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.

lymphangiomyomatosis

/lym·phan·gio·my·o·ma·to·sis/ (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.
References in periodicals archive ?
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.
Rabban and coworkers (8) detected no ALK-1 staining among 7 uterine leiomyomas, 6 uterine leiomyosarcomas, 4 uterine carcinosarcomas, 4 endometrial stromal sarcomas, and 1 uterine lymphangiomyomatosis.
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.
1) Pulmonary lesions are less common and occur mainly as lymphangiomyomatosis and/or multifocal micronodular pneumocyte hyperplasia.
Comparison of melanoma antigen recognized by T cells (MART-1) to HMB-45: additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell sugar tumor.
Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism.
Warren SE, Lee D, Martin V, et al: Pulmonary lymphangiomyomatosis causing bilateral pneumothorax during pregnancy.
Transbronchial biopsy in lymphangiomyomatosis of the lung: HMB45 for diagnosis.
4,5) Angiomyolipomas are now included in a family of tumors called PEComas (tumors showing perivascular epithelioid cell differentiation) that also includes lymphangiomyomatosis, clear cell "sugar" tumor of the lung, and other rare, morphologically and immunophenotypically similar lesions arising at a variety of visceral and soft tissue sites.
Comparison of melanoma antigen recognized by T-cells (MART-1) to HMB-45: additional evidence to support a common lineage for angiomyolipoma, lymphangiomyomatosis, and clear cell sugar tumor.