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</div> </div> </nav> <h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Hereditary leiomyomatosis and renal cell cancer syndrome</span></h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox mw-interlanguage-selector" aria-label="Go to an article in another language. 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id="mw-content-subtitle"><span class="mw-redirectedfrom">(Redirected from <a href="/w/index.php?title=Multiple_cutaneous_leiomyoma&redirect=no" class="mw-redirect" title="Multiple cutaneous leiomyoma">Multiple cutaneous leiomyoma</a>)</span></div></div> <div id="mw-content-text" class="mw-body-content"><div class="mw-content-ltr mw-parser-output" lang="en" dir="ltr"><p class="mw-empty-elt"> </p> <div class="shortdescription nomobile noexcerpt noprint searchaux" style="display:none">Medical condition</div><style data-mw-deduplicate="TemplateStyles:r1257001546">.mw-parser-output .infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Reed’s syndrome</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data"><b>Familial leiomyomatosis cutis et uteri</b></td></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/b/bf/Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg/220px-Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg" decoding="async" width="220" height="330" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/b/bf/Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg/330px-Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/b/bf/Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg/440px-Hereditary_leiomyomatosis_and_renal_cell_carcinoma_associated_RCC_--_high_mag.jpg 2x" data-file-width="2848" data-file-height="4272" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data"><a href="/wiki/Micrograph" title="Micrograph">Micrograph</a> showing the characteristic hyalinized papillary cores found in some hereditary leiomyomatosis and renal cell carcinoma syndrome-associated renal cell carcinomas. <a href="/wiki/H%26E_stain" title="H&E stain">H&E stain</a>.</td></tr></tbody></table> <p><b>Hereditary leiomyomatosis and renal cell carcinoma</b> (<b>HLRCC</b>) or <b>Reed's syndrome</b> is rare autosomal dominant disorder associated with benign <a href="/wiki/Smooth_muscle" title="Smooth muscle">smooth muscle</a> tumors and an increased risk of <a href="/wiki/Renal_cell_carcinoma" title="Renal cell carcinoma">renal cell carcinoma</a>. It is characterised by <a href="/wiki/Multiple_cutaneous_leiomyoma" class="mw-redirect" title="Multiple cutaneous leiomyoma">multiple cutaneous leiomyomas</a> and, in women, <a href="/wiki/Uterine_fibroid" title="Uterine fibroid">uterine leiomyomas</a>. It predisposes individuals to <a href="/wiki/Renal_cell_cancer" class="mw-redirect" title="Renal cell cancer">renal cell cancer</a>, an association denominated hereditary leiomyomatosis and renal cell cancer.<sup id="cite_ref-Tolvanen2012_1-0" class="reference"><a href="#cite_note-Tolvanen2012-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Toro2003_2-0" class="reference"><a href="#cite_note-Toro2003-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> It is also associated with increased risk of uterine <a href="/wiki/Leiomyosarcoma" title="Leiomyosarcoma">leiomyosarcoma</a>.<sup id="cite_ref-Pithukpakorn2006_3-0" class="reference"><a href="#cite_note-Pithukpakorn2006-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> The syndrome is caused by a mutation in the <a href="/wiki/Fumarate_hydratase" class="mw-redirect" title="Fumarate hydratase">fumarate hydratase</a> gene, which leads to an accumulation of <a href="/wiki/Fumaric_acid" title="Fumaric acid">fumarate</a>. The inheritance pattern is <a href="/wiki/Genetic_disorder#Autosomal_dominant" title="Genetic disorder">autosomal dominant</a> and screening can typically begin in childhood. </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Almost all women present with <a href="/wiki/Uterine_fibroid" title="Uterine fibroid">uterine fibroids</a>, approximately 76% with dermal manifestations and 10–16% with renal tumors.<sup id="cite_ref-Pithukpakorn2006_3-1" class="reference"><a href="#cite_note-Pithukpakorn2006-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>The uterine fibroids tend to occur at younger age and are larger and more numerous than in the general population. They may be distinguishable from sporadic fibroids by special histological features such as prominent nucleoli with perinucleolar halos.<sup id="cite_ref-Garg_2011_4-0" class="reference"><a href="#cite_note-Garg_2011-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p><p>The skin presentation is of asymmetrical, reddish-brown nodules or papules with a firm consistency, predominantly located on the limbs (<a href="/wiki/Multiple_cutaneous_leiomyoma" class="mw-redirect" title="Multiple cutaneous leiomyoma">multiple cutaneous leiomyoma</a>), although they may occur anywhere, including the face. The lesions, which are typically painful and most often present during the third decade of life, are <a href="/w/index.php?title=Piloleiomyomata&action=edit&redlink=1" class="new" title="Piloleiomyomata (page does not exist)">piloleiomyomata</a>—a benign <a href="/wiki/Smooth_muscle" title="Smooth muscle">smooth muscle</a> tumour arising from the <a href="/wiki/Arrectores_pilorum" class="mw-redirect" title="Arrectores pilorum">arrectores pilorum</a> muscles of the skin. These tumours may also arise in the <a href="/wiki/Dartos_fascia" title="Dartos fascia">tunica dartos</a> of the <a href="/wiki/Scrotum" title="Scrotum">scrotum</a> and the mammillary muscle of the nipple (<a href="/wiki/Genital_leiomyoma" title="Genital leiomyoma">genital leiomyoma</a>), the smooth muscle of blood vessels (<a href="/wiki/Angioleiomyoma" title="Angioleiomyoma">angioleiomyoma</a>) and the lung (<a href="/wiki/Pulmonary_lymphangioleiomyomatosis" class="mw-redirect" title="Pulmonary lymphangioleiomyomatosis">pulmonary lymphangioleiomyomatosis</a>).<sup id="cite_ref-Kogan2001_5-0" class="reference"><a href="#cite_note-Kogan2001-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> A pseudo-<a href="/wiki/Darier_sign" class="mw-redirect" title="Darier sign">Darier sign</a> may be present. </p><p>The renal cell carcinoma tends to be of the papillary (type 2) form and tends to occur more commonly in women than men with this syndrome. These cancers present earlier than is usual for renal cell carcinomas (typically in the twenties and thirties) and tend to be at relatively advanced stages at presentation. Tumours have rarely been reported in children. These tumours occur in ~20% of those with this mutation suggesting that other factors are involved in the pathogenesis. </p><p>These growths increase in size and number throughout the lifetime.<sup id="cite_ref-NORD_6-0" class="reference"><a href="#cite_note-NORD-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> People with HLRCC have an approximately 15% chance of developing <a href="/wiki/Renal_cell_carcinoma" title="Renal cell carcinoma">renal cell carcinoma</a> in their lifetime.<sup id="cite_ref-menko_7-0" class="reference"><a href="#cite_note-menko-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> This is most commonly <a href="/w/index.php?title=Type_II_papillary_renal_cell_carcinoma&action=edit&redlink=1" class="new" title="Type II papillary renal cell carcinoma (page does not exist)">type II papillary renal cell carcinoma</a>, which is an aggressive form.<sup id="cite_ref-NORD_6-1" class="reference"><a href="#cite_note-NORD-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Associated_conditions">Associated conditions</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=2" title="Edit section: Associated conditions"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Other relatively rare conditions have been reported in association with this disease. It is not yet known if these associations are fortuitous or manifestations of the condition itself. </p><p>Cerebral <a href="/wiki/Cavernoma" class="mw-redirect" title="Cavernoma">cavernomas</a> and massive, macronodular adrenocortical disease have also been reported in association with this syndrome.<sup id="cite_ref-Campione2007_8-0" class="reference"><a href="#cite_note-Campione2007-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Matyakhina2005_9-0" class="reference"><a href="#cite_note-Matyakhina2005-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> A case of <a href="/wiki/Cutis_verticis_gyrata" title="Cutis verticis gyrata">cutis verticis gyrata</a>, disseminated <a href="/wiki/Collagenoma" class="mw-redirect" title="Collagenoma">collagenoma</a> and <a href="/wiki/Charcot%E2%80%93Marie%E2%80%93Tooth_disease" title="Charcot–Marie–Tooth disease">Charcot–Marie–Tooth disease</a> in association with a mutation in the fumarate hydratase gene has also been reported.<sup id="cite_ref-Marque2010_10-0" class="reference"><a href="#cite_note-Marque2010-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> Two cases of ovarian mucinous cystadenoma have also been reported with this mutation.<sup id="cite_ref-Ylisaukko-oja2006_11-0" class="reference"><a href="#cite_note-Ylisaukko-oja2006-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=3" title="Edit section: Cause"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>HLRCC is an <a href="/wiki/Autosomal_dominant" class="mw-redirect" title="Autosomal dominant">autosomal dominant</a> condition caused by a mutation in the <a href="/wiki/Fumarate_hydratase" class="mw-redirect" title="Fumarate hydratase">FH</a> gene, which results in dysfunction of the <a href="/wiki/Citric_acid_cycle" title="Citric acid cycle">citric acid cycle</a>, leading to an accumulation of <a href="/wiki/Fumarate" class="mw-redirect" title="Fumarate">fumarate</a>.<sup id="cite_ref-menko_7-1" class="reference"><a href="#cite_note-menko-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-GHR_12-0" class="reference"><a href="#cite_note-GHR-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p><p>The fumarate hydratase gene is located on the long arm of <a href="/wiki/Chromosome_1" title="Chromosome 1">chromosome 1</a> (1q42.3-43), spans 22 kilobases and has 10 <a href="/wiki/Exon" title="Exon">exons</a>; the first exon codes for a <a href="/wiki/Signal_peptide" title="Signal peptide">signal peptide</a>. </p> <div class="mw-heading mw-heading2"><h2 id="Pathogenesis">Pathogenesis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=4" title="Edit section: Pathogenesis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>While the pathogenetic mechanisms underlying the lesions remain unclear, it has been suggested that the accumulation of fumarate may lead to overexpression of the <a href="/wiki/Aldo-keto_reductase" title="Aldo-keto reductase">aldo-keto reductase</a> enzyme, <a href="/wiki/AKR1B10" title="AKR1B10">AKR1B10</a>.<sup id="cite_ref-Ooi2011_13-0" class="reference"><a href="#cite_note-Ooi2011-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> It has also been found that fumarate is present in the <a href="/wiki/Mitochondrion" title="Mitochondrion">mitochondria</a> and in the <a href="/wiki/Cytoplasm" title="Cytoplasm">cytoplasm</a>. The cytoplasmic form appears to have a role in the protection of DNA from molecular injury.<sup id="cite_ref-Yogev2010_14-0" class="reference"><a href="#cite_note-Yogev2010-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> Fumarate has been shown to be a competitive inhibitor of <a href="/wiki/Prolyl_hydroxylase" class="mw-redirect" title="Prolyl hydroxylase">prolyl hydroxylase</a>. This inhibition leads to the stabilisation of a number of <a href="/wiki/Hypoxia-inducible_factors" class="mw-redirect" title="Hypoxia-inducible factors">hypoxia-inducible factors</a> which are thought to predispose to tumorigenesis. An alternative pathway for the metabolism of fumarate in the presence of these mutations has been described.<sup id="cite_ref-Frezza2011_15-0" class="reference"><a href="#cite_note-Frezza2011-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup> Other genes involved affected by this mutation are <a href="/wiki/Keap1" class="mw-redirect" title="Keap1">Keap1</a>, <a href="/wiki/Nrf2" class="mw-redirect" title="Nrf2">Nrf2</a> and <a href="/wiki/HMOX1" title="HMOX1">HMOX1</a>.<sup id="cite_ref-Kinch2011_16-0" class="reference"><a href="#cite_note-Kinch2011-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=5" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The diagnosis is made either by testing of the fumarate hydratase activity in cultured skin <a href="/wiki/Fibroblast" title="Fibroblast">fibroblasts</a> or lymphoblastoid cells and demonstrating reduced activity (≤60%) or by molecular <a href="/wiki/Genetic_testing" title="Genetic testing">genetic testing</a>.<sup id="cite_ref-Pithukpakorn2006_3-2" class="reference"><a href="#cite_note-Pithukpakorn2006-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> Special histologic features of fibroids may allow an early diagnosis in absence of other symptoms.<sup id="cite_ref-Garg_2011_4-1" class="reference"><a href="#cite_note-Garg_2011-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading3"><h3 id="Histology">Histology</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=6" title="Edit section: Histology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The skin lesions may be difficult to diagnose clinically but a <a href="/wiki/Punch_biopsy" class="mw-redirect" title="Punch biopsy">punch biopsy</a> will usually reveal a <a href="/w/index.php?title=Grenz_zone&action=edit&redlink=1" class="new" title="Grenz zone (page does not exist)">Grenz zone</a> <a rel="nofollow" class="external autonumber" href="http://medical-dictionary.thefreedictionary.com/Grenz+Zone">[1]</a> separating the tumour from the overlying skin. Histological examination shows dense dermal nodules composed of elongated cells with abundant eosinophilic <a href="/wiki/Cytoplasm" title="Cytoplasm">cytoplasm</a> arranged in <a href="/wiki/Nerve_fascicle" title="Nerve fascicle">fascicles</a> (spindle cells). The <a href="/wiki/Cell_nucleus" title="Cell nucleus">nuclei</a> are uniform, blunt-ended and cigar-shaped with only occasional <a href="/wiki/Mitosis" title="Mitosis">mitoses</a>. Special stains that may be of use in the diagnosis include <a href="/wiki/Masson%27s_trichrome" class="mw-redirect" title="Masson's trichrome">Masson's trichrome</a>, <a href="/wiki/Van_Gieson%27s_stain" title="Van Gieson's stain">Van Gieson's stain</a> and <a href="/wiki/Phosphotungstic_acid" title="Phosphotungstic acid">phosphotungstic acid</a>–<a href="/wiki/Haematoxylin" title="Haematoxylin">haematoxylin</a>. </p><p>The renal cell carcinomas have prominent eosinophilic <a href="/wiki/Nucleolus" title="Nucleolus">nucleoli</a> surrounded by a clear halo. </p> <div class="mw-heading mw-heading3"><h3 id="Differential_diagnosis">Differential diagnosis</h3><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=7" title="Edit section: Differential diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Differential diagnosis of this condition includes the <a href="/wiki/Birt%E2%80%93Hogg%E2%80%93Dub%C3%A9_syndrome" title="Birt–Hogg–Dubé syndrome">Birt–Hogg–Dubé syndrome</a> and <a href="/wiki/Tuberous_sclerosis" title="Tuberous sclerosis">tuberous sclerosis</a>. As the skin lesions are typically painful, it is also often necessary to exclude other painful tumors of the skin (including <a href="/wiki/Blue_rubber_bleb_nevus" class="mw-redirect" title="Blue rubber bleb nevus">blue rubber bleb nevus</a>, <a href="/wiki/Leiomyoma" title="Leiomyoma">leiomyoma</a>, <a href="/wiki/Eccrine_spiradenoma" class="mw-redirect" title="Eccrine spiradenoma">eccrine spiradenoma</a>, <a href="/wiki/Neuroma" title="Neuroma">neuroma</a>, <a href="/wiki/Dermatofibroma" title="Dermatofibroma">dermatofibroma</a>, <a href="/wiki/Angiolipoma" title="Angiolipoma">angiolipoma</a>, <a href="/wiki/Neurilemmoma" class="mw-redirect" title="Neurilemmoma">neurilemmoma</a>, <a href="/wiki/Endometrioma" title="Endometrioma">endometrioma</a>, <a href="/wiki/Glomus_tumor" title="Glomus tumor">glomus tumor</a> and <a href="/wiki/Granular_cell_tumor" title="Granular cell tumor">granular cell tumor</a>; the mnemonic "BLEND-AN-EGG" may be helpful). Other skin lesions that may need to be considered include <a href="/wiki/Dermal_cylindroma" title="Dermal cylindroma">cylindroma</a>, <a href="/wiki/Lipoma" title="Lipoma">lipoma</a>, <a href="/wiki/Poroma" title="Poroma">poroma</a> and <a href="/wiki/Trichoepithelioma" title="Trichoepithelioma">trichoepithelioma</a>; these tend to be painless and have other useful distinguishing features. </p> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=8" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Leiomyomas do not typically require treatment unless they cause pain.<sup id="cite_ref-NORD_6-2" class="reference"><a href="#cite_note-NORD-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> The skin lesions may be difficult to treat as they tend to recur after excision or destructive treatment. Drugs which affect smooth muscle contraction, such as <a href="/wiki/Doxazosin" title="Doxazosin">doxazosin</a>, <a href="/wiki/Nitroglycerine" class="mw-redirect" title="Nitroglycerine">nitroglycerine</a>, <a href="/wiki/Nifedipine" title="Nifedipine">nifedipine</a> and <a href="/wiki/Phenoxybenzamine" title="Phenoxybenzamine">phenoxybenzamine</a>, may provide pain relief. </p><p><a href="/wiki/Uterine_fibroid" title="Uterine fibroid">Uterine fibroids</a> can be treated with the same methods as sporadic uterine fibroids including anti-hormonal treatment, surgery, or <a href="/wiki/Embolisation" class="mw-redirect" title="Embolisation">embolisation</a>. Substantially elevated risk of progression to or independent development of uterine leiomyosarcoma has been reported which may influence treatment methods.<sup id="cite_ref-Pithukpakorn2006_3-3" class="reference"><a href="#cite_note-Pithukpakorn2006-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>The predisposition to renal cell cancer calls for screening and, if necessary, <a href="/wiki/Urologist" class="mw-redirect" title="Urologist">urological</a> management. </p><p>Topical lidocaine patches have been reported to decrease the severity and frequency of pain associated with cutaneous leiomyomas.<sup id="cite_ref-17" class="reference"><a href="#cite_note-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Prognosis">Prognosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=9" title="Edit section: Prognosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>A 2006 review stated that Reed's Syndrome often is the leading cause of renal cancer between ages 30–50. Renal cancer kills about 1 in 3 people, but 5-year survival rates improved between 1974–1976 and 1995–2000, from 52% to 64%.<sup id="cite_ref-18" class="reference"><a href="#cite_note-18"><span class="cite-bracket">[</span>18<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="History">History</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=10" title="Edit section: History"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The syndrome was first described by Reed <i>et al</i> in 1973.<sup id="cite_ref-Reed1973_19-0" class="reference"><a href="#cite_note-Reed1973-19"><span class="cite-bracket">[</span>19<span class="cite-bracket">]</span></a></sup> The link with the fumarate hydratase gene was uncovered in 2002.<sup id="cite_ref-Tomlinson2002_20-0" class="reference"><a href="#cite_note-Tomlinson2002-20"><span class="cite-bracket">[</span>20<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=11" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>HLRCC affects males and females equally.<sup id="cite_ref-NORD_6-3" class="reference"><a href="#cite_note-NORD-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> Thus far, HLRCC has been found in 300 families worldwide.<sup id="cite_ref-GHR_12-1" class="reference"><a href="#cite_note-GHR-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Notes">Notes</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=12" title="Edit section: Notes"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>A database of the mutations of the fumarate hydratase gene is available.<sup id="cite_ref-Bayley2008_21-0" class="reference"><a href="#cite_note-Bayley2008-21"><span class="cite-bracket">[</span>21<span class="cite-bracket">]</span></a></sup> Autosomal recessive mutations cause a serious neurological disease known as <a href="/wiki/Fumarase_deficiency" title="Fumarase deficiency">fumarase deficiency</a>, which is associated with a variety of congenital lesions in the brain. </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=13" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/Von_Hippel%E2%80%93Lindau_disease" title="Von Hippel–Lindau disease">Von Hippel–Lindau disease</a></li> <li><a href="/wiki/Birt-Hogg-Dube_syndrome" class="mw-redirect" title="Birt-Hogg-Dube syndrome">Birt-Hogg-Dube syndrome</a></li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hereditary_leiomyomatosis_and_renal_cell_cancer_syndrome&action=edit&section=14" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media 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style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q3270312" class="extiw" title="d:Q3270312">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></b>: <a rel="nofollow" class="external text" href="https://omim.org/entry/150800">150800</a> <a rel="nofollow" class="external text" href="https://omim.org/entry/136850">136850</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=C537112">C537112</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb34278.htm">34278</a></li></ul></div></div></td></tr></tbody></table></div> <ul><li><a rel="nofollow" class="external text" href="https://web.archive.org/web/20120224004212/http://rarediseases.info.nih.gov/GARD/Condition/10160/Reed_syndrome.aspx">Reed syndrome</a> and <a rel="nofollow" class="external text" href="https://rarediseases.info.nih.gov/diseases/10096/hereditary-leiomyomatosis-and-renal-cell-cancer">Hereditary leiomyomatosis and renal cell cancer</a> at the Genetic and Rare Diseases Information Center (GARD)</li></ul> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Glandular_and_epithelial_cancer" style="padding:3px"><table class="nowraplinks hlist mw-collapsible expanded navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output .navbar-brackets::after{margin-left:-0.125em;content:" ]"}.mw-parser-output .navbar li{word-spacing:-0.125em}.mw-parser-output .navbar a>span,.mw-parser-output .navbar a>abbr{text-decoration:inherit}.mw-parser-output .navbar-mini abbr{font-variant:small-caps;border-bottom:none;text-decoration:none;cursor:inherit}.mw-parser-output .navbar-ct-full{font-size:114%;margin:0 7em}.mw-parser-output .navbar-ct-mini{font-size:114%;margin:0 4em}html.skin-theme-clientpref-night .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}@media(prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .navbar li a abbr{color:var(--color-base)!important}}@media print{.mw-parser-output .navbar{display:none!important}}</style><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Epithelial_neoplasms" title="Template:Epithelial neoplasms"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Epithelial_neoplasms" title="Template talk:Epithelial neoplasms"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Epithelial_neoplasms" title="Special:EditPage/Template:Epithelial neoplasms"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Glandular_and_epithelial_cancer" style="font-size:114%;margin:0 4em">Glandular and epithelial <a href="/wiki/Cancer" title="Cancer">cancer</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Epithelium" title="Epithelium">Epithelium</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Papilloma" title="Papilloma">Papilloma</a>/<a href="/wiki/Carcinoma" title="Carcinoma">carcinoma</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Small-cell_carcinoma" title="Small-cell carcinoma">Small-cell carcinoma</a></li> <li><a href="/wiki/Combined_small-cell_lung_carcinoma" title="Combined small-cell lung carcinoma">Combined small-cell lung carcinoma</a></li> <li><a href="/wiki/Verrucous_carcinoma" title="Verrucous carcinoma">Verrucous carcinoma</a></li> <li><a href="/wiki/Squamous-cell_carcinoma" title="Squamous-cell carcinoma">Squamous-cell carcinoma</a></li> <li><a href="/wiki/Basal-cell_carcinoma" title="Basal-cell carcinoma">Basal-cell carcinoma</a></li> <li><a href="/wiki/Transitional_cell_carcinoma" title="Transitional cell carcinoma">Transitional cell carcinoma</a></li> <li><a href="/wiki/Inverted_papilloma" title="Inverted papilloma">Inverted papilloma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mixed_tumor" title="Mixed tumor">Complex epithelial</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Warthin%27s_tumor" title="Warthin's tumor">Warthin's tumor</a></li> <li><a href="/wiki/Thymoma" title="Thymoma">Thymoma</a></li> <li><a href="/wiki/Bartholin_gland_carcinoma" title="Bartholin gland carcinoma">Bartholin gland carcinoma</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Gland" title="Gland">Glands</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="Adenomas/adenocarcinomas" scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Adenoma" title="Adenoma">Adenomas</a>/<br /><a href="/wiki/Adenocarcinoma" title="Adenocarcinoma">adenocarcinomas</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Digestive_system_neoplasm" title="Digestive system neoplasm">Gastrointestinal</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i>tract:</i> <a href="/wiki/Linitis_plastica" title="Linitis plastica">Linitis plastica</a></li> <li><a href="/wiki/Familial_adenomatous_polyposis" title="Familial adenomatous polyposis">Familial adenomatous polyposis</a></li></ul> <ul><li><i>pancreas</i> <ul><li><a href="/wiki/Insulinoma" title="Insulinoma">Insulinoma</a></li> <li><a href="/wiki/Glucagonoma" title="Glucagonoma">Glucagonoma</a></li> <li><a href="/wiki/Gastrinoma" title="Gastrinoma">Gastrinoma</a></li> <li><a href="/wiki/VIPoma" title="VIPoma">VIPoma</a></li> <li><a href="/wiki/Somatostatinoma" title="Somatostatinoma">Somatostatinoma</a></li></ul></li></ul> <ul><li><a href="/wiki/Cholangiocarcinoma" title="Cholangiocarcinoma">Cholangiocarcinoma</a></li> <li><a href="/wiki/Klatskin_tumor" title="Klatskin tumor">Klatskin tumor</a></li> <li><a href="/wiki/Hepatocellular_adenoma" title="Hepatocellular adenoma">Hepatocellular adenoma</a>/<a href="/wiki/Hepatocellular_carcinoma" title="Hepatocellular carcinoma">Hepatocellular carcinoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Urogenital_neoplasm" title="Urogenital neoplasm">Urogenital</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Renal_cell_carcinoma" title="Renal cell carcinoma">Renal cell carcinoma</a></li> <li><a href="/wiki/Endometrioid_tumor" title="Endometrioid tumor">Endometrioid tumor</a></li> <li><a href="/wiki/Renal_oncocytoma" title="Renal oncocytoma">Renal oncocytoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Endocrine_gland_neoplasm" title="Endocrine gland neoplasm">Endocrine</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Prolactinoma" title="Prolactinoma">Prolactinoma</a></li> <li><a href="/wiki/Multiple_endocrine_neoplasia" title="Multiple endocrine neoplasia">Multiple endocrine neoplasia</a></li> <li><a href="/wiki/Adrenocortical_adenoma" title="Adrenocortical adenoma">Adrenocortical adenoma</a>/<a href="/wiki/Adrenocortical_carcinoma" title="Adrenocortical carcinoma">Adrenocortical carcinoma</a></li> <li><a href="/wiki/H%C3%BCrthle_cell" title="Hürthle cell">Hürthle cell</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other/multiple</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Neuroendocrine_tumor" title="Neuroendocrine tumor">Neuroendocrine tumor</a> <ul><li><a href="/wiki/Carcinoid" title="Carcinoid">Carcinoid</a></li></ul></li> <li><a href="/wiki/Adenoid_cystic_carcinoma" title="Adenoid cystic carcinoma">Adenoid cystic carcinoma</a></li> <li><a href="/wiki/Oncocytoma" title="Oncocytoma">Oncocytoma</a></li> <li><a href="/wiki/Clear-cell_adenocarcinoma" title="Clear-cell adenocarcinoma">Clear-cell adenocarcinoma</a></li> <li><a href="/wiki/Apudoma" title="Apudoma">Apudoma</a></li> <li><a href="/wiki/Adenoid_cystic_carcinoma" title="Adenoid cystic carcinoma">Cylindroma</a></li> <li><a href="/wiki/Papillary_hidradenoma" title="Papillary hidradenoma">Papillary hidradenoma</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Adnexal and<br />skin appendage</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i>sweat gland</i> <ul><li><a href="/wiki/Hidrocystoma" title="Hidrocystoma">Hidrocystoma</a></li> <li><a href="/wiki/Syringoma" title="Syringoma">Syringoma</a></li></ul></li> <li><a href="/wiki/Syringocystadenoma_papilliferum" title="Syringocystadenoma papilliferum">Syringocystadenoma papilliferum</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Cystic,<br />mucinous,<br />and serous</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Cystic" class="mw-redirect" title="Cystic">Cystic</a> general</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Cystadenoma" title="Cystadenoma">Cystadenoma</a>/<a href="/wiki/Cystadenocarcinoma" title="Cystadenocarcinoma">Cystadenocarcinoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mucous_gland" title="Mucous gland">Mucinous</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Signet_ring_cell_carcinoma" title="Signet ring cell carcinoma">Signet ring cell carcinoma</a> <ul><li><a href="/wiki/Krukenberg_tumor" title="Krukenberg tumor">Krukenberg tumor</a></li></ul></li> <li><span class="wrap"><a href="/wiki/Mucinous_cystadenoma" title="Mucinous cystadenoma">Mucinous cystadenoma</a> / <a href="/wiki/Mucinous_cystadenocarcinoma" title="Mucinous cystadenocarcinoma">Mucinous cystadenocarcinoma</a></span> <ul><li><a href="/wiki/Pseudomyxoma_peritonei" title="Pseudomyxoma peritonei">Pseudomyxoma peritonei</a></li></ul></li> <li><a href="/wiki/Mucoepidermoid_carcinoma" title="Mucoepidermoid carcinoma">Mucoepidermoid carcinoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Serous_gland" title="Serous gland">Serous</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><span class="wrap"><a href="/wiki/Ovarian_serous_cystadenoma" title="Ovarian serous cystadenoma">Ovarian serous cystadenoma</a> / <a href="/wiki/Pancreatic_serous_cystadenoma" title="Pancreatic serous cystadenoma">Pancreatic serous cystadenoma</a> / <a href="/wiki/Serous_cystadenocarcinoma" title="Serous cystadenocarcinoma">Serous cystadenocarcinoma</a> / <a href="/wiki/Papillary_serous_cystadenocarcinoma" title="Papillary serous cystadenocarcinoma">Papillary serous cystadenocarcinoma</a> </span></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Ductal,<br />lobular,<br />and medullary</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Ductal_carcinoma" title="Ductal carcinoma">Ductal carcinoma</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Mammary_ductal_carcinoma" class="mw-redirect" title="Mammary ductal carcinoma">Mammary ductal carcinoma</a></li> <li><a href="/wiki/Pancreatic_ductal_carcinoma" class="mw-redirect" title="Pancreatic ductal carcinoma">Pancreatic ductal carcinoma</a></li> <li><a href="/wiki/Comedocarcinoma" title="Comedocarcinoma">Comedocarcinoma</a></li> <li><span class="wrap"><a href="/wiki/Paget%27s_disease_of_the_breast" title="Paget's disease of the breast">Paget's disease of the breast</a> / <a href="/wiki/Extramammary_Paget%27s_disease" title="Extramammary Paget's disease">Extramammary Paget's disease</a></span></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Lobular_carcinoma" title="Lobular carcinoma">Lobular carcinoma</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Lobular_carcinoma_in_situ" title="Lobular carcinoma in situ">Lobular carcinoma in situ</a></li> <li><a href="/wiki/Invasive_lobular_carcinoma" title="Invasive lobular carcinoma">Invasive lobular carcinoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Medullary_carcinoma" title="Medullary carcinoma">Medullary carcinoma</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Medullary_breast_carcinoma" title="Medullary breast carcinoma">Medullary breast carcinoma</a></li> <li><a href="/wiki/Medullary_thyroid_cancer" title="Medullary thyroid cancer">Medullary thyroid cancer</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Acinus" title="Acinus">Acinar</a> cell</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Acinic_cell_carcinoma" title="Acinic cell carcinoma">Acinic cell carcinoma</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Connective/soft_tissue_tumors_and_sarcomas" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1239400231"><div class="navbar plainlinks hlist navbar-mini"><ul><li class="nv-view"><a href="/wiki/Template:Soft_tissue_tumors_and_sarcomas" title="Template:Soft tissue tumors and sarcomas"><abbr title="View this template">v</abbr></a></li><li class="nv-talk"><a href="/wiki/Template_talk:Soft_tissue_tumors_and_sarcomas" title="Template talk:Soft tissue tumors and sarcomas"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Soft_tissue_tumors_and_sarcomas" title="Special:EditPage/Template:Soft tissue tumors and sarcomas"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Connective/soft_tissue_tumors_and_sarcomas" style="font-size:114%;margin:0 4em"><a href="/wiki/Connective_tissue" title="Connective tissue">Connective</a>/<a href="/wiki/Soft_tissue" title="Soft tissue">soft tissue</a> <a href="/wiki/Neoplasm" title="Neoplasm">tumors</a> and <a href="/wiki/Sarcoma" title="Sarcoma">sarcomas</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%">Not otherwise specified</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Soft-tissue_sarcoma" title="Soft-tissue sarcoma">Soft-tissue sarcoma</a></li> <li><a href="/wiki/Desmoplastic_small-round-cell_tumor" title="Desmoplastic small-round-cell tumor">Desmoplastic small-round-cell tumor</a></li> <li><a href="/wiki/Skin_sarcoma" title="Skin sarcoma">Skin sarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Connective_tissue_neoplasm" title="Connective tissue neoplasm">Connective tissue neoplasm</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibrous_tissue_neoplasm" title="Fibrous tissue neoplasm">Fibromatous</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibroma" title="Fibroma">Fibroma</a>/<a href="/wiki/Fibrosarcoma" title="Fibrosarcoma">fibrosarcoma</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Dermatofibrosarcoma_protuberans" title="Dermatofibrosarcoma protuberans">Dermatofibrosarcoma protuberans</a></li> <li><a href="/wiki/Desmoplastic_fibroma" title="Desmoplastic fibroma">Desmoplastic fibroma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibroma" title="Fibroma">Fibroma</a>/<a href="/wiki/Fibromatosis" title="Fibromatosis">fibromatosis</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Aggressive_fibromatosis" title="Aggressive fibromatosis">Aggressive fibromatosis</a></li> <li><a href="/wiki/Aggressive_infantile_fibromatosis" class="mw-redirect" title="Aggressive infantile fibromatosis">Aggressive infantile fibromatosis</a></li> <li><a href="/wiki/Aponeurotic_fibroma" title="Aponeurotic fibroma">Aponeurotic fibroma</a></li> <li><a href="/wiki/Collagenous_fibroma" title="Collagenous fibroma">Collagenous fibroma</a></li> <li><a href="/wiki/Diffuse_infantile_fibromatosis" title="Diffuse infantile fibromatosis">Diffuse infantile fibromatosis</a></li> <li>Familial myxovascular fibromas</li> <li><a href="/wiki/Fibroma_of_tendon_sheath" title="Fibroma of tendon sheath">Fibroma of tendon sheath</a></li> <li><a href="/wiki/Fibromatosis_colli" title="Fibromatosis colli">Fibromatosis colli</a></li> <li><a href="/wiki/Infantile_digital_fibromatosis" title="Infantile digital fibromatosis">Infantile digital fibromatosis</a></li> <li><a href="/wiki/Juvenile_hyaline_fibromatosis" title="Juvenile hyaline fibromatosis">Juvenile hyaline fibromatosis</a></li> <li><a href="/wiki/Plantar_fibromatosis" title="Plantar fibromatosis">Plantar fibromatosis</a></li> <li><a href="/wiki/Pleomorphic_fibroma" title="Pleomorphic fibroma">Pleomorphic fibroma</a></li> <li><a href="/wiki/Oral_submucous_fibrosis" title="Oral submucous fibrosis">Oral submucous fibrosis</a></li> <li><a href="/wiki/Pachydermodactyly" title="Pachydermodactyly">Pachydermodactyly</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Histiocytoma" title="Histiocytoma">Histiocytoma</a>/<a href="/wiki/Histiocytic_sarcoma" title="Histiocytic sarcoma">histiocytic sarcoma</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Benign_fibrous_histiocytoma" class="mw-redirect" title="Benign fibrous histiocytoma">Benign fibrous histiocytoma</a></li> <li><a href="/wiki/Pleomorphic_undifferentiated_sarcoma" class="mw-redirect" title="Pleomorphic undifferentiated sarcoma">Malignant fibrous histiocytoma</a></li> <li><a href="/wiki/Atypical_fibroxanthoma" title="Atypical fibroxanthoma">Atypical fibroxanthoma</a></li> <li><a href="/wiki/Solitary_fibrous_tumor" title="Solitary fibrous tumor">Solitary fibrous tumor</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Myxoma" title="Myxoma">Myxomatous</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Myxoma" title="Myxoma">Myxoma</a>/<a href="/wiki/Myxosarcoma" title="Myxosarcoma">myxosarcoma</a> <ul><li><a href="/wiki/Cutaneous_myxoma" title="Cutaneous myxoma">Cutaneous myxoma</a></li> <li><a href="/wiki/Superficial_acral_fibromyxoma" title="Superficial acral fibromyxoma">Superficial acral fibromyxoma</a></li></ul></li> <li><a href="/wiki/Aggressive_angiomyxoma" title="Aggressive angiomyxoma">Angiomyxoma</a></li> <li><a href="/wiki/Ossifying_fibromyxoid_tumour" title="Ossifying fibromyxoid tumour">Ossifying fibromyxoid tumour</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Fibroepithelial_neoplasms" class="mw-redirect" title="Fibroepithelial neoplasms">Fibroepithelial</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Brenner_tumour" title="Brenner tumour">Brenner tumour</a></li> <li><a href="/wiki/Fibroadenoma" title="Fibroadenoma">Fibroadenoma</a></li> <li><a href="/wiki/Phyllodes_tumor" title="Phyllodes tumor">Phyllodes tumor</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Synovial_joint" title="Synovial joint">Synovial</a>-like</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Synovial_sarcoma" title="Synovial sarcoma">Synovial sarcoma</a></li> <li><a href="/wiki/Clear-cell_sarcoma" class="mw-redirect" title="Clear-cell sarcoma">Clear-cell sarcoma</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Adipose_tissue_neoplasm" title="Adipose tissue neoplasm">Lipomatous</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Lipoma" title="Lipoma">Lipoma</a>/<a href="/wiki/Liposarcoma" title="Liposarcoma">liposarcoma</a> <ul><li><a href="/wiki/Myelolipoma" title="Myelolipoma">Myelolipoma</a></li> <li><a href="/wiki/Myxoid_liposarcoma" title="Myxoid liposarcoma">Myxoid liposarcoma</a></li></ul></li> <li><a href="/wiki/Perivascular_epithelioid_cell_tumour" title="Perivascular epithelioid cell tumour">PEComa</a> <ul><li><a href="/wiki/Angiomyolipoma" title="Angiomyolipoma">Angiomyolipoma</a></li></ul></li></ul> <ul><li><a href="/wiki/Chondroid_lipoma" title="Chondroid lipoma">Chondroid lipoma</a></li> <li><a href="/wiki/Intradermal_spindle_cell_lipoma" class="mw-redirect" title="Intradermal spindle cell lipoma">Intradermal spindle cell lipoma</a></li> <li><a href="/wiki/Pleomorphic_lipoma" title="Pleomorphic lipoma">Pleomorphic lipoma</a></li> <li><a href="/wiki/Lipoblastomatosis" title="Lipoblastomatosis">Lipoblastomatosis</a></li> <li><a href="/wiki/Spindle_cell_lipoma" title="Spindle cell lipoma">Spindle cell lipoma</a></li> <li><a href="/wiki/Hibernoma" title="Hibernoma">Hibernoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Muscle_tissue_neoplasm" class="mw-redirect" title="Muscle tissue neoplasm">Myomatous</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold">General</span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Myoma" title="Myoma">Myoma</a>/<a href="/wiki/Myosarcoma" title="Myosarcoma">myosarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Smooth_muscle_tissue" class="mw-redirect" title="Smooth muscle tissue">Smooth muscle</a></span></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Leiomyoma" title="Leiomyoma">Leiomyoma</a>/<a href="/wiki/Leiomyosarcoma" title="Leiomyosarcoma">leiomyosarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><span class="nobold"><a href="/wiki/Skeletal_muscle" title="Skeletal muscle">Skeletal muscle</a></span></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Rhabdomyoma" title="Rhabdomyoma">Rhabdomyoma</a>/<a href="/wiki/Rhabdomyosarcoma" title="Rhabdomyosarcoma">rhabdomyosarcoma</a>: <a href="/wiki/Embryonal_rhabdomyosarcoma" title="Embryonal rhabdomyosarcoma">Embryonal rhabdomyosarcoma</a> <ul><li><a href="/wiki/Sarcoma_botryoides" title="Sarcoma botryoides">Sarcoma botryoides</a></li></ul></li> <li><a href="/wiki/Alveolar_rhabdomyosarcoma" title="Alveolar rhabdomyosarcoma">Alveolar rhabdomyosarcoma</a></li></ul> </div></td></tr><tr><td colspan="2" class="navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Leiomyoma" title="Leiomyoma">Leiomyoma</a> <ul><li><a href="/wiki/Cutaneous_leiomyoma" title="Cutaneous leiomyoma">Cutaneous</a></li></ul></li> <li><a href="/wiki/Angioleiomyoma" title="Angioleiomyoma">Angioleiomyoma</a></li> <li><a href="/wiki/Angiolipoleiomyoma" title="Angiolipoleiomyoma">Angiolipoleiomyoma</a></li> <li><a href="/wiki/Genital_leiomyoma" title="Genital leiomyoma">Genital leiomyoma</a></li> <li><a href="/wiki/Leiomyosarcoma" title="Leiomyosarcoma">Leiomyosarcoma</a></li> <li><a class="mw-selflink selflink">Multiple cutaneous and uterine leiomyomatosis syndrome</a></li> <li><a href="/wiki/Multiple_cutaneous_leiomyoma" class="mw-redirect" title="Multiple cutaneous leiomyoma">Multiple cutaneous leiomyoma</a></li> <li><a href="/wiki/Neural_fibrolipoma" title="Neural fibrolipoma">Neural fibrolipoma</a></li> <li><a href="/wiki/Solitary_cutaneous_leiomyoma" class="mw-redirect" title="Solitary cutaneous leiomyoma">Solitary cutaneous leiomyoma</a></li> <li><a href="/wiki/Smooth_muscle_tumor_of_uncertain_malignant_potential" title="Smooth muscle tumor of uncertain malignant potential">STUMP</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Complex_and_mixed_tumor" class="mw-redirect" title="Complex and mixed tumor">Complex mixed and stromal</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Adenomyoma" title="Adenomyoma">Adenomyoma</a></li> <li><a href="/wiki/Pleomorphic_adenoma" title="Pleomorphic adenoma">Pleomorphic adenoma</a></li> <li><a href="/wiki/Mixed_M%C3%BCllerian_tumor" title="Mixed Müllerian tumor">Mixed Müllerian tumor</a></li> <li><a href="/wiki/Mesoblastic_nephroma" title="Mesoblastic nephroma">Mesoblastic nephroma</a></li> <li><a href="/wiki/Wilms%27_tumor" title="Wilms' tumor">Wilms' tumor</a></li> <li><a href="/wiki/Malignant_rhabdoid_tumour" title="Malignant rhabdoid tumour">Malignant rhabdoid tumour</a></li> <li><a href="/wiki/Clear-cell_sarcoma_of_the_kidney" title="Clear-cell sarcoma of the kidney">Clear-cell sarcoma of the kidney</a></li> <li><a href="/wiki/Hepatoblastoma" title="Hepatoblastoma">Hepatoblastoma</a></li> <li><a href="/wiki/Pancreatoblastoma" title="Pancreatoblastoma">Pancreatoblastoma</a></li> <li><a href="/wiki/Carcinosarcoma" title="Carcinosarcoma">Carcinosarcoma</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Mesothelium" title="Mesothelium">Mesothelial</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Mesothelioma" title="Mesothelioma">Mesothelioma</a></li> <li><a href="/wiki/Adenomatoid_tumor" title="Adenomatoid tumor">Adenomatoid tumor</a></li></ul> </div></td></tr></tbody></table></div>    </div><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?type=1x1&useformat=desktop" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Retrieved from "<a dir="ltr" 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