Background: Lymphangioleiomyomatosis (LAM) of the lung is a very rare disease ofunknown etiology which occurs particularly in women of childbearing age.So far, there have been very few reports of LAM in Taiwan.Methods: Data on all patients with a clinical or pathological diagnosis of LAM inChang Gung Memorial Hospital from 1990 to 2001 were collected from thepathology files or by consultation with thoracic surgeons, chest physicians,and radiologists of this institution. Cases were confirmed by lung biopsy andhigh-resolution computed tomographic scanning. Clinical data were obtainedfrom patients' charts. Additional immunostaining for HMB-45 antigen, estrogenreceptor, and progesterone receptor was carried out for cases with availableparaffin blocks.Results: In total, 10 patients confirmed with a diagnosis of LAM and adequate clinicalfollow-up information were found from 1991 to 2001. All were femalesof fertility age. The follow-up periods ranged from 18 to 167 months. Sixpatients received hormonal therapy. According to the pulmonary functiontests and clinical symptoms, 6 patients were in stable condition and 4 showedslow progression of disease by the time of the last follow-up. Two patientsdied after lung transplantation and abdominal surgery for retroperitonealLAM, respectively, at another medical center.Conclusions: The clinicopathological features of our series are similar to those reportedfrom other countries. The clinical course was slowly progressive with no significantresponse to hormonal therapy. Lung biopsies are important for adiagnosis, but the size and location of the biopsy specimens greatly affect thediagnostic reliability.