TITLE:
A Rare Case of Progressive Dyspnea-Lymphangioleiomyomatosis
AUTHORS:
Palak Grover, Pritha Mohanta, Gurleen Kaur, Bipneet Singh
KEYWORDS:
Lymphangioleiomyomatosis, Dyspnea, HRCT
JOURNAL NAME:
Journal of Biosciences and Medicines,
Vol.14 No.7,
July
24,
2026
ABSTRACT: This report details the diagnosis and management of a 60-year-old postmenopausal woman presenting with a 3-year history of progressive exertional dyspnea. High-resolution computed tomography (HRCT) revealed diffuse, thin-walled cystic lung lesions. Serum vascular endothelial growth factor-D (VEGF-D) was below the diagnostic threshold (800 pg/mL), and no other non-invasive confirmatory features, such as tuberous sclerosis complex, renal angiomyolipoma, chylous effusion, or lymphangioleiomyoma, were present; therefore, a video-assisted thoracoscopic surgery (VATS)-guided surgical lung biopsy was pursued. Histopathology confirmed the diagnosis of lymphangioleiomyomatosis (LAM) through the identification of proliferating spindle-shaped cells within cyst walls that stained positive for HMB-45, smooth muscle actin (SMA), and estrogen receptors. The patient was treated with sirolimus (titrated to a target trough of 5 - 15 ng/mL), resulting in stabilized lung function and the ability to discontinue supplemental oxygen after 12 months. The case underscores the importance of considering LAM in the differential diagnosis of diffuse cystic lung disease in women of all ages, including those who are postmenopausal.