Pulmonary Lymphangioleiomyomatosis: A Case Report and Literature Review.
Pulmonary lymphangioleiomyomatosis (LAM) is a rare lung disease characterized by diffuse cystic changes caused by a destructive proliferation of smooth muscle-like cells or LAM cells. It is a part of the perivascular epithelioid cell family of tumors. LAM may be associated with the genetic disorder tuberous sclerosis complex or may occur sporadically. Individuals affected by LAM are typically females of child-bearing age who present with recurrent spontaneous pneumothorax. The microscopic findings can be subtle and careful examination is needed to identify the neoplastic cells of LAM. Immunohistochemical markers in cases of LAM demonstrate a characteristic co-expression of myogenic and melanocytic markers. We report a case of a 41-year-old woman who presented with multiple episodes of spontaneous pneumothorax and microscopic findings characteristic of LAM.
['Sathirareuangchai|Sakda|S|', 'Shimizu|David|D|', 'Vierkoetter|Koah Robin|KR|']
D000328:Adult D005260:Female D006801:Humans D008168:Lung D017563:Lung Diseases, Interstitial D018192:Lymphangioleiomyomatosis D011030:Pneumothorax D014057:Tomography, X-Ray Computed
Pulmonary lymphangioleiomyomatosis tuberous sclerosis spontaneous pneumothorax