右子宫宽韧带肿块切除后乳糜胸诊断为淋巴管肌瘤病1例报告并文献复习

Liwan Dai, Tao Chen, Jinxing Wu
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摘要

摘要淋巴管平滑肌瘤病(LAM)是一种罕见的疾病,常见于育龄妇女。它是一种多系统疾病,以平滑肌细胞在肺和其他组织中的增殖、转移和浸润为特征。我们将报告一例被诊断为LAM的患者在子宫阔韧带切除术后出现乳糜胸。我们将报告一例确诊为LAM的患者,在手术治疗后出现乳糜胸,术后出现呼吸困难。胸部CT发现双侧胸腔积液及双肺多发囊性病变。胸膜液乳糜试验阳性,术后病理证实LAM。患者给予西罗莫司治疗,疗效理想。然而,附件占位性切除后乳糜胸的出现尚未见报道。结论LAM的发病机制尚不完全清楚,但该患者的临床表现似乎证实了LAM起源于女性生殖系统的假设。女性生殖系统肿瘤的鉴别诊断应考虑LAM,我们建议术前完善胸部CT检查,警惕肺部多发囊性改变的可能性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Chylothorax after Excision of The Right Broad Ligament Mass of The Uterus was Diagnosed Lymphangiomyomatosis A Case Report and Literature Review
Lymphangioleiomyomatosis (LAM) is a rare disease frequently (often) occurs in women of childbearing age. It is a multi-systemic disorder characterized by the proliferation, metastasis, and infiltration of smooth muscle cells in the lung and other tissues. We will report a case of a patient diagnosed with LAM who presented with chylothorax after hysterectomy of broad ligament. of etiology of this but the this the this We will a case of a patient diagnosed with LAM who presented with chylothorax after broad ligament developed dyspnea after surgical treatment. Chest CT found bilateral pleural effusion and multiple cystic lesions in both lungs. The pleural fluid Chyle test was positive, subsequent postoperative pathology confirmed LAM. The patient was treated by Sirolimus with Ideal therapeutic effect. However the presence of chylothorax after adnexal occupying resection has not been reported. Conclusions The pathogenesis of LAM is not completely clear, but the clinical manifestations of this patient seem to confirm the hypothesis that LAM originates from the female reproductive system. LAM should be considered in the differential diagnosis of female reproductive system tumors, we suggested to improve chest CT before surgery to be vigilant against the possibility of LAM if multiple cystic changes occur in pulmonary.
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