淋巴管肌瘤病的诊治进展与长期预后

Advances in the Diagnosis, Treatment and Prognosis of Lymphangioleiomyomatosis

摘要: 淋巴管肌瘤病(lymphangioleiomyomatosis, LAM)是一种罕见多系统低度恶性肿瘤性疾病，以弥漫性肺部囊性改变为主要特征。近年来，LAM研究取得了快速发展。基因谱研究使LAM的遗传学机制更为清晰。新的影像学技术⁶⁸Ga-NEB PET-CT扫描为LAM患者的淋巴循环异常提供无创、快速和精确的评估方法。西罗莫司治疗LAM的长期有效性和安全性获得了更多的证据。LAM患者的预后较过去有了很大的提高。本文主要对国内外LAM诊断和治疗的研究进展做简要综述。

Abstract: Lymphangioleiomyomatosis (LAM) is a rare, multisystemic, low-grade neoplasm character-ized by diffuse cystic lesions in the lung.In recent years, emerging imaging examination such as ⁶⁸Ga-NEB PET-CT scan provides efficient and precise non-invasive diagnostic methods to detect lymphatic circulation abnormalities in LAM patients. The long-term efficacy and safety of sirolimus for LAM has accumulated further evidence, and genetic profiling studies have unveiled more information of genetic mechanisms. Prognosis of LAM has been much improved. We briefly reviewed the research advances of LAM in China and other countires.