Pulmonary Lymphangioleiomyomatosis and Extra-Pulmonary Lymphangioleiomyomas in Postmenopausal Women
Keywords:
Lymphangioleimyomatosis, mediastinal lymphangioleiomyomas, postmenopausal women, cystics lungs
Abstract
Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by progressive cystic destruction of the lung, lymphatic abnormalities and abdominal tumors. It occurs almost exclusively in women of fertile age due to a hormonal influence, for this reason it is extremely rare in post-menopausal patients. While LAM is a systemic disease, the main manifestations are pulmonary. Lymphangioleiomyomas are larger cystic masses; these most commonly occur in the abdomen, retroperitoneum and pelvis but occasionally in the mediastinum and neck. In the cases of older women the disease presents a similar clinical manifestation than younger#x2019;s female, with the exception that the clinical course is benign and longer.
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2020-12-15
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