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Disease results from the proliferation of neoplastic cells (LAM cells), which, in many cases, have a smooth muscle cell phenotype, express melanoma antigens, and have mutations in one of the tuberous sclerosis complex genes (<jats:italic>TSC1<\/jats:italic> or <jats:italic>TSC2<\/jats:italic>). In the lung, LAM cells found in the vicinity of cysts are, at times, localized in nodules and may be responsible for cyst formation through the production of proteases. Lymphatic channels, expressing characteristic lymphatic endothelial cell markers, are found within the LAM lung nodules. LAM cells may also be localized within the walls of the axial lymphatics, and, in some cases, penetrate the wall and proliferate in the surrounding adipose tissue. Consistent with extensive lymphatic involvement in LAM, the serum concentration of VEGF\u2010D, a lymphangiogenic factor, is higher in LAM patients than in healthy volunteers.<\/jats:p>","DOI":"10.1196\/annals.1413.018","type":"journal-article","created":{"date-parts":[[2008,6,3]],"date-time":"2008-06-03T00:11:08Z","timestamp":1212451868000},"page":"206-214","source":"Crossref","is-referenced-by-count":35,"title":["Lymphatic Involvement in Lymphangioleiomyomatosis"],"prefix":"10.1111","volume":"1131","author":[{"given":"Connie G.","family":"Glasgow","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelo M.","family":"Taveira\u2010DaSilva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas N.","family":"Darling","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel","family":"Moss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,5,28]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.151.2.7842216"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199909000-00004"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200409-1298OC"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.115.4.1041"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199011013231807"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1055\/s-2002-25298"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2007.01101.x"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1177\/107327480601300405"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2004.023283"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.164.6.2102125"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.214.2.r00fe41441"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1272\/jnms.67.311"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1053\/hupa.2000.18500"},{"key":"e_1_2_6_15_2","doi-asserted-by":"crossref","first-page":"1642","DOI":"10.5858\/2000-124-1642-HOTIPI","article-title":"Hyperplasia of type II pneumocytes in pulmonary lymphangioleiomyomatosis: immunohistochemical and electron microscopic study","volume":"124","author":"Matsui K.","year":"2000","journal-title":"Arch. 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