摘要目的 探讨子宫静脉内平滑肌瘤病累及心脏的临床病理学特征.方法 分析19例子宫静脉内平滑肌瘤病累及心脏患者的临床表现,对其手术标本进行常规HE染色及免疫组织化学EnVision法染色观察.结果 患者年龄33 ～59岁,平均年龄44岁.临床表现主要为心慌、胸闷、气短等心血管症状;少数为下肢水肿、月经异常、腹胀等症状,亦可无明确临床症状.病变大体表现:盘绕成结节状位于子宫肌层的肿瘤,可呈蠕虫状延伸到静脉内,直至右心房,切面实性,质地较软,灰白色,部分可囊性变;镜下表现为梭行平滑肌细胞构成,核分裂象罕见,间质血管丰富,除薄壁血管外,可见较多厚壁血管,血管扩张扭曲,并可见明显的水肿,黏液样变及玻璃样变;免疫组织化学染色平滑肌肌动蛋白、雌激素受体、孕激素受体、结蛋白阳性,HMI45阴性,4例CD34及CD10阳性,余均为阴性.结论 子宫静脉内平滑肌瘤病累及心脏为育龄期妇女少见疾病,具有特定的大体表现和免疫组织化学特点.

abstractsObjective To investigate clinicopathological features,immunophenotype and differential diagnosis of intravenous leiomyomatosis with intracardiac extension.Methods Clinical manifestations,morphologic features,and immunohistochemical staining were retrospectively analyzed in 19 cases of intravenous leiomyomatosis with intracardiac extension.Results The patients' age ranged from 33 to 59 years (mean 44 years).Clinical presentation included chest tightness,palpitation,dyspnea,edema of low extremity,abdominal distention or hypermenorrhea.However,a few patients were asymptomatic.Grossly,intravenous leiomyomatosis in most cases demonstrated coiled or nodular growth within the myometrium with worm-like involvement of the uterine vein in broad ligament or other pelvic veins with continued extension into the vena cava and the heart.The intravenous tumor surface was generally smooth and rubbery,with a greyish-white color.Microscopically,the tumors were composed of spindle cells with rare mitotic figures and the presence of abundant thick-walled vessels.Marked fibrosis,hyalinization,myxoid and edematous changes were common.The tumor cells were positive for SMA,ER,PR and desmin but negative for HMB45 and S-100.CD10 and CD34 were positive in 4 patients and negative in the remaining cases.Conclusion Intravenous leiomyomatosis with intracardiac extension is a rare disease among women of child-bearing age,with specific morphologic manifestations and immunohistochemical profiles.