Lymphangioleiomyomatosis presented by pulmonary nodules

Abstract: Background: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease in reproductive-aged females, leading to progressive respiratory failure. The common radiographic findings are homogenously distributed multiple well-defined thin-walled cysts throughout all lung zones.
Case Report: We report a case of LAM presenting with pulmonary nodules without cystic lesion. A 48-year-old female visited our out-patient department for breast cancer evaluation. Breast cancer was detected during a regular physical examination. Computerized tomography revealed a 10 mm-sized irregular marginated nodule with perilesional ground glass opacity in the superior segment of the right lower lobe. The patient underwent video-assisted thoracoscopic wedge resection and the specimen confirmed a diagnosis of pulmonary LAM. After surgery there was no recurrence of breast cancer and LAM.
Conclusions: Unlike typical radiographic findings of LAM, our case presented as incidentally detected pulmonary nodules and was confirmed after surgical biopsy. We hypothesize that pulmonary nodules can be an early sign of LAM and surgery may prevent the progressive cystic changes in later stages.

Keywords: lymphangioleiomyomatosis (LAM), lung surgery, pulmonary nodule