Diagnosis and treatment for 2 cases of tuberous sclerosis complex with pulmonary lymphangioleiomyomatosis.
10.11817/j.issn.1672-7347.2016.07.018
- Author:
Dandan ZONG
1
;
Caihong LIU
1
;
Zhihui SHI
1
;
Li ZHOU
1
;
Yating PENG
1
;
Ruoyun OUYANG
1
Author Information
1. Department of Respiratory Medicine, Second Xiangya Hospital, Central South University, Changsha 410011, China.
- Publication Type:Case Reports
- MeSH:
Humans;
Kidney;
Lung;
Lung Neoplasms;
Lymphangioleiomyomatosis;
Retrospective Studies;
Tuberous Sclerosis
- From:
Journal of Central South University(Medical Sciences)
2016;41(7):771-776
- CountryChina
- Language:Chinese
-
Abstract:
To improve the diagnosis and treatment for tuberous sclerosis complex (TSC) with pulmonary lymphangioleiomyomatosis, a retrospective analysis was performed based on the clinical data of 2 patients with such disease. Both of them have typical thin-walled cystic lesion throughout the lung field, renal angioleiomyolipoma, and various degrees of skin lesions. Central nervous system is involved in one patient. Lesions in the lung and kidney in one patient were improved significantly after 5 months of rapamycin treatment. The clinical phenotypes were diverse in TSC patients. The CT imaging showed typical characteristics when the lung was invaded by the tumor. When a patient was diagnosed as pulmonary lymphangioleiomyomatosis, we should pay attention to the clinical screening of TSC. Rapamycin is an effective and safe treatment for this disease.
