Lymphangioleiomyomatosis

Case contributed by Dr Lukas Valkovic

Presentation

39 year old patient with repeated pneumothorax.

Patient Data

Age: 39-year-old
Gender: Female
Modality: CT

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

Modality: MRI

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

Case Discussion

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

Lymphangioleiomyomatosis (LAM) is a rare systemic disease first described in 1937 by Stossel. It is characterised 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 haemoptysis. 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
Case created: 25th May 2016
Last edited: 25th May 2016
Systems: Vascular, Chest
Inclusion in quiz mode: Included

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