lymphangiomyomatosis

lymphangiomyomatosis

 [lim-fan″je-o-mi″o-mah-to´sis]
a progressive disorder of women of child-bearing age, marked by nodular and diffuse interstitial proliferation of smooth muscle in the lungs, lymph nodes, and thoracic duct. Called also lymphangioleiomyomatosis.

lym·phan·gi·o·lei·o·my·o·ma·to·sis

(lim-fam'jē-ō-lī'ō-nū'ō-mă-tō'sis), [MIM*606690]
A rare disorder of unknown etiology seen in women of reproductive age and in patients of either sex with tuberous sclerosis. Pulmonary complications are due to hamartomatous proliferation of smooth muscle cells preferentially along bronchovascular structures resulting in obliteration of the airways and consecutive development of cysts in the lungs. Usually progressive, leading to death from respiratory failure. Treatment by lung transplantation has been successful.
References in periodicals archive ?
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.
Fletcher, "Lymphangiomyomatosis and angiomyolipoma: closely related entities characterized by hamartomatous proliferation of HMB-45-positive smooth muscle," Histopathology, vol.
Similar effects were noted in TSC patients with other types of benign tumors, including renal angiomyolipoma, fascial angiofibroma, lymphangiomyomatosis, cardiac rhabdomyoma, and retinal astrocytic hamartoma [35].
(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. This feature is also present, albeit focally, in most triphasic and myomatous or muscle-predominant AMLs.
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. Minor features include bone cysts, confetti skin lesions, CNS white matter migration abnormalities, dental enamel pits, gingival fibromas, rectal polyps, multiple renal cysts, nonrenal hamartomas, and a retinal achromic patch.
- Cardiac involvement occurs during the intrauterine or neonatal period.- Rhabdomyomas tend to regress over time.- Epilepsy autism and developmental delays manifest themselves from infancy to adolescence.- Polycystic kidney disease usually is apparent in infancy or early childhood.- AMLs may develop at any time from childhood into adult life.- 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. [4]
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.
* Lymphangiomyomatosis in the visualized lung bases.