Lymphangiomyomatosis
From Radipedia
Non-neoplastic hamartomatous proliferation of atypical muscle cells that leads to progressive cystic destruction of lung parenchyma.
- Five year survival of 50%
- Exclusively women, mean age 34
- Most do not have tuberous sclerosis, but when present it is usually obvious from childhood
- Spontaneous pneumothorax
- Round, thin wall cysts
- Diffuse in distribution
- Hyperinflated lungs
- Not smoking related
- Chylothorax
