Pulmonary lymphangioleiomyomatosis: a clinicopathological analysis of ten cases

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.

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, estrogen receptor, and progesterone receptor was carried out for cases with available paraffin blocks.

In total, 10 patients confirmed with a diagnosis of LAM and adequate clinical 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 function tests and clinical symptoms, 6 patients were in stable condition and 4 showed slow progression of disease by the time of the last follow-up. Two patients died after lung transplantation and abdominal surgery for retroperitoneal LAM, respectively, at another medical center.

The clinicopathological features of our series are similar to those reported from other countries. The clinical course was slowly progressive with no significant response to hormonal therapy. Lung biopsies are important for a diagnosis, but the size and location of the biopsy specimens greatly affect the diagnostic reliability.