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</div>
<div class="action-panel-control-wrap">
<label for="email-citation-format" class="action-panel-label">
Format:
</label>
<select id="email-citation-format" name="citation-format" class="action-panel-selector email-citation-format">
<option selected="selected" value="summary">Summary</option>
<option value="summary-text">Summary (text)</option>
<option value="abstract">Abstract</option>
<option value="abstract-text">Abstract (text)</option>
</select>
</div>
<div class="include-supplemental-container">
<input type="checkbox" aria-label="Include MeSH and other data" name="include-supplemental" id="email-include-supplemental" class="email-include-supplemental">
<label for="email-include-supplemental" class="email-include-supplemental-label">MeSH and other data</label>
</div>
<div class="form-field recaptcha ">
<div class="g-recaptcha" id="id-recaptcha" data-sitekey="6LfsWHMdAAAAAClKbtOpjQ2pMjgsGxvv7NdZW9uI"></div>
</div>
<div id="captcha-error-message" class="usa-input-error-message captcha-validation-message" role="alert"></div>
<div class="action-panel-actions">
<button class="action-panel-submit"
type="submit"
data-loading-label="Sending..."
data-ga-category="save_share"
data-ga-action="email"
data-ga-label="send">
Send email
</button>
<button class="action-panel-cancel"
aria-label="Close 'Email citations' panel"
ref="linksrc=close_email_panel"
aria-controls="email-action-panel"
aria-expanded="false"
data-ga-category="save_share"
data-ga-action="email"
data-ga-label="cancel">
Cancel
</button>
</div>
<input type="hidden" name="email-search-details" value="" />
<input type="hidden" name="email-search-details-hash" value="0e42663a6c3bd85498fcb88798998fed7bfdc45d457db35281e41afe13cc0524" />
</form>
</div>
</div>
<div id="collections-action-panel"
class="collections-action-panel action-panel in-progress-dots-panel"
aria-hidden="true"
data-collections-open-panel-enabled="false"
data-collections-open-panel-url-hash="#open-collections-panel">
<div class="inner-wrap">
<h3 class="action-panel-heading">
Add to Collections
</h3>
<form id="collections-action-panel-form"
class="collections-action-panel-form action-panel-content action-form action-panel-smaller-selectors"
data-existing-collections-url="/list-existing-collections/"
data-add-to-existing-collection-url="/add-to-existing-collection/"
data-create-and-add-to-new-collection-url="/create-and-add-to-new-collection/"
data-get-article-ids-by-search-url="/get-article-ids-by-search/"
data-myncbi-max-collection-name-length="100"
data-add-to-collection-max-amount="1000"
data-collections-root-url="https://www.ncbi.nlm.nih.gov/myncbi/collections/">
<input type="hidden" name="csrfmiddlewaretoken" value="VHibIu3ExhDC53S5vgUJxC5bmn5tv6decu2glkw07Sq6ZuYV8zxL1KBPUrNFoHLy">
<div class="choice-group" role="radiogroup">
<ul class="radio-group-items">
<li>
<input type="radio"
id="collections-action-panel-new"
class="collections-new"
name="collections"
value="new"
data-ga-category="save_share"
data-ga-action="collections"
data-ga-label="collections_radio_new">
<label for="collections-action-panel-new">Create a new collection</label>
</li>
<li>
<input type="radio"
id="collections-action-panel-existing"
class="collections-existing"
name="collections"
value="existing"
checked="true"
data-ga-category="save_share"
data-ga-action="collections"
data-ga-label="collections_radio_existing">
<label for="collections-action-panel-existing">Add to an existing collection</label>
</li>
</ul>
</div>
<div class="controls-wrapper">
<div class="action-panel-control-wrap new-collections-controls">
<label for="collections-action-panel-add-to-new" class="action-panel-label required-field-asterisk">
Name your collection:
</label>
<input
type="text"
name="add-to-new-collection"
id="collections-action-panel-add-to-new"
class="collections-action-add-to-new"
pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/"
maxlength="100"
data-ga-category="save_share"
data-ga-action="create_collection"
data-ga-label="non_favorties_collection">
<div class="collections-new-name-too-long usa-input-error-message selection-validation-message">
Name must be less than 100 characters
</div>
</div>
<div class="action-panel-control-wrap existing-collections-controls">
<label for="collections-action-panel-add-to-existing" class="action-panel-label">
Choose a collection:
</label>
<select id="collections-action-panel-add-to-existing"
class="action-panel-selector collections-action-add-to-existing"
data-ga-category="save_share"
data-ga-action="select_collection"
data-ga-label="($('#collections-action-add-to-existing').val() === 'Favorites') ? 'Favorites' : 'non_favorites_collection'">
</select>
<div class="collections-retry-load-on-error usa-input-error-message selection-validation-message">
Unable to load your collection due to an error<br>
<a href="#">Please try again</a>
</div>
</div>
</div>
<div class="action-panel-actions">
<button class="action-panel-submit"
type="submit"
data-loading-label="Adding..."
data-pinger-ignore
data-ga-category="save_share"
data-ga-action="collections"
data-ga-label="add">
Add
</button>
<button class="action-panel-cancel"
aria-label="Close 'Add to Collections' panel"
ref="linksrc=close_collections_panel"
aria-controls="collections-action-panel"
aria-expanded="false"
data-ga-category="save_share"
data-ga-action="collections"
data-ga-label="cancel">
Cancel
</button>
</div>
</form>
</div>
</div>
<div id="bibliography-action-panel"
class="bibliography-action-panel action-panel in-progress-dots-panel"
aria-hidden="true"
data-bibliography-open-panel-enabled="false"
data-bibliography-open-panel-url-hash="#open-bibliography-panel">
<div class="inner-wrap">
<h3 class="action-panel-heading">
Add to My Bibliography
</h3>
<form id="bibliography-action-panel-form"
class="bibliography-action-panel-form action-panel-content action-form action-panel-smaller-selectors"
data-add-to-bibliography-max-amount="100"
data-add-to-bibliography-batch-size="10"
data-bibliography-delegates-url="/list-bibliography-delegates/"
data-add-to-bibliography-url="/add-to-bibliography/"
data-get-article-ids-by-search-url="/get-article-ids-by-search/"
data-mybib-root-url="https://www.ncbi.nlm.nih.gov/myncbi/collections/mybibliography/">
<input type="hidden" name="csrfmiddlewaretoken" value="VHibIu3ExhDC53S5vgUJxC5bmn5tv6decu2glkw07Sq6ZuYV8zxL1KBPUrNFoHLy">
<div class="action-panel-control-wrap bibliographies-controls">
<div class="choice-group">
<ul class="bibliographies-action-add radio-group-items">
<li>
<input name="bibliography" id="my-bibliography" class="my-bibliography" type="radio" checked/>
<label for="my-bibliography">My Bibliography</label>
</li>
</ul>
</div>
</div>
<div class="bibliographies-retry-load-on-error usa-input-error-message selection-validation-message">
Unable to load your delegates due to an error<br>
<a href="#">Please try again</a>
</div>
<div class="action-panel-actions">
<button class="action-panel-submit"
type="submit"
data-loading-label="Adding..."
data-pinger-ignore>
Add
</button>
<button class="action-panel-cancel"
aria-label="Close 'Add to bibliography' panel"
ref="linksrc=close_bibliography_panel"
aria-controls="bibliography-action-panel"
aria-expanded="false"
data-ga-category="save_share"
data-ga-action="mybib"
data-ga-label="cancel">
Cancel
</button>
</div>
</form>
</div>
</div>
<div id="saved-search-action-panel" class="saved-search-action-panel action-panel " aria-hidden="true"
data-saved-search-open-panel-enabled="false"
data-saved-search-open-panel-url-hash="#open-saved-search-panel">
<div class="inner-wrap">
<h2 class="action-panel-heading">
Your saved search
</h2>
<form id="saved-search-action-panel-form"
class="saved-search-action-panel-form action-panel-content action-form"
data-create-saved-search-url="/create-saved-search/"
data-try-search-terms-url="/try-search-term/"
data-saved-search-root-url="https://www.ncbi.nlm.nih.gov/myncbi/searches/">
<input type="hidden" name="csrfmiddlewaretoken" value="VHibIu3ExhDC53S5vgUJxC5bmn5tv6decu2glkw07Sq6ZuYV8zxL1KBPUrNFoHLy">
<div class="action-panel-control-wrap">
<label for="saved-search-name" class="action-panel-label saved-search-name-label required-field-asterisk">
Name of saved search:
</label>
<input maxlength="200"
type="text"
name="saved-search-name"
id="saved-search-name"
class="saved-search-name"
value=""
required
pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/">
</div>
<div class="action-panel-control-wrap">
<label for="saved-search-term" class="action-panel-label required-field-asterisk">
Search terms:
</label>
<textarea name="saved-search-term" id="saved-search-term" class="saved-search-term" required></textarea>
</div>
<div class="test-search-term-wrap">
<a href="#" class="try-search-term">Test search terms</a>
</div>
<div class="choice-group action-panel-extra-margin-top">
<span class="action-panel-label" id="fieldset-label">
Would you like email updates of new search results?
</span>
<fieldset id="saved-search-alert" aria-describedby="fieldset-label">
<legend class="usa-sr-only">Saved Search Alert Radio Buttons</legend>
<ul class="radio-group-items">
<li>
<input type="radio" id="saved-search-alert-yes" class="saved-search-alert-yes" name="saved-search-alert" value="yes" checked>
<label for="saved-search-alert-yes" class="action-panel-label">Yes</label>
</li>
<li>
<input aria-label="No radio input" type="radio" id="saved-search-alert-no" class="saved-search-alert-no" name="saved-search-alert" value="no">
<label for="saved-search-alert-no" class="action-panel-label">No</label>
</li>
</ul>
</fieldset>
</div>
<div class="alert-schedule-wrap">
<div class="action-panel-control-wrap">
<label class="action-panel-label">
Email:
</label>
<span aria-label="Email address" id="saved-search-email" class="action-panel-label"><span class="action-panel-label-bold"></span> (<a class="myncbi-account-settings" href="https://www.ncbi.nlm.nih.gov/account/settings/">change</a>)</span>
</div>
<div class="action-panel-control-wrap action-panel-extra-margin-top">
<label for="saved-search-frequency" class="action-panel-label">
Frequency:
</label>
<select id="saved-search-frequency" class="no-border-panel-selector saved-search-frequency">
<option value="monthly">Monthly</option>
<option value="weekly">Weekly</option>
<option value="daily">Daily</option>
</select>
</div>
<div class="action-panel-control-wrap saved-search-monthly-additional">
<label for="saved-search-monthly-on-day" class="action-panel-label">
Which day?
</label>
<select id="saved-search-monthly-on-day" class="no-border-panel-selector">
<option value="Sunday">The first Sunday</option>
<option value="Monday">The first Monday</option>
<option value="Tuesday">The first Tuesday</option>
<option value="Wednesday">The first Wednesday</option>
<option value="Thursday">The first Thursday</option>
<option value="Friday">The first Friday</option>
<option value="Saturday">The first Saturday</option>
<option value="day">The first day</option>
<option value="weekday">The first weekday</option>
</select>
</div>
<div class="action-panel-control-wrap saved-search-weekly-additional">
<label for="saved-search-weekly-on-day" class="action-panel-label">
Which day?
</label>
<select id="saved-search-weekly-on-day" class="no-border-panel-selector saved-search-weekly-on-day">
<option value="Sunday">Sunday</option>
<option value="Monday">Monday</option>
<option value="Tuesday">Tuesday</option>
<option value="Wednesday">Wednesday</option>
<option value="Thursday">Thursday</option>
<option value="Friday">Friday</option>
<option value="Saturday">Saturday</option>
</select>
</div>
<div class="action-panel-control-wrap">
<label for="saved-search-report" class="action-panel-label">
Report format:
</label>
<select id="saved-search-report" class="no-border-panel-selector saved-search-report">
<option value="DocSum">Summary</option>
<option value="DocSumText">Summary (text)</option>
<option value="Abstract">Abstract</option>
<option value="AbstractText">Abstract (text)</option>
<option value="MEDLINE">PubMed</option>
</select>
</div>
<div class="action-panel-control-wrap">
<label for="saved-search-amount" class="action-panel-label">
Send at most:
</label>
<select id="saved-search-amount" class="no-border-panel-selector saved-search-amount">
<option value="1">1 item</option>
<option value="5" selected>5 items</option>
<option value="10">10 items</option>
<option value="20">20 items</option>
<option value="50">50 items</option>
<option value="100">100 items</option>
<option value="200">200 items</option>
</select>
</div>
<div>
<input type="checkbox" id="saved-search-send-if-no-result" class="saved-search-send-if-no-result" name="saved-search-send-if-no-result">
<label for="saved-search-send-if-no-result" class="action-panel-label smaller-checkbox">
Send even when there aren't any new results
</label>
</div>
<div class="action-panel-control-wrap option-text-in-email-wrap">
<label for="saved-search-email-text" class="action-panel-label">
Optional text in email:
</label>
<textarea name="saved-search-email-text"
id="saved-search-email-text"
class="saved-search-email-text"></textarea>
</div>
</div>
<div class="action-panel-actions">
<button class="action-panel-submit"
type="submit"
data-loading-label="Saving..."
data-ga-category="save_share"
data-ga-action="alert"
data-ga-label="save">
Save
</button>
<button class="action-panel-cancel"
aria-label="Close 'Your saved search' panel"
ref="linksrc=close_saved_search_panel"
aria-controls="saved-search-action-panel"
aria-expanded="false"
data-ga-category="save_share"
data-ga-action="alert"
data-ga-label="cancel">
Cancel
</button>
</div>
</form>
</div>
</div>
<div id="citation-manager-action-panel" class="citation-manager-action-panel action-panel" aria-hidden="true">
<div class="inner-wrap">
<h2 class="action-panel-heading">
Create a file for external citation management software
</h2>
<form id="citation-manager-action-panel-form"
class="action-panel-content action-form"
action="/results-export-ids/"
data-by-search-action="/results-export-search-data/"
data-by-ids-action="/results-export-ids/"
method="post"
data-by-search-method="post"
data-by-ids-method="post">
<input type="hidden" name="csrfmiddlewaretoken" value="VHibIu3ExhDC53S5vgUJxC5bmn5tv6decu2glkw07Sq6ZuYV8zxL1KBPUrNFoHLy">
<input name="results-format" type="hidden" value="pubmed"/>
<div class="action-panel-actions">
<button class="action-panel-submit"
type="submit"
data-loading-label="Sending..."
data-ga-category="save_share"
data-ga-action="citation_manager"
data-ga-label="save">
Create file
</button>
<button class="action-panel-cancel"
aria-label="Close 'Send citations to citation manager' panel"
ref="linksrc=close_citation_manager_panel"
aria-controls="citation-manager-action-panel"
aria-expanded="false"
data-ga-category="save_share"
data-ga-action="citation_manager"
data-ga-label="cancel">
Cancel
</button>
</div>
</form>
</div>
</div>
<div id="rss-action-panel" class="rss-action-panel action-panel " aria-hidden="true">
<div class="inner-wrap">
<h2 class="action-panel-heading">
Your RSS Feed
</h2>
<form id="rss-action-panel-form"
class="rss-action-panel-form action-panel-content action-form"
data-create-rss-feed-url="/create-rss-feed-url/"
data-search-form-term-value="">
<input type="hidden" name="csrfmiddlewaretoken" value="VHibIu3ExhDC53S5vgUJxC5bmn5tv6decu2glkw07Sq6ZuYV8zxL1KBPUrNFoHLy">
<div class="action-panel-control-wrap">
<label for="rss-name" class="action-panel-label required-field-asterisk">
Name of RSS Feed:
</label>
<input maxlength="200"
placeholder="Name"
type="text"
name="rss-name"
id="rss-name"
class="rss-name"
value=''
required
pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/">
</div>
<div class="rss-limit-wrap">
<div class="action-panel-control-wrap action-panel-extra-margin-top">
<label for="rss-limit" class="action-panel-label">
Number of items displayed:
</label>
<select id="rss-limit" class="no-border-panel-selector rss-limit">
<option value="5">5</option>
<option value="10">10</option>
<option value="15" selected="selected">15</option>
<option value="20">20</option>
<option value="50">50</option>
<option value="100">100</option>
</select>
</div>
</div>
<div class="action-panel-actions">
<button class="action-panel-submit"
type="submit"
data-loading-label="Creating..."
data-ga-category="save_share"
data-ga-action="alert"
data-ga-label="save">
Create RSS
</button>
<button class="action-panel-cancel"
aria-label="Close 'Your RSS' panel"
ref="linksrc=close_rss_panel"
aria-controls="rss-action-panel"
aria-expanded="false"
data-ga-category="save_share"
data-ga-action="alert"
data-ga-label="cancel">
Cancel
</button>
</div>
<div class="action-panel-control-wrap rss-link-copy-wrap">
<label for="rss-link" class="usa-sr-only">RSS Link</label>
<input placeholder="Your RSS Feed Link" type="text" name="rss-link" id="rss-link" class="rss-link" title="RSS Link">
<button
type="button"
disabled
class="rss-link-copy-button disabled"
data-ga-category="save_share"
data-ga-action="rss"
data-ga-label="copy">
Copy
</button>
</div>
</form>
</div>
</div>
</div>
</div>
<div class="article-page" id="article-page" data-article-pmid="34599283">
<aside class="page-sidebar">
<div class="inner-wrap">
<div class="full-text-links">
<div class="full-view">
<h3 class="title">
Full text links
</h3>
<div class="full-text-links-list">
<a class="link-item
dialog-focus"
href="https://linkinghub.elsevier.com/retrieve/pii/S0893-3952(22)00346-5"
target="_blank"
rel="noopener"
ref="linksrc=fulltextorjournal_fulltext&amp;is_pmc=False&amp;PrId=3048&amp;itool=Abstract-def&amp;log$=linkouticon&amp;uid=34599283&amp;db=pubmed&amp;nlmid=8806605"
title="See full text options at Elsevier Science"
data-ga-category="full_text"
data-ga-action="Elsevier Science"
data-ga-label="34599283"
><img src="https://cdn.ncbi.nlm.nih.gov/corehtml/query/egifs/https:--linkinghub.elsevier.com-ihub-images-elsevieroa.png" alt="Elsevier Science full text link"><span class="text">
Elsevier Science
</span></a><a class="link-item
pmc
"
href="https://pmc.ncbi.nlm.nih.gov/articles/pmid/34599283/"
target="_blank"
rel="noopener"
ref="linksrc=fulltextorjournal_fulltext&amp;is_pmc=True&amp;PrId=3494&amp;itool=Abstract-def&amp;log$=linkouticon&amp;uid=34599283&amp;db=pubmed&amp;nlmid=8806605"
title="Free full text at PubMed Central"
data-ga-category="full_text"
data-ga-action="PMC"
data-ga-label="34599283"
><span class="text">
Free PMC article
</span></a>
</div>
</div>
<div class="short-view">
<a href="#" class="full-text-links-button full-text-links-dialog-trigger">
Full text links
</a>
</div>
</div>
<div class="actions-buttons sidebar"><h3 class="title">Actions</h3><div class="inner-wrap"><button class="citation-button citation-dialog-trigger"
aria-label="Open dialog with citation text in different styles"
data-ga-category="save_share"
data-ga-action="cite"
data-ga-label="open"
data-all-citations-url="/34599283/citations/"
data-citation-style="nlm"
data-pubmed-format-link="/34599283/export/"><span class="button-label">Cite</span></button><link type="text/css" href="ncbi-overlay-block/src/overlay-block.css"><div class="collections-button-container" data-article-id="34599283" data-article-db="pubmed"><button class="collections-button collections-dialog-trigger"
aria-label="Save article in MyNCBI collections."
data-ga-category="collections_button"
data-ga-action="click"
data-ga-label="collections_button"
data-collections-open-dialog-enabled="false"
data-collections-open-dialog-url="https://account.ncbi.nlm.nih.gov/?back_url=https%3A%2F%2Fpubmed.ncbi.nlm.nih.gov%2F34599283%2F%23open-collections-dialog"
data-in-collections="false"><span class="button-label">Collections</span></button><div class="overlay" role="dialog"><div id="collections-action-dialog"
class="dialog collections-dialog"
aria-hidden="true"><div class="title">Add to Collections</div><div class="collections-action-panel action-panel"><form id="collections-action-dialog-form"
class="collections-action-panel-form action-panel-content action-form action-panel-smaller-selectors"
data-existing-collections-url="/list-existing-collections/"
data-add-to-existing-collection-url="/add-to-existing-collection/"
data-create-and-add-to-new-collection-url="/create-and-add-to-new-collection/"
data-myncbi-max-collection-name-length="100"
data-collections-root-url="https://www.ncbi.nlm.nih.gov/myncbi/collections/"><input type="hidden" name="csrfmiddlewaretoken" value="VHibIu3ExhDC53S5vgUJxC5bmn5tv6decu2glkw07Sq6ZuYV8zxL1KBPUrNFoHLy"><div class="choice-group" role="radiogroup"><ul class="radio-group-items"><li><input type="radio"
id="collections-action-dialog-new"
class="collections-new"
name="collections"
value="new"
data-ga-category="collections_button"
data-ga-action="click"
data-ga-label="collections_radio_new"><label for="collections-action-dialog-new">Create a new collection</label></li><li><input type="radio"
id="collections-action-dialog-existing"
class="collections-existing"
name="collections"
value="existing"
checked="true"
data-ga-category="collections_button"
data-ga-action="click"
data-ga-label="collections_radio_existing"><label for="collections-action-dialog-existing">Add to an existing collection</label></li></ul></div><div class="controls-wrapper"><div class="action-panel-control-wrap new-collections-controls"><label for="collections-action-dialog-add-to-new" class="action-panel-label required-field-asterisk">
Name your collection:
</label><input
type="text"
name="add-to-new-collection"
id="collections-action-dialog-add-to-new"
class="collections-action-add-to-new"
pattern="[^&quot;&amp;=&lt;&gt;\/]*" title="The following characters are not allowed in the Name field: &quot;&amp;=&lt;&gt;/"
maxlength="100"
data-ga-category="collections_button"
data-ga-action="create_collection"
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data-ga-label="D Ashley Hill">D Ashley Hill</a><sup class="affiliation-links"><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="International Pleuropulmonary Blastoma/DICER1 Registry, Children&#x27;s Minnesota, Minneapolis, MN, USA." href="#short-view-affiliation-3" ref="linksrc=author_aff">
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</a><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="Division of Pathology, Children&#x27;s National Medical Center, George Washington University School of Medicine and Health Sciences, Washington, DC, USA." href="#short-view-affiliation-6" ref="linksrc=author_aff">
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</a><span class="author-sup-separator">&nbsp;</span><a class="affiliation-link" title="The Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children&#x27;s Hospitals, Washington University Medical Center, St. Louis, MO, USA. dehner@wustl.edu." href="#short-view-affiliation-8" ref="linksrc=author_aff">
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<li data-affiliation-id="short-view-affiliation-1"
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><sup class="key">1</sup> Department of Pathology and Laboratory Medicine, Children&#x27;s Hospital of Philadelphia, Philadelphia, PA, USA.</li>
<li data-affiliation-id="short-view-affiliation-2"
id="short-view-affiliation-2"
><sup class="key">2</sup> Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD, USA.</li>
<li data-affiliation-id="short-view-affiliation-3"
id="short-view-affiliation-3"
><sup class="key">3</sup> International Pleuropulmonary Blastoma/DICER1 Registry, Children&#x27;s Minnesota, Minneapolis, MN, USA.</li>
<li data-affiliation-id="short-view-affiliation-4"
id="short-view-affiliation-4"
><sup class="key">4</sup> Cancer and Blood Disorders, Children&#x27;s Minnesota, Minneapolis, MN, USA.</li>
<li data-affiliation-id="short-view-affiliation-5"
id="short-view-affiliation-5"
><sup class="key">5</sup> ResourcePath LLC, Sterling, VA, USA.</li>
<li data-affiliation-id="short-view-affiliation-6"
id="short-view-affiliation-6"
><sup class="key">6</sup> Division of Pathology, Children&#x27;s National Medical Center, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.</li>
<li data-affiliation-id="short-view-affiliation-7"
id="short-view-affiliation-7"
><sup class="key">7</sup> International Pleuropulmonary Blastoma/DICER1 Registry, Children&#x27;s Minnesota, Minneapolis, MN, USA. dehner@wustl.edu.</li>
<li data-affiliation-id="short-view-affiliation-8"
id="short-view-affiliation-8"
><sup class="key">8</sup> The Lauren V. Ackerman Laboratory of Surgical Pathology, Barnes-Jewish and St. Louis Children&#x27;s Hospitals, Washington University Medical Center, St. Louis, MO, USA. dehner@wustl.edu.</li>
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<p>
DICER1 syndrome (OMIM 606241, 601200) is a rare autosomal dominant familial tumor predisposition disorder with a heterozygous DICER1 germline mutation. The most common tumor seen clinically is the pleuropulmonary blastoma (PPB), a lung neoplasm of early childhood which is classified on its morphologic features into four types (IR, I, II and III) with tumor progression over time within the first 4-5 years of life from the prognostically favorable cystic type I to the unfavorable solid type III. Following the initial report of PPB, its association with other cystic neoplasms was demonstrated in family studies. The detection of the germline mutation in DICER1 provided the opportunity to identify and continue to recognize a number seemingly unrelated extrapulmonary neoplasms: Sertoli-Leydig cell tumor, gynandroblastoma, embryonal rhabdomyosarcomas of the cervix and other sites, multinodular goiter, differentiated and poorly differentiated thyroid carcinoma, cervical-thyroid teratoma, cystic nephroma-anaplastic sarcoma of kidney, nasal chondromesenchymal hamartoma, intestinal juvenile-like hamartomatous polyp, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma, primary central nervous system sarcoma, embryonal tumor with multilayered rosettes-like cerebellar tumor, PPB-like peritoneal sarcoma, DICER1-associated presacral malignant teratoid neoplasm and other non-neoplastic associations. Each of these neoplasms is characterized by a second somatic mutation in DICER1. In this review, we have summarized the salient clinicopathologic aspects of these tumors whose histopathologic features have several overlapping morphologic attributes particularly the primitive mesenchyme often with rhabdomyoblastic and chondroid differentiation and an uncommitted spindle cell pattern. Several of these tumors have an initial cystic stage from which there is progression to a high grade, complex patterned neoplasm. These pathologic findings in the appropriate clinical setting should serve to alert the pathologist to the possibility of a DICER1-associated neoplasm and initiate appropriate testing on the neoplasm and to alert the clinician about the concern for a DICER1 mutation.
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<p xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:p1="http://pubmed.gov/pub-one">The authors declare no competing interests.</p>
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Figures
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itemprop="contentUrl"
aria-describedby="figure-caption-0"
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data-image-width="700"
data-image-height="144"
data-image-alt="Fig. 1"
data-pmc-id="PMC8695383"
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<img class="figure-thumb" itemprop="thumbnail"
id="article-image-0"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/b8b82ef10894/41379_2021_905_Fig1_HTML.gif"
alt="Fig. 1" />
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<strong class="figure-label">
<p> Fig. 1. Pleuropulmonary blastoma, type III. </p>
</strong>
<div class="figure-caption-contents"><b> A… </b></div>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 1. Pleuropulmonary blastoma, type III. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> Pleuropulmonary blastoma, type III occurring in a 3-year-old…</div>
</div>
<figcaption id="figure-caption-0" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 1. Pleuropulmonary blastoma, type III.
</strong>
<div class="figure-caption-contents"><b>A</b> Pleuropulmonary blastoma, type III occurring in a 3-year-old male as a large solid thoracic mass. Piecemeal resection revealed a tan-white, myxoid and hemorrhagic neoplasm (one of the original 11 PPB cases). <b>B</b> Focus of embryonal type rhabdomyosarcoma and nodule of malignant cartilage. <b>C</b> Another focus of primitive sarcoma with individual anaplastic cells including bizarre mitotic figure.</div>
</figcaption>
</figure>
<figure class="figure-item "
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<img class="figure-thumb" itemprop="thumbnail"
id="article-image-1"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/0626ab179866/41379_2021_905_Fig2_HTML.gif"
alt="Fig. 2" />
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<strong class="figure-label">
<p> Fig. 2. Pleuropulmonary blastoma, type I presenting… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 2. Pleuropulmonary blastoma, type I presenting as a cystic lesion in the lung of… </p>
</strong>
</div>
<figcaption id="figure-caption-1" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 2. Pleuropulmonary blastoma, type I presenting as a cystic lesion in the lung of a 1-year-old female.
</strong>
<div class="figure-caption-contents"><b>A</b> Septal markings and cysts are appreciated in this unbisected specimen whose multicystic structure is lost with bisection as the delicate septa collapse. <b>B</b> The peripheral multicystic lesion is composed of uniform septal structures abutting the pleural surface, a characteristic feature. <b>C</b> Uniformly expanded septa by primitive-appearing small, rounded and spindled-shaped cells, many showing desmin and myogenin positivity (not shown). <b>D</b> Cambium layer-like concentration of primitive small cells with adjacent fibrous stroma.</div>
</figcaption>
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<img class="figure-thumb" itemprop="thumbnail"
id="article-image-2"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/bf87e091678d/41379_2021_905_Fig3_HTML.gif"
alt="Fig. 3" />
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<strong class="figure-label">
<p> Fig. 3. Pleuropulmonary blastoma, type I showing… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 3. Pleuropulmonary blastoma, type I showing septal widening. </p>
</strong>
<div class="figure-caption-contents"><p> Pleuropulmonary blastoma, type I showing a… </p></div>
</div>
<figcaption id="figure-caption-2" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 3. Pleuropulmonary blastoma, type I showing septal widening.
</strong>
<div class="figure-caption-contents">Pleuropulmonary blastoma, type I showing a focus of septal widening by primitive small cells as a feature of presumed progression before the formation of a grossly visible mass, as a requisite for the diagnosis of type II PPB.</div>
</figcaption>
</figure>
<figure class="figure-item "
itemscope itemtype="http://schema.org/ImageObject"
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data-slide-index="3"
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href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/dbcf6811c968/41379_2021_905_Fig4_HTML.jpg"
itemprop="contentUrl"
aria-describedby="figure-caption-3"
role="button"
data-image-width="750"
data-image-height="655"
data-image-alt="Fig. 4"
data-pmc-id="PMC8695383"
data-figure-id="Fig4">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-3"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/f616579ae0b6/41379_2021_905_Fig4_HTML.gif"
alt="Fig. 4" />
</a>
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<strong class="figure-label">
<p> Fig. 4. Pleuropulmonary blastoma, type I showing… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 4. Pleuropulmonary blastoma, type I showing a nodule of cartilage within a septum. </p>
</strong>
<div class="figure-caption-contents"><p> This… </p></div>
</div>
<figcaption id="figure-caption-3" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 4. Pleuropulmonary blastoma, type I showing a nodule of cartilage within a septum.
</strong>
<div class="figure-caption-contents">This finding is seen in 15% of type I PPBs, as one or several foci.</div>
</figcaption>
</figure>
<figure class="figure-item "
itemscope itemtype="http://schema.org/ImageObject"
itemprop="associatedMedia"
data-slide-index="4"
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itemprop="contentUrl"
aria-describedby="figure-caption-4"
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data-image-width="675"
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data-image-alt="Fig. 5"
data-pmc-id="PMC8695383"
data-figure-id="Fig5">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-4"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/2a2363c55c7c/41379_2021_905_Fig5_HTML.gif"
alt="Fig. 5" />
</a>
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<div class="caption-wrap">
<strong class="figure-label">
<p> Fig. 5. Pleuropulmonary blastoma, type II. </p>
</strong>
<div class="figure-caption-contents"><b> A… </b></div>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 5. Pleuropulmonary blastoma, type II. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> Focus of PPB type I in a mass…</div>
</div>
<figcaption id="figure-caption-4" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 5. Pleuropulmonary blastoma, type II.
</strong>
<div class="figure-caption-contents"><b>A</b> Focus of PPB type I in a mass lesion in this 2-year-old male whose tumor was predominantly solid. <b>B</b>, <b>C</b> A cambium layer-like growth pattern in this transitional area from type I to the solid pattern. <b>D</b> Solid focus showing a mixture of small primitive round and spindle cells.</div>
</figcaption>
</figure>
<figure class="figure-item "
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data-label-slug="fig-6">
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itemprop="contentUrl"
aria-describedby="figure-caption-5"
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data-image-height="497"
data-image-alt="Fig. 6"
data-pmc-id="PMC8695383"
data-figure-id="Fig6">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-5"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/755a3638d783/41379_2021_905_Fig6_HTML.gif"
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<strong class="figure-label">
<p> Fig. 6. Pleuropulmonary blastoma, type IR. </p>
</strong>
<div class="figure-caption-contents"><p> Pleuropulmonary… </p></div>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 6. Pleuropulmonary blastoma, type IR. </p>
</strong>
<div class="figure-caption-contents"><p> Pleuropulmonary blastoma, type IR showing the characteristic multicystic architecture… </p></div>
</div>
<figcaption id="figure-caption-5" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 6. Pleuropulmonary blastoma, type IR.
</strong>
<div class="figure-caption-contents">Pleuropulmonary blastoma, type IR showing the characteristic multicystic architecture of type I, but in the absence of a primitive small cell or rhabdomyoblastic population only ascertained after a complete microscopic examination of the resected cyst.</div>
</figcaption>
</figure>
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<strong class="figure-label">
<p> Fig. 7. Sertoli-Leydig cell tumor, moderately differentiated… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 7. Sertoli-Leydig cell tumor, moderately differentiated in an adolescent presenting with an abdominal mass. </p>
</strong>
</div>
<figcaption id="figure-caption-6" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 7. Sertoli-Leydig cell tumor, moderately differentiated in an adolescent presenting with an abdominal mass.
</strong>
<div class="figure-caption-contents"><b>A</b> Solid foci of this solid and cystic mass showing groups of Sertoli cells and surrounding Leydig cells. <b>B</b> Cystic focus with a delicate septum with a cambium layer-like localization of Sertoli cells with its resemblance to PPB type I.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
itemscope itemtype="http://schema.org/ImageObject"
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data-label-slug="fig-8">
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itemprop="contentUrl"
aria-describedby="figure-caption-7"
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data-image-width="660"
data-image-height="168"
data-image-alt="Fig. 8"
data-pmc-id="PMC8695383"
data-figure-id="Fig8">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-7"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/bc4603f8b71a/41379_2021_905_Fig8_HTML.gif"
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<strong class="figure-label">
<p> Fig. 8. Sertoli-Leydig cell tumor, moderately differentiated… </p>
</strong>
</div>
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<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 8. Sertoli-Leydig cell tumor, moderately differentiated in a 15-year-old female. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> A cystic focus…</div>
</div>
<figcaption id="figure-caption-7" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 8. Sertoli-Leydig cell tumor, moderately differentiated in a 15-year-old female.
</strong>
<div class="figure-caption-contents"><b>A</b> A cystic focus showing a group of Sertoli cells beneath a low cuboidal epithelial surface. <b>B</b> Nests of Sertoli cells surrounded by malignant-appearing, primitive-appearing spindle and round cells in another cystic area of the tumor. <b>C</b> MyoD immunostaining showing intense nuclear positivity to corroborate heterologous rhabdomyosarcoma.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
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data-label-slug="fig-9">
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itemprop="contentUrl"
aria-describedby="figure-caption-8"
role="button"
data-image-width="675"
data-image-height="308"
data-image-alt="Fig. 9"
data-pmc-id="PMC8695383"
data-figure-id="Fig9">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-8"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/eab731b8edb7/41379_2021_905_Fig9_HTML.gif"
alt="Fig. 9" />
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<strong class="figure-label">
<p> Fig. 9. Embryonal rhabdomyosarcoma of the cervix. </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 9. Embryonal rhabdomyosarcoma of the cervix. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> A polypoid “vaginal” tumor presented in a…</div>
</div>
<figcaption id="figure-caption-8" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 9. Embryonal rhabdomyosarcoma of the cervix.
</strong>
<div class="figure-caption-contents"><b>A</b> A polypoid “vaginal” tumor presented in a 9-year-old female showing scattered endocervical glands with variably prominent subepithelial concentration of small cells and nodules of cartilage. PPB-type IR was also detected in the lung and a pathogenic heterozygous germline <i>DICER1</i> mutation was detected. <b>B</b> Endocervical gland encircled by embryonal rhabdomyosarcoma in an 11-year-old female with a cervical-vaginal mass. She subsequently developed a Sertoli-Leydig cell tumor at age 13 years and had a pathogenic heterozygous germline <i>DICER1</i> mutation.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
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itemprop="contentUrl"
aria-describedby="figure-caption-9"
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data-image-width="660"
data-image-height="173"
data-image-alt="Fig. 10"
data-pmc-id="PMC8695383"
data-figure-id="Fig10">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-9"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/401608852d78/41379_2021_905_Fig10_HTML.gif"
alt="Fig. 10" />
</a>
<meta itemprop="width" itemtype="http://schema.org/ImageObject" content="660">
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<strong class="figure-label">
<p> Fig. 10. Cystic nephroma, pediatric type. </p>
</strong>
<div class="figure-caption-contents"><b> A… </b></div>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 10. Cystic nephroma, pediatric type. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> Circumscribed multicystic renal lesion in this CT image…</div>
</div>
<figcaption id="figure-caption-9" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 10. Cystic nephroma, pediatric type.
</strong>
<div class="figure-caption-contents"><b>A</b> Circumscribed multicystic renal lesion in this CT image from a 2-year-old male who presented with an abdominal mass. A similar-appearing lesion on CT was present in the lung (PPB type I not shown). <b>B</b> Several delicate septal structures display architectural similarities to PPB type IR. <b>C</b> Multiple cysts in a fibrous stroma and one larger cyst with a hypercellular subepithelial mantle of spindle cells with a resemblance to PPB type I. Overtly sarcomatous elements were not present.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
itemscope itemtype="http://schema.org/ImageObject"
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data-label-slug="fig-11">
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itemprop="contentUrl"
aria-describedby="figure-caption-10"
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data-image-height="437"
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data-figure-id="Fig11">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-10"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/0496412648c6/41379_2021_905_Fig11_HTML.gif"
alt="Fig. 11" />
</a>
<meta itemprop="width" itemtype="http://schema.org/ImageObject" content="675">
<meta itemprop="height" itemtype="http://schema.org/ImageObject" content="437">
<div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true">
<div class="caption-wrap">
<strong class="figure-label">
<p> Fig. 11. Anaplastic sarcoma of kidney (… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 11. Anaplastic sarcoma of kidney ( <i> DICER1 </i> renal sarcoma) in the right kidney of… </p>
</strong>
</div>
<figcaption id="figure-caption-10" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 11. Anaplastic sarcoma of kidney (<i>DICER1</i> renal sarcoma) in the right kidney of a 4-year-old male.
</strong>
<div class="figure-caption-contents"><b>A</b> The solid and cystic neoplasm showing a focus of residual pCN and adjacent cysts with surrounding hypercellular mantles of primitive small cells with rhabdomyoblastic differentiation (not shown) (inset, focus of hyperchromatic cells beneath a cyst). <b>B</b> A focus of primitive small, round and spindle-shaped cells projecting into a cyst with a subepithelial mantle of similar-appearing cells. <b>C</b> Malignant-appearing spindle cells surrounding a focus of neoplastic cartilage. <b>D</b> Focus of undifferentiated round cells with adjacent neoplastic cartilage. As in the case of PPB types II and III, anaplasia is not present in every ASK despite the implication of its appellation.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
itemscope itemtype="http://schema.org/ImageObject"
itemprop="associatedMedia"
data-slide-index="11"
data-label-slug="fig-12">
<a class="figure-link"
href="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/7863b48ac414/41379_2021_905_Fig12_HTML.jpg"
itemprop="contentUrl"
aria-describedby="figure-caption-11"
role="button"
data-image-width="633"
data-image-height="496"
data-image-alt="Fig. 12"
data-pmc-id="PMC8695383"
data-figure-id="Fig12">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-11"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/0ca51b154aa3/41379_2021_905_Fig12_HTML.gif"
alt="Fig. 12" />
</a>
<meta itemprop="width" itemtype="http://schema.org/ImageObject" content="633">
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<div class="caption-wrap">
<strong class="figure-label">
<p> Fig. 12. <i> DICER1 </i> cystic hepatic neoplasm presenting… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 12. <i> DICER1 </i> cystic hepatic neoplasm presenting as a cystic lesion of the liver in… </p>
</strong>
</div>
<figcaption id="figure-caption-11" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 12. <i>DICER1</i> cystic hepatic neoplasm presenting as a cystic lesion of the liver in a 1-year-old male.
</strong>
<div class="figure-caption-contents">A 10.7cm mass with a multicystic cut surface showing one of multiple cysts lined by biliary type epithelium with a subepithelial zone of primitive small cells extending into the surrounding stroma. These cells demonstrated desmin immunopositivity. A pathogenic heterozygous germline mutation in <i>DICER1</i> was detected.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
itemscope itemtype="http://schema.org/ImageObject"
itemprop="associatedMedia"
data-slide-index="12"
data-label-slug="fig-13">
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itemprop="contentUrl"
aria-describedby="figure-caption-12"
role="button"
data-image-width="633"
data-image-height="633"
data-image-alt="Fig. 13"
data-pmc-id="PMC8695383"
data-figure-id="Fig13">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-12"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/1ce071fcb1c1/41379_2021_905_Fig13_HTML.gif"
alt="Fig. 13" />
</a>
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<div class="figure-caption figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="caption" aria-hidden="true">
<div class="caption-wrap">
<strong class="figure-label">
<p> Fig. 13. Hamartomatous polyp with juvenile polyp-like… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 13. Hamartomatous polyp with juvenile polyp-like features. </p>
</strong>
<div class="figure-caption-contents"><p> Small intestinal polyp in a child with… </p></div>
</div>
<figcaption id="figure-caption-12" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 13. Hamartomatous polyp with juvenile polyp-like features.
</strong>
<div class="figure-caption-contents">Small intestinal polyp in a child with PPB showing an ulcerated surface with elongated crypts and a lamina propria with bands of smooth muscle.</div>
</figcaption>
</figure>
<figure class="figure-item tail"
itemscope itemtype="http://schema.org/ImageObject"
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data-label-slug="fig-14">
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aria-describedby="figure-caption-13"
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data-image-height="261"
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data-figure-id="Fig14">
<img class="figure-thumb" itemprop="thumbnail"
id="article-image-13"
src="https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dd48/8695383/62f22baee4ae/41379_2021_905_Fig14_HTML.gif"
alt="Fig. 14" />
</a>
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<strong class="figure-label">
<p> Fig. 14. PPB-like peritoneal sarcoma arising in… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 14. PPB-like peritoneal sarcoma arising in the pelvis of an adolescent female. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> Cystic…</div>
</div>
<figcaption id="figure-caption-13" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 14. PPB-like peritoneal sarcoma arising in the pelvis of an adolescent female.
</strong>
<div class="figure-caption-contents"><b>A</b> Cystic focus of the tumor demonstrating a hypercellular zone of primitive round cells with patchy myogenin positivity (not shown). <b>B</b> Solid areas of the tumor showing undifferentiated sarcoma with round and spindle cell features and an enlarged, anaplastic tumor cell.</div>
</figcaption>
</figure>
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itemprop="associatedMedia"
data-slide-index="14"
data-label-slug="fig-15">
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<img class="figure-thumb" itemprop="thumbnail"
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<strong class="figure-label">
<p> Fig. 15. Poorly differentiated thyroid carcinoma in… </p>
</strong>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 15. Poorly differentiated thyroid carcinoma in a 22-year-old female with a past history of… </p>
</strong>
</div>
<figcaption id="figure-caption-14" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 15. Poorly differentiated thyroid carcinoma in a 22-year-old female with a past history of Wilms tumor at age 7 years and a peritoneal sarcoma with cystic and rhabdomyosarcoma at age 11 years who was a DICER1 heterozygous germline variant.
</strong>
<div class="figure-caption-contents"><b>A</b> The 3.5cm, tan-pink, encapsulated mass showing tumor in the capsule and closely apposed small nests of uniforms tumor cells with dense nuclei and pale cytoplasm. <b>B</b> Trabecular and small follicular profiles of basophilic tumor cells demonstrating scattered mitotic figures.</div>
</figcaption>
</figure>
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data-slide-index="15"
data-label-slug="fig-16">
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<img class="figure-thumb" itemprop="thumbnail"
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<strong class="figure-label">
<p> Fig. 16. Nasal chondromesenchymal hamartoma. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> This…</div>
</div>
</div>
<div class="figure-caption-medium figure-caption-text" aria-hidden="true">
<strong class="figure-label">
<p> Fig. 16. Nasal chondromesenchymal hamartoma. </p>
</strong>
<div class="figure-caption-contents"><b> A </b> This tumor demonstrating a well-differentiated nodule of cartilage with…</div>
</div>
<figcaption id="figure-caption-15" class="figure-caption-full figure-caption-text" itemtype="http://schema.org/ImageObject" itemprop="description">
<strong class="figure-label">
Fig. 16. Nasal chondromesenchymal hamartoma.
</strong>
<div class="figure-caption-contents"><b>A</b> This tumor demonstrating a well-differentiated nodule of cartilage with an accompanying spindle cell stroma without rhabdomyoblastic features. <b>B</b> Another example with a less cellular myxoid stroma and cartilaginous nodules with variation in differentiation.</div>
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