Advances in the Diagnosis, Treatment and Prognosis of Lymphangioleiomyomatosis

Chongsheng Cheng; Song Liu; Yanli Yang; Guozhu Hou; Wuying Chenh; Tengyue Zhang; Danjing HU; Xinlun Tian; Kaifeng XU

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Advances in the Diagnosis, Treatment and Prognosis of Lymphangioleiomyomatosis

VernacularTitle:淋巴管肌瘤病的诊治进展与长期预后
Author: Chongsheng CHENG ^{1
,

2
,

3} ; Song LIU ^{2
,

3
,

4} ; Yanli YANG ^{1
,

2
,

3} ; Guozhu HOU ^{2
,

3
,

5} ; Wuying CHENH ^{2
,

3
,

5} ; Tengyue ZHANG ^{1
,

2
,

3} ; Danjing HU ^{1
,

2
,

3} ; Xinlun TIAN ^{1
,

2
,

3} ; Kaifeng XU ^{1
,

2
,

3}
Author Information

1. Department of Pulmonary and Critical Care Medicine, Chinese Academy of Medical Sciences &
2. Peking Union Medical College, Beijing 100730, China.State Key Laboratory of Complex, Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &
3. Peking Union Medical College, Beijing 100730, China.
4. Medical Research Center, Chinese Academy of Medical Sciences &
5. Department of Nuclear Medicine, Chinese Academy of Medical Sciences &
Publication Type:Journal Article
Keywords: lymphangioleiomyomatosis; diagnosis; treatment
From: JOURNAL OF RARE DISEASES 2022;1(1):38-44
CountryChina
Language:Chinese
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.