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<meta name="keywords" content="C0751674, lam, lymphangio-myomatosis, lymphangioleiomyomatoses, lymphangioleiomyomatosis, lymphangioleiomyomatosis, somatic, lymphangiomyomatoses, lymphangiomyomatosis, neoplastic process, tsc1, tsc1-related lymphangioleiomyomatosis, tsc2, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Lymphangioleiomyomatosis (LAM) is a condition that affects the lungs, the kidneys, and the lymphatic system. The lymphatic system consists of a network of vessels that transport lymph fluid and immune cells throughout the body. Lymph fluid helps exchange immune cells, proteins, and other substances between the blood and tissues.\n\nLAM is found almost exclusively in women. It often occurs as a feature of an inherited syndrome called tuberous sclerosis complex. When LAM occurs alone it is called isolated or sporadic LAM.\n\nSigns and symptoms of LAM most often appear during a woman's thirties. Affected women have an overgrowth of abnormal smooth muscle-like cells (LAM cells) in the lungs, resulting in the formation of lung cysts and the destruction of normal lung tissue. They may also have an accumulation of fluid in the cavity around the lungs (chylothorax).\n\nThe lung abnormalities resulting from LAM may cause difficulty breathing (dyspnea), chest pain, and coughing, which may bring up blood (hemoptysis). Many women with this disorder have recurrent episodes of collapsed lung (spontaneous pneumothorax). The lung problems may be progressive and, without lung transplantation, may eventually lead to limitations in activities of daily living, the need for oxygen therapy, and respiratory failure. Although LAM cells are not considered cancerous, they may spread between tissues (metastasize). As a result, the condition may recur even after lung transplantation.\n\nWomen with LAM may develop cysts in the lymphatic vessels of the chest and abdomen. These cysts are called lymphangioleiomyomas. Affected women may also develop tumors called angiomyolipomas made up of LAM cells, fat cells, and blood vessels. Angiomyolipomas usually develop in the kidneys. Internal bleeding is a common complication of angiomyolipomas." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
UID=148366
ConceptID=C0751674
-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Lymphangiomyomatosis<span class="h1sub">(LAM)</span></div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>148366</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information."><span class="highlight" style="background-color:">C0751674</span></a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
<td>LAM; Lymphangioleiomyomatosis; Lymphangioleiomyomatosis, somatic; TSC1-Related Lymphangioleiomyomatosis</td></tr>
<tr><td><span class="bold">SNOMED CT: </span></td>
<td>Lymphangiomyomatosis (73017001); Lymphangioleiomyomatosis (73017001)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td><a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#target-gene-loc">Genes (locations):<img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a><div class="display-none" id="target-gene-loc">
Gene(s) directly associated with<br />
this condition or phenotype.</div></td>
<td><a target="_blank" title="TSC1 - ID: 7248 - NCBI Gene" href="/gene/7248" class="medgenPMinfo">TSC1</a> (9q34.13); <a target="_blank" title="TSC2 - ID: 7249 - NCBI Gene" href="/gene/7249" class="medgenPMinfo">TSC2</a> (16p13.3)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0011705" target="_blank">MONDO:0011705</a></td></tr>
<tr><td>OMIM<span class="superscript">®</span>:</td>
<td><a href="https://omim.org/entry/606690" target="_blank">606690</a></td></tr>
</tbody></table></div><div class="rprt-body jig-ncbiinpagenav" data-jigconfig="smoothScroll: false, gotoTopLink: true, gotoTopLinkText: '', gotoTopLinkAttrs: {'title': 'Go to the top of the page'},allHeadingLevels: ['h1'], topOfPageTOC: true, tocId: 'my-toc'"><div id="rprt-tabs-1" class="rprt-tab"><div id="tb-termsProp-1"><div class="leftCol mgCol"><div>
<div class="portlet mgSection" id="ID_100">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln">Lymphangioleiomyomatosis (LAM) is a condition that affects the lungs, the kidneys, and the lymphatic system. The lymphatic system consists of a network of vessels that transport lymph fluid and immune cells throughout the body. Lymph fluid helps exchange immune cells, proteins, and other substances between the blood and tissues.<br /><br />LAM is found almost exclusively in women. It often occurs as a feature of an inherited syndrome called tuberous sclerosis complex. When LAM occurs alone it is called isolated or sporadic LAM.<br /><br />Signs and symptoms of LAM most often appear during a woman's thirties. Affected women have an overgrowth of abnormal smooth muscle-like cells (LAM cells) in the lungs, resulting in the formation of lung cysts and the destruction of normal lung tissue. They may also have an accumulation of fluid in the cavity around the lungs (chylothorax).<br /><br />The lung abnormalities resulting from LAM may cause difficulty breathing (dyspnea), chest pain, and coughing, which may bring up blood (hemoptysis). Many women with this disorder have recurrent episodes of collapsed lung (spontaneous pneumothorax). The lung problems may be progressive and, without lung transplantation, may eventually lead to limitations in activities of daily living, the need for oxygen therapy, and respiratory failure. Although LAM cells are not considered cancerous, they may spread between tissues (metastasize). As a result, the condition may recur even after lung transplantation.<br /><br />Women with LAM may develop cysts in the lymphatic vessels of the chest and abdomen. These cysts are called lymphangioleiomyomas. Affected women may also develop tumors called angiomyolipomas made up of LAM cells, fat cells, and blood vessels. Angiomyolipomas usually develop in the kidneys. Internal bleeding is a common complication of angiomyolipomas. [from <a title="MedlinePlus Genetics" href="https://medlineplus.gov/genetics/" class="defSource" target="_blank">MedlinePlus Genetics</a>]</div>
</div>
<div class="portlet mgSection" id="ID_102">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Clinical_features">Clinical features</h1><a sid="102" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat"><strong>From HPO</strong><br />
<div class="divPopper rprt" id="clin_852455"><div><strong>Pulmonary lymphangiomyomatosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>852455</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0238399</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">Infiltration of smooth muscle-like cells in lymph vessels as well as the lung (pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum). Focal emphysema can develop because of airway narrowing, and the thoracic duct may be obliterated. Pulmonary lymphangiomyomatosis may lead to multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/852455">Feature record</a> | <a href="/medgen?term=%22Pulmonary%20lymphangiomyomatosis%22%5BClinical%20Features%5D%20OR%20852455%5Buid%5D">Search on this feature</a></div></div><div class="TreeLite"><ul><li><span class="TLline">Neoplasm</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_852455" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Pulmonary lymphangiomyomatosis</a></span></li></ul></li></ul></div></div>
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<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li><li><a href="#tabORDO">Orphanet</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C0751674[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=148366">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=148366" target="_blank" href="/omim/605284">O</a></span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=148366" ref="ncbi_uid=148366">V</a></span></span><span class="TLline">Lymphangiomyomatosis</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/867443" ref="tree=MeSH" title="MedGen record for Phenotypic abnormality">Phenotypic abnormality</a></span><ul><li><span class="TLline"><a href="/medgen/867388" ref="tree=MeSH" title="MedGen record for Abnormality of the immune system">Abnormality of the immune system</a></span><ul><li><span class="TLline"><a href="/medgen/892707" ref="tree=MeSH" title="MedGen record for Abnormality of the lymphatic system">Abnormality of the lymphatic system</a></span><ul><li><span class="TLline"><a href="/medgen/867589" ref="tree=MeSH" title="MedGen record for Abnormal lymphatic vessel morphology">Abnormal lymphatic vessel morphology</a></span><ul><li><span class="TLline"><a href="/medgen/64616" ref="tree=MeSH" title="MedGen record for Lymphatic vessel neoplasm">Lymphatic vessel neoplasm</a></span><ul><li><span class="TLline"><a href="/medgen/44216" ref="tree=MeSH" title="MedGen record for Lymphangiomyoma">Lymphangiomyoma</a></span><ul><li><span class="matched_ds">Lymphangiomyomatosis</span><ul><li><span class="TLline"><a href="/medgen/91161" ref="tree=MeSH" title="MedGen record for Lung lymphangioleiomyomatosis">Lung lymphangioleiomyomatosis</a></span></li><li><span class="TLline"><a href="/medgen/90948" ref="tree=MeSH" title="MedGen record for Skin Lymphangioleiomyomatosis">Skin Lymphangioleiomyomatosis</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div><div id="tabORDO">Follow <a target="_blank" href="http://www.orpha.net/consor/cgi-bin/Disease_Classif.php?lng=EN&amp;data_id=156&amp;PatId=3386&amp;search=Disease_Classif_Simple&amp;new=1" class="ital bold">this link</a> to review classifications for <span class="ital">Lymphangiomyomatosis</span> in Orphanet.</div></div></div>
</div>
<div class="portlet mgSection" id="ID_105">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/36085075">Rapamycin nanoparticles improves drug bioavailability in PLAM treatment by interstitial injection.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shi Y,
Jiao C,
Lu X,
Nie Y,
Li X,
Han D</span><br />
<span class="medgenPMjournal">Orphanet J Rare Dis</span>
2022 Sep 9;17(1):349.
doi: 10.1186/s13023-022-02511-6.
<span class="bold">PMID: </span><a href="/pubmed/36085075" target="_blank">36085075</a><a href="/pmc/articles/PMC9463820" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22lymphangiomyomatosis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (1)</a></div><h3 class="subhead">Curated<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpCurated"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3><h3 class="nl vspace"><a href="https://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf" target="_blank">NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024</a></h3>
</div>
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<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/36360169">The Impact of TSC-1 and -2 Mutations on Response to Therapy in Malignant PEComa: A Multicenter Retrospective Analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Liu L,
Dehner C,
Grandhi N,
Lyu Y,
Borcherding DC,
Chrisinger JSA,
Zhang X,
Luo J,
Tao Y,
Parkes A,
Bui NQ,
Davis EJ,
Milhem MM,
Monga V,
Weiss M,
Tine BV,
Hirbe AC</span><br />
<span class="medgenPMjournal">Genes (Basel)</span>
2022 Oct 24;13(11)
doi: 10.3390/genes13111932.
<span class="bold">PMID: </span><a href="/pubmed/36360169" target="_blank">36360169</a><a href="/pmc/articles/PMC9689779" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34767702">Left uniportal VATS for lung decortication after chemical pleurodesis under spontaneous breathing in patient with lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Mantovani S,
Vannucci J,
Bassi M,
Mottola E,
Venuta F,
Anile M</span><br />
<span class="medgenPMjournal">Multimed Man Cardiothorac Surg</span>
2021 Oct 13;2021
doi: 10.1510/mmcts.2021.061.
<span class="bold">PMID: </span><a href="/pubmed/34767702" target="_blank">34767702</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26618803">RETINAL ASTROCYTIC HAMARTOMA: Optical Coherence Tomography Classification and Correlation With Tuberous Sclerosis Complex.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Pichi F,
Massaro D,
Serafino M,
Carrai P,
Giuliari GP,
Shields CL,
Veronese C,
Ciardella AP,
Nucci P</span><br />
<span class="medgenPMjournal">Retina</span>
2016 Jun;36(6):1199-208.
doi: 10.1097/IAE.0000000000000829.
<span class="bold">PMID: </span><a href="/pubmed/26618803" target="_blank">26618803</a></div>
<div class="nl"><a target="_blank" href="/pubmed/16359539">PEComa: what do we know so far?</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hornick JL,
Fletcher CD</span><br />
<span class="medgenPMjournal">Histopathology</span>
2006 Jan;48(1):75-82.
doi: 10.1111/j.1365-2559.2005.02316.x.
<span class="bold">PMID: </span><a href="/pubmed/16359539" target="_blank">16359539</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1554228">Pulmonary lymphangiomyomatosis treated by single lung transplantation.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sleiman C,
Mal H,
Jebrak G,
Darne C,
Meeus E,
Dubois F,
Luisetti M,
Fournier M,
Pariente R,
Andreassian B</span><br />
<span class="medgenPMjournal">Am Rev Respir Dis</span>
1992 Apr;145(4 Pt 1):964-6.
doi: 10.1164/ajrccm/145.4_Pt_1.964.
<span class="bold">PMID: </span><a href="/pubmed/1554228" target="_blank">1554228</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Lymphangiomyomatosis%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (35)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/36952511">Resection of Extrapulmonary Lymphangiomyoma and Post-Operative Management Considerations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Abramov R,
Rufizen L,
Gilshtein H</span><br />
<span class="medgenPMjournal">Vasc Endovascular Surg</span>
2023 Oct;57(7):756-759.
Epub 2023 Mar 23
doi: 10.1177/15385744221144503.
<span class="bold">PMID: </span><a href="/pubmed/36952511" target="_blank">36952511</a></div>
<div class="nl"><a target="_blank" href="/pubmed/10969906">Pulmonary lymphangiomyomatosis (LAM) developing chylothorax.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Morimoto N,
Hirasaki S,
Kamei T,
Horiike A,
Miyatake H,
Ogita Y,
Nakano H</span><br />
<span class="medgenPMjournal">Intern Med</span>
2000 Sep;39(9):738-41.
doi: 10.2169/internalmedicine.39.738.
<span class="bold">PMID: </span><a href="/pubmed/10969906" target="_blank">10969906</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1487466">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Aerts P,
Van Fraeyenhoven L,
Verschakelen J</span><br />
<span class="medgenPMjournal">J Belge Radiol</span>
1992 Oct;75(5):404-5.
<span class="bold">PMID: </span><a href="/pubmed/1487466" target="_blank">1487466</a></div>
<div class="nl"><a target="_blank" href="/pubmed/6698139">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">El Allaf D,
Borlee G,
Hadjoudj H,
Henrard L,
Marcelle R,
van Cauwenberge H</span><br />
<span class="medgenPMjournal">Eur J Respir Dis</span>
1984 Feb;65(2):147-52.
<span class="bold">PMID: </span><a href="/pubmed/6698139" target="_blank">6698139</a></div>
<div class="nl"><a target="_blank" href="/pubmed/453961">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Shuman RL,
Engelman R,
Kittle CF</span><br />
<span class="medgenPMjournal">Ann Thorac Surg</span>
1979 Jan;27(1):70-5.
doi: 10.1016/s0003-4975(10)62975-x.
<span class="bold">PMID: </span><a href="/pubmed/453961" target="_blank">453961</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Lymphangiomyomatosis%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (79)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/36952511">Resection of Extrapulmonary Lymphangiomyoma and Post-Operative Management Considerations.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Abramov R,
Rufizen L,
Gilshtein H</span><br />
<span class="medgenPMjournal">Vasc Endovascular Surg</span>
2023 Oct;57(7):756-759.
Epub 2023 Mar 23
doi: 10.1177/15385744221144503.
<span class="bold">PMID: </span><a href="/pubmed/36952511" target="_blank">36952511</a></div>
<div class="nl"><a target="_blank" href="/pubmed/10969906">Pulmonary lymphangiomyomatosis (LAM) developing chylothorax.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Morimoto N,
Hirasaki S,
Kamei T,
Horiike A,
Miyatake H,
Ogita Y,
Nakano H</span><br />
<span class="medgenPMjournal">Intern Med</span>
2000 Sep;39(9):738-41.
doi: 10.2169/internalmedicine.39.738.
<span class="bold">PMID: </span><a href="/pubmed/10969906" target="_blank">10969906</a></div>
<div class="nl"><a target="_blank" href="/pubmed/3184096">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Eisera NB,
Venegoni E,
Colombi R</span><br />
<span class="medgenPMjournal">J R Soc Med</span>
1988 Oct;81(10):607-9.
doi: 10.1177/014107688808101021.
<span class="bold">PMID: </span><a href="/pubmed/3184096" target="_blank">3184096</a><a href="/pmc/articles/PMC1291813" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/3586575">Lymphangiomyomatosis: a report of three cases treated with tamoxifen.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Clemm C,
Jehn U,
Wolf-Hornung B,
Siemon G,
Walter G</span><br />
<span class="medgenPMjournal">Klin Wochenschr</span>
1987 Apr 15;65(8):391-3.
doi: 10.1007/BF01745582.
<span class="bold">PMID: </span><a href="/pubmed/3586575" target="_blank">3586575</a></div>
<div class="nl"><a target="_blank" href="/pubmed/7432404">Pulmonary lymphangiomyomatosis responsive to progesterone.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McCarty KS Jr,
Mossler JA,
McLelland R,
Sieker HO</span><br />
<span class="medgenPMjournal">N Engl J Med</span>
1980 Dec 18;303(25):1461-5.
doi: 10.1056/NEJM198012183032506.
<span class="bold">PMID: </span><a href="/pubmed/7432404" target="_blank">7432404</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Lymphangiomyomatosis%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (27)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/36360169">The Impact of TSC-1 and -2 Mutations on Response to Therapy in Malignant PEComa: A Multicenter Retrospective Analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Liu L,
Dehner C,
Grandhi N,
Lyu Y,
Borcherding DC,
Chrisinger JSA,
Zhang X,
Luo J,
Tao Y,
Parkes A,
Bui NQ,
Davis EJ,
Milhem MM,
Monga V,
Weiss M,
Tine BV,
Hirbe AC</span><br />
<span class="medgenPMjournal">Genes (Basel)</span>
2022 Oct 24;13(11)
doi: 10.3390/genes13111932.
<span class="bold">PMID: </span><a href="/pubmed/36360169" target="_blank">36360169</a><a href="/pmc/articles/PMC9689779" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/31847710">Renal angiomyolipoma (AML) harboring a missense mutation of TSC2 with copy-neutral loss of heterozygosity (CN-LOH).</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Idogawa M,
Hida T,
Tanaka T,
Ohira N,
Tange S,
Sasaki Y,
Uhara H,
Masumori N,
Tokino T,
Natori H</span><br />
<span class="medgenPMjournal">Cancer Biol Ther</span>
2020 Apr 2;21(4):315-319.
Epub 2019 Dec 17
doi: 10.1080/15384047.2019.1702406.
<span class="bold">PMID: </span><a href="/pubmed/31847710" target="_blank">31847710</a><a href="/pmc/articles/PMC7515489" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/16359539">PEComa: what do we know so far?</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hornick JL,
Fletcher CD</span><br />
<span class="medgenPMjournal">Histopathology</span>
2006 Jan;48(1):75-82.
doi: 10.1111/j.1365-2559.2005.02316.x.
<span class="bold">PMID: </span><a href="/pubmed/16359539" target="_blank">16359539</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1554228">Pulmonary lymphangiomyomatosis treated by single lung transplantation.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sleiman C,
Mal H,
Jebrak G,
Darne C,
Meeus E,
Dubois F,
Luisetti M,
Fournier M,
Pariente R,
Andreassian B</span><br />
<span class="medgenPMjournal">Am Rev Respir Dis</span>
1992 Apr;145(4 Pt 1):964-6.
doi: 10.1164/ajrccm/145.4_Pt_1.964.
<span class="bold">PMID: </span><a href="/pubmed/1554228" target="_blank">1554228</a></div>
<div class="nl"><a target="_blank" href="/pubmed/3370906">Early onset of childhood pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Nussbaum E,
Groncy P,
Finklestein J,
Dickman P,
Harrell JH 3rd</span><br />
<span class="medgenPMjournal">Clin Pediatr (Phila)</span>
1988 Jun;27(6):279-84.
doi: 10.1177/000992288802700603.
<span class="bold">PMID: </span><a href="/pubmed/3370906" target="_blank">3370906</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Lymphangiomyomatosis%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (30)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/36360169">The Impact of TSC-1 and -2 Mutations on Response to Therapy in Malignant PEComa: A Multicenter Retrospective Analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Liu L,
Dehner C,
Grandhi N,
Lyu Y,
Borcherding DC,
Chrisinger JSA,
Zhang X,
Luo J,
Tao Y,
Parkes A,
Bui NQ,
Davis EJ,
Milhem MM,
Monga V,
Weiss M,
Tine BV,
Hirbe AC</span><br />
<span class="medgenPMjournal">Genes (Basel)</span>
2022 Oct 24;13(11)
doi: 10.3390/genes13111932.
<span class="bold">PMID: </span><a href="/pubmed/36360169" target="_blank">36360169</a><a href="/pmc/articles/PMC9689779" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35483879">Tuberous sclerosis complex: a complex case.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Powell RM,
Pattison S,
Moravec JC,
Bhat B,
Guirguis N,
Markie D,
Jones GT,
Copedo J,
Print CG,
Morison IM,
Gavryushkin A,
Gray B,
Wyeth LJ,
Eccles MR,
Macaulay EC</span><br />
<span class="medgenPMjournal">Cold Spring Harb Mol Case Stud</span>
2022 Apr;8(3)
Epub 2022 Apr 28
doi: 10.1101/mcs.a006182.
<span class="bold">PMID: </span><a href="/pubmed/35483879" target="_blank">35483879</a><a href="/pmc/articles/PMC9059781" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/23729367">Exacerbation and remission of pulmonary micronodules with lymphangioleiomyomatosis around the time of childbirth.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ogawa R,
Miyagawa M,
Ide K,
Akamune A,
Ohtsuki Y,
Mochizuki T</span><br />
<span class="medgenPMjournal">Jpn J Radiol</span>
2013 Sep;31(9):633-6.
Epub 2013 Jun 2
doi: 10.1007/s11604-013-0221-9.
<span class="bold">PMID: </span><a href="/pubmed/23729367" target="_blank">23729367</a></div>
<div class="nl"><a target="_blank" href="/pubmed/1487466">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Aerts P,
Van Fraeyenhoven L,
Verschakelen J</span><br />
<span class="medgenPMjournal">J Belge Radiol</span>
1992 Oct;75(5):404-5.
<span class="bold">PMID: </span><a href="/pubmed/1487466" target="_blank">1487466</a></div>
<div class="nl"><a target="_blank" href="/pubmed/6698139">Pulmonary lymphangiomyomatosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">El Allaf D,
Borlee G,
Hadjoudj H,
Henrard L,
Marcelle R,
van Cauwenberge H</span><br />
<span class="medgenPMjournal">Eur J Respir Dis</span>
1984 Feb;65(2):147-52.
<span class="bold">PMID: </span><a href="/pubmed/6698139" target="_blank">6698139</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Lymphangiomyomatosis%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (30)</a></div></div>
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<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C0751674%5bDISCUI%5d&amp;filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (46)</a></li>
<li><a href="/gtr/tests?term=C0751674%5bDISCUI%5d&amp;filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (49)</a></li>
<li><a href="/gtr/tests?term=C0751674%5bDISCUI%5d&amp;filter=method%3A2%5F19" target="_blank">Targeted variant analysis (2)</a></li>
<li class="portletSeeAll portletSeeAllPad"><total><a href="/gtr/tests?term=C0751674%5bDISCUI%5d" target="_blank">See all (59)</a></total></li>
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