Disease relevance of Lymphangioleiomyomatosis

• CONTEXT: Lymphangioleiomyomatosis (LAM), a cystic lung disease associated with progressive respiratory failure, is found predominantly in women of childbearing age and therefore has been treated with progesterone and other hormonal agents [1].
• These mouse model observations indicate the importance of estrogen signaling in vivo for the growth of tuberous sclerosis lesions, suggesting the possible benefits of tamoxifen therapy for the treatment of angiomyolipoma and lymphangioleiomyomatosis [2].
• Estrogen enhances whereas tamoxifen retards development of Tsc mouse liver hemangioma: a tumor related to renal angiomyolipoma and pulmonary lymphangioleiomyomatosis [2].
• The true prevalence of pulmonary lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex (TSC) is unknown [3].
• Basic fibroblast growth factor (bFGF) is a potent mitogenic factor for smooth muscle cells, myofibroblasts, and fibroblasts, proliferation of which is a hallmark of idiopathic pulmonary fibrosis (IPF) and lymphangioleiomyomatosis (LAM) [4].

High impact information on Lymphangioleiomyomatosis

• Tamoxifen for lymphangioleiomyomatosis [5].
• Pulmonary lymphangiomyomatosis responsive to progesterone [6].
• These results may offer a novel therapeutic perspective for the treatment of TSC complications and lymphangioleiomyomatosis [7].
• Regulated remodeling of the ECM in diseases such as atherosclerosis and lymphangioleiomyomatosis (LAM), both characterized by excessive accumulation of smooth muscle cells (SMCs), is thought to be controlled in part by cell surface receptors for specific ECM components [8].
• Pathways involving Rho activation may have relevance to the clinical manifestations of TSC, including pulmonary lymphangioleiomyomatosis [9].

Chemical compound and disease context of Lymphangioleiomyomatosis

• We studied the rate of decline in FEV(1) and carbon monoxide transfer factor (TL(CO)) in a national cohort of patients with lymphangioleiomyomatosis in the United Kingdom and its relation to two factors that might influence the disease, menopausal status and progesterone treatment [10].
• Immunohistochemical detection of steroid receptors in a case of pulmonary lymphangioleiomyomatosis [11].
• Lymphangioleiomyomatosis may result either from the expression of a mutant tuberin with abnormal function, as a result of mutations in functionally related proteins, or from more than one mechanism [12].
• Pulmonary lymphangiomyomatosis. Long-term survival in a patient with poor response to medroxyprogesterone [13].
• BACKGROUND: Matrix metalloproteinases (MMPs) have been shown to be involved in the pathogenesis of the destructive pulmonary lesions in patients with lymphangioleiomyomatosis (LAM); in the present report, the activation of these enzymes is examined [14].

Biological context of Lymphangioleiomyomatosis

• Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment [10].
• Chromosome 16 loss of heterozygosity in tuberous sclerosis and sporadic lymphangiomyomatosis [15].
• Modulation of cell migration and invasiveness by tumor suppressor TSC2 in lymphangioleiomyomatosis [16].
• Chromosome typing in lymphangioleiomyomatosis of the lung with and without tuberous sclerosis [17].
• We used immunohistochemistry to test the hypothesis that uncontrolled cellular proliferation in lymphangioleiomyomatosis is the result of reduced tuberin protein expression [12].

Anatomical context of Lymphangioleiomyomatosis

• Two of the four patients with group B tumors had pelvic lymph nodes involved by lymphangioleiomyomatosis, and one of these patients had the tuberous sclerosis complex [18].
• An increased frequency of complex chromosomal rearrangements was found in peripheral lymphocytes of the patient with tuberous sclerosis, in comparison to the patient with isolated lymphangioleiomyomatosis [17].

Gene context of Lymphangioleiomyomatosis

• The spectrum of mutations in TSC1 and TSC2 in women with tuberous sclerosis and lymphangiomyomatosis [19].
• Expression of human telomerase reverse transcriptase in lymphangioleiomyomatosis [20].
• Imbalanced plasminogen system in lymphangioleiomyomatosis: potential role of serum response factor [21].
• Transforming growth factor-beta 1 and extracellular matrix-associated fibronectin expression in pulmonary lymphangioleiomyomatosis [22].
• Oestrogen metabolism in lymphangioleiomyomatosis: catechol-O-methyltransferase pathway is not involved [23].

Analytical, diagnostic and therapeutic context of Lymphangioleiomyomatosis

• Chest radiographs, thin-section computed tomographic (CT) scans, and results of pulmonary function tests in nine women with pulmonary lymphangiomyomatosis and two women with pulmonary tuberous sclerosis were retrospectively evaluated [24].
• To study the pulmonary structural remodeling in pulmonary lymphangiomyomatosis, electron microscopy and light and electron microscopic immunohistochemical observations for elastin and alpha 1-antitrypsin were performed on five open lung biopsy samples [25].
• Successful treatment of pulmonary lymphangiomyomatosis with oophorectomy and progesterone [26].
• Nephron-sparing nephrectomy for giant renal angiomyolipoma associated with lymphangioleiomyomatosis [27].

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