The American Journal of Surgical Pathology

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this.features)));n.length>0&&(0,e.R)(36,{targetFeature:r.featureName,missingDependencies:n}),this.features[r.featureName]=new r(this)}))}catch(t){(0,e.R)(22,t);for(const e in this.features)this.features[e].abortHandler?.();const r=(0,R.Zm)();delete r.initializedAgents[this.agentIdentifier]?.api,delete r.initializedAgents[this.agentIdentifier]?.features,delete this.sharedAggregator;return r.ee.get(this.agentIdentifier).abort(),!1}}}({features:[he,w,N,Ne,_e,_,D,ot,ct,je,nt],loaderType:"spa"})})()})();</script><meta charset="utf-8" /><meta name="viewport" content="width=device-width, initial-scale=1" /><meta http-equiv="Expires" content="0" /><meta name="DCSext.AbsHTMLview" content="1" /><meta name="WT.cg_n" content="ajsp" /><meta name="WT.cg_s" content="Articles" /><meta name="wkhealth_journal_title" content="The American Journal of Surgical Pathology" /><meta name="wkhealth_journal_title_legacy" content="The American Journal of Surgical Pathology" /><meta name="wkhealth_issue" content="2" /><meta name="wkhealth_article_publication_date" content="February 2014" /><meta name="wkhealth_volume" content="38" /><meta name="wkhealth_ovid_accession_number" content="00000478-201402000-00004" /><meta name="wkhealth_issn" content="0147-5185" /><meta name="wkhealth_title" content="Perivascular Epithelioid Cell Neoplasm (PEComa) of the Gynecologic Tract: Clinicopathologic and Immunohistochemical Characterization of 16 Cases" /><meta name="wkhealth_authors" content="Schoolmeester, John Kenneth;Howitt, Brooke E.;Hirsch, Michelle S.;Dal Cin, Paola;Quade, Bradley J.;Nucci, Marisa R." /><meta name="wkhealth_doi" content="10.1097/PAS.0000000000000133" /><meta name="wkhealth_abstract_html_url" content="https://journals.lww.com/ajsp/abstract/2014/02000/perivascular_epithelioid_cell_neoplasm__pecoma__of.4.aspx" /><meta name="wkhealth_fulltext_html_url" content="https://journals.lww.com/ajsp/fulltext/2014/02000/perivascular_epithelioid_cell_neoplasm__pecoma__of.4.aspx" /><meta name="wkhealth_toc_section" content="Original Articles" /><meta name="wkhealth_toc_sub_section" /><meta name="wkhealth_eJpId" content="ajsp:2014:02000:00004" /><meta name="wkhealth_firstpage" content="176" /><meta name="wkhealth_date" content="February 2014" /><meta name="wkhealth_pdf_url" content="https://journals.lww.com/ajsp/_layouts/15/oaks.journals/downloadpdf.aspx?trckng_src_pg=Other&an=00000478-201402000-00004" /><meta name="Description" content="Perivascular epithelioid cell tumor (PEComa) belongs to a family of tumors characterized by coexpres" /><meta property="og:site_name" content="LWW" /><meta property="og:type" content="article" /><meta property="og:title" content="Perivascular Epithelioid Cell Neoplasm (PEComa) of the... : The American Journal of Surgical Pathology" /><meta property="og:description" content="izing this tumor. However, its occurrence in the gynecologic tract continues to be disputed owing to its common misclassification as other types of uterine sarcoma and its controversial relationship with epithelioid smooth muscle tumors. To more fully characterize PEComa of the female genital tract, 16 cases of gynecologic PEComa were identified (1990 to 2012) and formed the basis of this study. Each case was analyzed for conventional morphologic and immunohistochemical characteristics established for PEComa of extrauterine sites; clinical outcome data were obtained for all cases. The 16 patients were aged 28 to 60 (mean 49; median 50) years, and 1 had a history of tuberous sclerosis complex. Thirteen cases were primary of the uterus, 2 of the adnexa, and 1 of the vagina. Tumor size ranged from 0.3 to 25.0 (mean 8.7) cm. Three patients died of disease, 6 were alive with disease, and 7 were alive without evidence of disease at last follow-up (1 mo to 13 y follow-up; mean 26 mo). All patients with an adverse outcome met established criteria for malignancy as proposed for extrauterine sites (ie, 2 or more features present: size ≥5 cm, high-grade nuclear features, infiltration, necrosis, lymphovascular invasion, or a mitotic rate ≥1/50 high-power fields). Of the melanocytic markers, HMB45 was most commonly expressed (16/16 positive, 100%), followed by microphthalmia transcription factor (11/12 positive, 92%), MelanA (14/16 positive, 88%), and S100 protein (2/10 positive, 20%). Of the smooth muscle markers, desmin was most commonly expressed (15/15 cases, 100%), followed by SMA (14/15 cases, 93%) and h-caldesmon (11/12 cases, 92%). TFE3 immunopositivity was identified in 5 of 13 cases; however, 3 tested cases were negative for a TFE3 rearrangement by fluorescence in situ hybridization. Current criteria for malignancy appear to be valid in the female genital tract, although modified criteria, as described herein, may be more specific. Awareness of the characteristic features of PEComa is important to help distinguish it from epithelioid smooth muscle tumors and other mimics as PEComa may respond to unique chemotherapeutic regimens...." /><meta property="og:image" content="https://images.journals.lww.com/ajsp/SocialThumb.00000478-201402000-00004.T1-4.jpeg" /><meta property="og:image:width" content="1416" /><meta property="og:image:height" content="2292" /><meta property="og:url" content="https://journals.lww.com/ajsp/fulltext/2014/02000/perivascular_epithelioid_cell_neoplasm__pecoma__of.4.aspx" /><meta property="twitter:card" content="summary" /><meta name="robots" content="index, follow, noarchive" /><meta name="WT.sv" content="ZUSE1PEJPWEB01" /><meta name="Title" content="The American Journal of Surgical Pathology" /><style id="SPThemeHideForms" type="text/css">body {opacity:0 !important}</style>  <link 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PEComa","https://images.journals.lww.com/ajsp/Rollover.00000478-201402000-00004.T1-4.jpeg","https://images.journals.lww.com/ajsp/Gallery.00000478-201402000-00004.T1-4.jpeg","https://journals.lww.com/ajsp/_layouts/oaks.journals/ImageView.aspx?k=ajsp:2014:02000:00004&i=T1-4&year=2014&issue=02000&article=00004&type=Abstract","https://images.journals.lww.com/ajsp/Original.00000478-201402000-00004.T1-4.jpeg","/ajsp/pages/imagegallery.aspx?year=2014&issue=02000&article=00004", "ajsp_2014_02000_00004_T1-4");images["T2-4"]=new image("T2-4","F1-4","T1-4","TABLE 2","Architectural Features of Gynecologic PEComa","https://images.journals.lww.com/ajsp/Rollover.00000478-201402000-00004.T2-4.jpeg","https://images.journals.lww.com/ajsp/Gallery.00000478-201402000-00004.T2-4.jpeg","https://journals.lww.com/ajsp/_layouts/oaks.journals/ImageView.aspx?k=ajsp:2014:02000:00004&i=T2-4&year=2014&issue=02000&article=00004&type=Abstract","https://images.journals.lww.com/ajsp/Original.00000478-201402000-00004.T2-4.jpeg","/ajsp/pages/imagegallery.aspx?year=2014&issue=02000&article=00004", "ajsp_2014_02000_00004_T2-4");images["F1-4"]=new image("F1-4","T3-4","T2-4","FIGURE 1","The architectural variation of gynecologic PEComa. Patterns of invasion vary, with some tumors demonstrating multiple types such as a broad, pushing border (A), destructive infiltration (B), and/or a permeative growth analogous to the “finger-like” pattern of endometrial stromal sarcoma (C). An acute branching of thin-walled vessels imparting a hemangiopericytoma-like appearance is an uncommon, but occasionally focal, finding in PEComa (D). Differing degrees of stromal hyalinization are often present in PEComa, ranging from focal-moderate (E) to extensive (F).","https://images.journals.lww.com/ajsp/Rollover.00000478-201402000-00004.F1-4.jpeg","https://images.journals.lww.com/ajsp/Gallery.00000478-201402000-00004.F1-4.jpeg","https://journals.lww.com/ajsp/_layouts/oaks.journals/ImageView.aspx?k=ajsp:2014:02000:00004&i=F1-4&year=2014&issue=02000&article=00004&type=Abstract","https://images.journals.lww.com/ajsp/Original.00000478-201402000-00004.F1-4.jpeg","/ajsp/pages/imagegallery.aspx?year=2014&issue=02000&article=00004", "ajsp_2014_02000_00004_F1-4");images["T3-4"]=new image("T3-4","F2-4","F1-4","TABLE 3","Cytologic Features of Gynecologic PEComa","https://images.journals.lww.com/ajsp/Rollover.00000478-201402000-00004.T3-4.jpeg","https://images.journals.lww.com/ajsp/Gallery.00000478-201402000-00004.T3-4.jpeg","https://journals.lww.com/ajsp/_layouts/oaks.journals/ImageView.aspx?k=ajsp:2014:02000:00004&i=T3-4&year=2014&issue=02000&article=00004&type=Abstract","https://images.journals.lww.com/ajsp/Original.00000478-201402000-00004.T3-4.jpeg","/ajsp/pages/imagegallery.aspx?year=2014&issue=02000&article=00004", "ajsp_2014_02000_00004_T3-4");images["F2-4"]=new image("F2-4","T4-4","T3-4","FIGURE 2","The cytologic spectrum of gynecologic PEComa. PEComa is typically composed of epithelioid (A) and spindled (B) cells with clear-pink granular cytoplasm and a nested growth pattern accompanied by a delicate vasculature. Other less common features include heavily pigmented cells (C) and clear cells with glycogen-rich–appearing cytoplasm (D). Some PEComas show anaplastic features along with multinucleated tumor giant cells, melanoma-like macronucleoli, and intranuclear inclusions (E). Benign multinucleated tumor cells may also be present (F).","https://images.journals.lww.com/ajsp/Rollover.00000478-201402000-00004.F2-4.jpeg","https://images.journals.lww.com/ajsp/Gallery.00000478-201402000-00004.F2-4.jpeg","https://journals.lww.com/ajsp/_layouts/oaks.journals/ImageView.aspx?k=ajsp:2014:02000:00004&i=F2-4&year=2014&issue=02000&article=00004&type=Abstract","https://images.journals.lww.com/ajsp/Original.00000478-201402000-00004.F2-4.jpeg","/ajsp/pages/imagegallery.aspx?year=2014&issue=02000&article=00004", "ajsp_2014_02000_00004_F2-4");images["T4-4"]=new image("T4-4","T5-4","F2-4","TABLE 4","IHC Profile of Gynecologic 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function alerts_ShowUnSubscribeeTOCSuccessPopup() { var unSubscribeeTocPopup = $find('unSubscribeToAlerts'); if (unSubscribeeTocPopup != null) { unSubscribeeTocPopup.show(); } } $('.js-close').on('click', function() { var unSubscribeeTocPopup = $find('unSubscribeToAlerts'); if (unSubscribeeTocPopup != null) { unSubscribeeTocPopup.hide(); } return false; }); </script> <span id="ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_dummyTargetControl"></span> <input type="hidden" name="ctl00$ctl51$Header$UserActionTools$SubscribeToEtocPopupControl$hiddenSubscribeToEtocPopupState" id="ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_hiddenSubscribeToEtocPopupState" value="showemail" /> <input type="hidden" name="ctl00$ctl51$Header$UserActionTools$SubscribeToEtocPopupControl$hiddenConfirmSubscriptionPageUrl" id="ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_hiddenConfirmSubscriptionPageUrl" /> <input type="hidden" 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href="https://journals.lww.com/ajsp/_layouts/oaks.journals/privacy.aspx" target="_blank">Privacy Policy</a> </div> </div> </div> </section> </div> <div id="ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_pnlAuthenticatedUser" style="display: none;"> <section class="wk-modal-content"> <header class="wk-modal-header ejp-modal-header"> <div class="wk-modal-title ejp-modal-title"></div> <button aria-label="close dialog" class="js-close primary-button wk-button-icon wk-modal-close ejp-modal-close "><i class="fa fa-times"></i></button> </header> <div class="wk-modal-body"> <div class="wk-field-item"> <p> <strong> <span id="ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_lblAuthenticatedUserTitle"></span> </strong> </p> </div> <p id="ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_pAuthenticatedUserMessage"> </p> </div> </section> </div> </div> </div> <div id="ej-clear-float"> </div> <script type="text/javascript"> function 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document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_hiddenSubscribeToEtocPopupState'); var emailTextBox = document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_txtEmailAddress'); var thanksPanel = document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_pnlAuthenticatedUser'); var mainPanel = document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_pnlMain'); var thankYouMessageUnAuthenticated = document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_lblThankYouMessage'); var thankYouMessageAuthenticated = document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_lblAuthenticatedUserTitle'); if (!displaySubscriptionType) { displaySubscriptionType = subscriptionType; } if (modelPopupState.value == 'showthanksmessage') { thanksPanel.style.display = "block"; mainPanel.style.display = "none"; thankYouMessageAuthenticated.innerHTML = "Thank you for subscribing to the " + displaySubscriptionType + " for " + journalTitle + "."; } if (modelPopupState.value == 'showemail') { thanksPanel.style.display = "none"; mainPanel.style.display = "block"; thankYouMessageUnAuthenticated.innerHTML = "Thank you for choosing to subscribe to the " + displaySubscriptionType + " for " + journalTitle + "."; } if ($('.etoc-popup') != null) { document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_hiddenJournalId').value = journalId; if (modelPopupState.value == 'showemail') emailTextBox.focus(); } } $('.js-close').on('click', function () { document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_txtEmailAddress').value = ""; document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_cvEmailError').style.display = "none"; var pageUrl = document.getElementById('ctl00_ctl51_Header_UserActionTools_SubscribeToEtocPopupControl_hiddenConfirmSubscriptionPageUrl'); var subscribeeTocPopup = $find('subscribeToEtoc'); if (subscribeeTocPopup != null) { subscribeeTocPopup.hide(); document.getElementsByTagName( 'html' )[0].classList.remove('no-scroll'); if (pageUrl.value !== '' && pageUrl.value !== window.location.pathname) window.location = pageUrl.value; } return false; }); </script> <div id="ctl00_ctl51_Header_UserActionTools_eTocTopContainer"> <div id="eTocTop" style="display: none;"> <a rel="nofollow" title href="javascript:javascript: alerts_ShowSubscribeeTOCPopup('The American Journal of Surgical Pathology','ajsp','eTOC', 'eTOC');;" onclick="javascript: alerts_ShowSubscribeeTOCPopup('The American Journal of Surgical Pathology','ajsp','eTOC', 'eTOC');"><span id="ctl00_ctl51_Header_UserActionTools_alertLinkControlTop_defaultText" class="hideOnFixed">Subscribe to eTOC</span></a> </div> </div> <script> $(function () { //find 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Epithelioid Cell Neoplasm (PEComa) of the Gynecologic Tract: Clinicopathologic and Immunohistochemical Characterization of 16 Cases', '02012014', 'John Schoolmeester, Brooke Howitt, Michelle Hirsch, et al', '38', '2', '\u00A9 2014 by Lippincott Williams & Wilkins.', '00000478-201402000-00004', '', false, ''); } </script> <div id="ArticleContainer"> <article id="ej-abstract-view" xmlns:ssr="http://ssr-eus-go-csi.cloudapp.net/v1/assets" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xhtml="http://www.w3.org/1999/xhtml"><section class="ejp-r-article-subsection"><div class="ejp-r-article-subsection__text">Original Articles</div></section><header class="ejp-article-header"><h1 class="ejp-article-title">Perivascular Epithelioid Cell Neoplasm (PEComa) of the Gynecologic Tract</h1> <h2 class="article-subtitle">Clinicopathologic and Immunohistochemical Characterization of 16 Cases</h2></header><section id="ejp-article-authors"><p id="P7">Schoolmeester, John Kenneth MD<sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">*</sup>; Howitt, Brooke E. MD<sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">†</sup>; Hirsch, Michelle S. MD, PhD<sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">†</sup>; Dal Cin, Paola PhD<sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">†</sup>; Quade, Bradley J. MD, PhD<sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">†</sup>; Nucci, Marisa R. MD<sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">†</sup></p><section><a href="#" id="ejp-article-authors-link"> Author Information <i class="wk-icon-arrow-down-r"></i></a></section></section><section id="ejp-article-authors-info" class="ejp-article-authors-info ejp-article-authors-info--hidden"><div class="ejp-article-authors-info-holder"> <p id="AF_ID_d2071e105"><sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">*</sup>Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN</p> <p id="AF_ID_d2071e105"><sup xmlns:mrws="http://webservices.ovid.com/mrws/1.0">†</sup>Division of Women’s and Perinatal Pathology, Brigham and Women’s Hospital, Boston, MA</p> <p id="BT_ID_d2071e120">Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.</p> <p id="BT_ID_d2071e120">Correspondence: Marisa R. Nucci, MD, Department of Pathology, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA 02115 (e-mail: <span><a href="/cdn-cgi/l/email-protection#0f62617a6c6c664f7f6e7d7b616a7d7c21607d68" onclick="javascript:window.open('mailto:mnucci@partners.org');return false"><span class="__cf_email__" data-cfemail="ea87849f898983aa9a8b989e848f9899c485988d">[email protected]</span></a></span>).</p> </div></section><section id="article-journal-info"><div class="ej-journal-info"><span class="ej-journal-name">The American Journal of Surgical Pathology </span><span id="ej-journal-date-volume-issue-pg"><a href="/ajsp/toc/2014/02000">38(2):p 176-188, February 2014.</a></span><span> | </span><span class="ej-journal-doi">DOI: </span>10.1097/PAS.0000000000000133</div></section><section id="ejp-article-indicators" class="ejp-article-indicators"><ul class="ejp-article-indicators__list"> <li id="ej-article-indicators-buy" class="ejp-article-indicators__list-item" onmouseover="javascript:Tooltip_Buy()" onmouseout="javascript:Tooltip_MouseOut()"><a href="#ContentAccessOptions"><span class="ejp-indicator">Buy</span></a></li> </ul><button id="openArticleMetricsPopupButton" type="button" class="wk-button wk-button-ghost wk-button-icon-left ejp-article-metrics-btn" data-articleMetricsEndpoint="//services.journals.lww.com/IntegrationServices/ArticleMetricsService.svc" data-articleMetricsDetailsUrl="https://wolterskluwer.altmetric.com/details/" data-doi="10.1097/PAS.0000000000000133" data-suiteId="vrs_woltere_journalsglobalvrs" data-articlePublicationDate="2014-02-01" data-articlePublicationDateString="February 2014" data-receivedDate="" data-revisionReceivedDate="" data-acceptedDate="" data-crossrefRefLinkRoot="https://doi.org/" data-medlineRefLinkRoot="http://www.ncbi.nlm.nih.gov/pubmed/" data-pubMedId="24418852"><i class="wk-icon-filled-chart-r"></i><i class="wk-icon-filled-chart-white-r"></i> Metrics </button></section><section class="ejp-article-tabs" id="abstractWrap"><h2 class="ejp-article-tabs-heading">Abstract</h2> <div class="ejp-article-tabs__tabpanel" id="panel1" role="tabpanel" aria-labelledby="tab1"> <div id="article-abstract-content1"> <div class="ejp-article-text-abstract"> <p>Perivascular epithelioid cell tumor (PEComa) belongs to a family of tumors characterized by coexpression of melanocytic and muscle markers. Recent studies have shown that sporadic and tuberous sclerosis complex–associated PEComa may respond to mTOR inhibitors underscoring the importance of recognizing this tumor. However, its occurrence in the gynecologic tract continues to be disputed owing to its common misclassification as other types of uterine sarcoma and its controversial relationship with epithelioid smooth muscle tumors. To more fully characterize PEComa of the female genital tract, 16 cases of gynecologic PEComa were identified (1990 to 2012) and formed the basis of this study. Each case was analyzed for conventional morphologic and immunohistochemical characteristics established for PEComa of extrauterine sites; clinical outcome data were obtained for all cases. The 16 patients were aged 28 to 60 (mean 49; median 50) years, and 1 had a history of tuberous sclerosis complex. Thirteen cases were primary of the uterus, 2 of the adnexa, and 1 of the vagina. Tumor size ranged from 0.3 to 25.0 (mean 8.7) cm. Three patients died of disease, 6 were alive with disease, and 7 were alive without evidence of disease at last follow-up (1 mo to 13 y follow-up; mean 26 mo). All patients with an adverse outcome met established criteria for malignancy as proposed for extrauterine sites (ie, 2 or more features present: size ≥5 cm, high-grade nuclear features, infiltration, necrosis, lymphovascular invasion, or a mitotic rate ≥1/50 high-power fields). Of the melanocytic markers, HMB45 was most commonly expressed (16/16 positive, 100%), followed by microphthalmia transcription factor (11/12 positive, 92%), MelanA (14/16 positive, 88%), and S100 protein (2/10 positive, 20%). Of the smooth muscle markers, desmin was most commonly expressed (15/15 cases, 100%), followed by SMA (14/15 cases, 93%) and h-caldesmon (11/12 cases, 92%). TFE3 immunopositivity was identified in 5 of 13 cases; however, 3 tested cases were negative for a TFE3 rearrangement by fluorescence in situ hybridization. Current criteria for malignancy appear to be valid in the female genital tract, although modified criteria, as described herein, may be more specific. Awareness of the characteristic features of PEComa is important to help distinguish it from epithelioid smooth muscle tumors and other mimics as PEComa may respond to unique chemotherapeutic regimens.</p> </div> </div> </div></section><section class="ej-article-copyright">© 2014 by Lippincott Williams & Wilkins.</section></article> </div> </div> <section id="ContentAccessOptions" class="article-not-authorized-msg"> <div class="ejp-access-options-wrapper"> <div class="ejp-access-options ejp-access-options--desktop"> <div id="ctl00_ctl29_g_60b9c391_4bd7_4f45_ae6b_9b5338b7d65c_ctl00_FullTextAccessOptionsContainer" class="ejp-access-options__subscriber-access"> <h3 class="ejp-access-options__subscriber-access-title">Full Text Access for Subscribers:</h3> <div class="ejp-access-options__subscriber-access-society-list"> <div id="ctl00_ctl29_g_60b9c391_4bd7_4f45_ae6b_9b5338b7d65c_ctl00_PrimaryLoginBox" class="box-ssologin primary-login-box"> <a 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