Pulmonary imaging manifestations and related research progress of lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis (LAM) is a rare multisystem neoplastic disease and is primarily affected women of childbearing age and premenopausal women. LAM lesions involve the lungs [known as pulmonary Lymphangioleiomyomatosis (PLAM)], kidneys [such as angiomyolipoma (AML)], and the lymphatic system (including lymphangioleiomyomas and chylous effusions). As the disease progresses, LAM disrupts lung tissue, alters lung structure, and leads to the development of lymphangioleiomyomas in the chest and abdominal lymphatic ducts. Early symptoms in LAM patients are mild, and clinical presentations lack specificity, making misdiagnosis common. Death can occur due to pulmonary function deterioration and recurrent pneumothorax. Currently, lung transplantation is considered the only effective treatment, although recurrence rates are relatively high. High-resolution computer tomography (HRCT) of the chest is a key diagnostic tool for LAM,which aid not only in the diagnosis but also in assessing the severity and prognosis of the condition. With the rapid development of medical imaging technology, particularly the use of photon counting detector CT (PCD-CT), which offers high resolution and noise reduction capabilities, significant improvements in image quality can be achieved. Compared to traditional CT scans, PCD-CT reduces radiation exposure by 35.7%, making it highly suitable for diagnosing and long-term monitoring of LAM.

Keywords

• |lymphangio-leiomyomatosis|radiology|photon counting detector computed tomography