Lymphangioleiomyomatosis
Lymphangioleiomyomatosis Updated: 11/01/2021 © Jun Wang, MD, PhD General features Cystic destruction with progressive dysfunction of lung Proliferation of lymphatic smooth muscle-like cells Probably originated from perivascular epithelioid cell Most common in reproductive age women Associated with tuberous sclerosis Key clinical features Most common presentations: Dyspnea, cough, pleural effusion, etc Pneumothorax commonly seen Key morphological features Cystic changes Proliferation of spindle cells (LAM cells), may be nodular Genetic abnormalities TSC1 and TSC2 Diagnosis Radiologic studies: Cystic changes Pathological identification of LAM cells Markers Positive: HMB45 Treatment Treatments of complications Lung function improvement Lung transplantation Back to respiratory tract neoplasms Back to contents