Pulmonary
lymphangiomyomatosis (LAM) associated with both renal and hepatic angiomyolipomas (AMLs) in a TSC patient is very rare.
Perivascular epithelioid cell tumors (PEComas) of the kidney are a group of tumor family covering classical angiomyolipoma (AML), microscopic AML (microhamartoma), intraglomerular lesions, cystic AML, epithelioid AML, oncocytoma-like AML and
lymphangiomyomatosis of the renal sinus.
3) As noted by Davis et al, (2) a dominant feature in all cases of AMLEC is a prominent lymphatic channel network in the smooth muscle component forming slitlike branched and curvilinear spaces strongly resembling those seen in
lymphangiomyomatosis.
Major Criteria Minor Criteria Facial angiofibromas or Multiple randomly distributed forehead plaque pits in dental enamel Ungual or periungual fibroma Hamartomatous rectal polyps (Non-traumatic) Bone cysts Hypomelanotic macules (>3) Cerebral white matter Shagreen patch (connective migration tracts tissue naevus) Gingival fibromas Cortical tuber Nonrenalhamartoma Subependymal nodule Retinal achromic patch Subependymal giant cell astrocytoma Confetti skin lesions Multiple retinal nodular hamartomas Multiple renal cysts Cardiac rhabdomyoma, single or multiple Nonrenal hamartomas Pulomary
lymphangiomyomatosis Renal angiomyolipoma.
Major features of TSC include cutaneous lesions such as adenoma sebaceum, more than three hypomelanotic macules, shagreen patch, and periungual fibromas; cortical tubers, subependymal nodules, retinal hamartomas, heart rhabdomyomas, renal angiomyolipomas, and lung
lymphangiomyomatosis.
Lymphangiomyomatosis typically presents in the third or fourth decade of life.
The four main causes of chylothorax include: malignancy; trauma; idiopathic; and miscellaneous causes such as thrombosis of the superior vena cava or subclavian vein, cirrhosis and rarely, pulmonary
lymphangiomyomatosis.
He had a history of right ureterolithotomy and diabetes, although no history of tuberous sclerosis,
lymphangiomyomatosis, renal cancer or renal cyst was present.
In comparison,
lymphangiomyomatosis (LAM) shows larger thin-walled cysts that are diffusely distributed in the lungs and eventually involve the entire lung parenchyma.
Mrs Priest, 61, of Colders Drive, suffers from a respiratory disease, pulmonary
lymphangiomyomatosis.
INTRODUCTION:
Lymphangiomyomatosis is a rare, diffuse and progressive lung disease that affects young women of child bearing age.
