Korean J Med.  2024 Feb;99(1):25-31. 10.3904/kjm.2024.99.1.25.

A 32-Year-Old Female with Incidentally Detected Multifocal Cystic Pulmonary Lesions on Chest Computed Tomography

• Yoon HY

• KMID: 2552050
• DOI: http://doi.org/10.3904/kjm.2024.99.1.25

Abstract

Lymphangioleiomyomatosis (LAM) is a rare condition that exclusively affects women and is characterized by lung parenchymal destruction and the formation of cysts due to infiltration by LAM cells. It can also impact the lymphatic system, leading to the development of fluid-filled cystic structures and abdominal involvement, including renal angiomyolipomas (AML) or lymphangioleiomyoma. LAM can occur sporadically or be associated with tuberous sclerosis complex. Common symptoms include respiratory issues such as dyspnea, pneumothorax, chylothorax, along with other manifestations like renal AML and lymphatic involvement. The diagnosis of LAM relies on a combination of clinical, radiological, and laboratory findings, and treatment options vary depending on symptoms and patient status, with mTOR inhibitors being considered as a treatment option. This case outlines the diagnostic journey and clinical course of a 32-year-old female patient with LAM.

Keyword

림프관평활근종증; 낭성 폐병변; 진단; Lymphangioleiomyomatosis; Cyst pulmonary lesions; Diagnosis