[Clinicopathologic analysis of pulmonary lymphangioleiomyomatosis].
OBJECTIVE
To improve the understanding and diagnosis of pulmonary lymphangioleiomyomatosis (LAM).


METHODS
Fifteen cases of LAM of our hospital were presented and 73 cases reported in domestic literature from 1993 to 2008 were reviewed. By means of histological and immunohistochemical(IHC)studies, the clinical and pathological features of LAM were analyzed.


RESULTS
All the 88 cases were female, with an average age of onset at (37 +/- 9) years. The main clinical manifestations included dyspnea (83/88, 94%), hemoptysis (48/88, 54%), pneumothorax (41/88, 47%), and chylothorax (28/88, 32%). High resolution computerized tomography (HRCT) showed thin-walled air-filled cysts throughout both lungs. Pathological features showed cystic changes in the lung, and abnormal smooth muscle cells (LAM cells) lined the airways, bronchioles, lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts. In the autopsy case, extrapulmonary organs (eg, kidney, lymph nodes and soft tissues) were also involved. Abnormal manifestations in abdomen, including renal mass, retroperitoneal mass and retroperitoneal lymphadenopathy, were detected in 23 cases.


CONCLUSIONS
LAM is a multisystem disease. Chest HRCT had confirmative value for diagnosis of LAM. In practice, chest HRCT, as well as other routine abdominal and pelvic imaging examinations, should be performed for child-bearing-age women with progressive dyspnea, hemoptysis, or spontaneous pneumothorax.
Lu
Shao-Hua
SH
Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
Tan
Yun-Shan
YS
Xu
Jian-Fang
JF
Sujie
Akesu
A
Bai
Chun-Xue
CX
Zhu
Xiong-Zeng
XZ
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology*; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging / Q000473:pathology*; D008875:Middle Aged; D011859:Radiography; D055815:Young Adult