Lymphangioleiomyomatosis Presenting with Perirenal Hemorrhage. Issue 1 (15th September 2015)

• Record Type: Journal Article
• Authors: Dekeyzer, S.; Peters, N.; Smeets, P.; De Visschere, P.; Decaestecker, K.; Gosselin, R.
• Is Part Of: Journal of the Belgian Society of Radiology. Volume 99:Issue 1(2015)
• Page Start: 46
• Page End: 49
• Publication Date: 2015-09-15
• Subjects: Lymphangiomyomatosis; Radiology -- Periodicals; Periodicals; 616.0757
• DOI: 10.5334/jbr-btr.836
• Languages: English
• ISSNs: 2514-8281

Abstract

Lymphangioleiomyomatosis (LAM) is a rare debilitating disease of unknown etiology, classically described as almost exclusively affecting women of childbearing age. The disease most commonly involves the lungs and is characterized by hamartomatous smooth muscle cell proliferations along blood vessels, airways and lymphatics. Most patients present with pulmonary symptoms, including shortness of breath, recurrent pneumothorax and pleural effusions. Extrapulmonary manifestations of LAM as the initial presentation of the disease are highly unusual. We present the case of a patient in whom LAM was incidentally discovered when the patient presented with retroperitoneal hemorrhage from a ruptured renal angiomyolipoma.