Case contributed by Dr Lukas Valkovic


39 year old patient with repeated pneumothorax.

Patient Data

Age: 39-year-old
Gender: Female

39 year old patient with repeated pneumothorax with subsequent findings of cystic changes in the lungs on HRCT. VATS was performed with repeated pleurodesis.


There were also found multiple retroperitoneal cystoid expansions with solid components. 

Case 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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Case information

rID: 45448
Published: 25th May 2016
Last edited: 14th Aug 2019
System: Vascular, Chest
Inclusion in quiz mode: Included

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