Lymphangioleiomyomatosis

Discussion:

The original histology suggested metastatic gynecological origin. After repeated review histology confirmed lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare systemic disease first described in 1937 by Stossel. It is characterized by infiltration of the lungs, respiratory tract and the lymphatic vessels by immature atypical smooth muscle cells.

The disease affects almost exclusively women of childbearing age. Among the first symptoms are shortness of breath, cough and pneumothorax. Later, there is a possible development of chylothorax or hemoptysis. LAM is an indication for a lung transplant. Dominant findings in HRCT are diffuse cystic lung changes. Other symptoms stem primarily from lymph nodes and obstruction of lymphatic vessels.

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