Lymphangioleiomyomatosis (LAM) is a rare disease caused by
proliferation of abnormal smooth muscle-like cells (LAM cells), which can
result in cystic destruction of lungs, formation of renal angiomyolipomas,
lymphadenopathy, cystic lymphangiomas, abdominal lymphangioleiomyomas,
chylothorax, and chyloperitoneum.
It is a low-grade metastasizing neoplasm spreading through lymphatic
routs and belongs to the group of the PEComas (perivascular epithelioid
cell tumours). This disease affects mainly women in childbearing age and occurs
in two forms: As a sporadic lymphangioleiomyomatosis (sLAM) or associated with
tuberous sclerosis complex (TSC-LAM).
The incidence of sLAM is about 3.4/ million to 7.5/million
women, and it was assessed that approximately 0.3 million to 0.4 million of
women with tuberous sclerosis (TSC) have a LAM.
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