Pulmonary Lymphangioleiomyomatosis: A Clinicopathological Analysis of Ten Cases

Background: Lymphangioleiomyomatosis (LAM) of the lung is a very rare disease of unknown 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 in Chang Gung Memorial Hospital from 1990 to 2001 were collected from the pathology files or by consultation with thoracic surgeons, chest physicians, and radiologists of this institution. Cases were confirmed by lung biopsy and high-resolution computed tomographic scanning. Clinical data were obtained from patients ' charts. Additional immunostaining for HMB-45 antigen, estro-gen receptor, and progesterone receptor was carried out for cases with avail-able paraffin blocks. Results: In total, 10 patients confirmed with a diagnosis of LAM and adequate clini-cal follow-up information were found from 1991 to 2001. All were females of fertility age. The follow-up periods ranged from 18 to 167 months. Six patients received hormonal therapy. According to the pulmonary functio