{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T02:10:48Z","timestamp":1791943848439,"version":"4.3.4"},"reference-count":35,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2018,8,17]],"date-time":"2018-08-17T00:00:00Z","timestamp":1534464000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005743","name":"Universit\u00e0 Cattolica del Sacro Cuore","doi-asserted-by":"publisher","award":["70200367"],"award-info":[{"award-number":["70200367"]}],"id":[{"id":"10.13039\/501100005743","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cellular Molecular Medi"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic> mutations in the gastrointestinal (<jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content>) tract can cause <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> stromal tumour (<jats:styled-content style=\"fixed-case\">GIST<\/jats:styled-content>) and inflammatory fibroid polyp (<jats:styled-content style=\"fixed-case\">IFP<\/jats:styled-content>). Hitherto no cell type has been identified as a physiological counterpart of the latter, while interstitial Cajal cells (<jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content>) are considered the precursor of the former. However, <jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content> hyperplasia (<jats:styled-content style=\"fixed-case\">ICCH<\/jats:styled-content>), which strongly supports the <jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content> role in <jats:styled-content style=\"fixed-case\">GIST<\/jats:styled-content> pathogenesis, has been identified in germline <jats:italic><jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content><\/jats:italic>\u2010mutant settings but not in <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic>\u2010mutant ones, challenging the precursor role of <jats:styled-content style=\"fixed-case\">ICC<\/jats:styled-content> for <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic>\u2010driven <jats:styled-content style=\"fixed-case\">GIST<\/jats:styled-content>s. Telocytes are a recently described interstitial cell type, <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>34+\/<jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content>+. Formerly considered fibroblasts, they are found in many organs, including the <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> tract where they are thought to be involved in neurotransmission. Alongside <jats:styled-content style=\"fixed-case\">IFP<\/jats:styled-content>s and gastric <jats:styled-content style=\"fixed-case\">GIST<\/jats:styled-content>s, <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> wall \u201cfibrosis\u201d has been reported in germline <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic>\u2010mutants. Taking the opportunity offered by its presence in a germline <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic>\u2010mutant individual, we demonstrate that this lesion is sustained by hyperplastic telocytes, constituting the <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic>\u2010mutant counterpart of germline <jats:italic><jats:styled-content style=\"fixed-case\">KIT<\/jats:styled-content><\/jats:italic> mutation\u2010associated <jats:styled-content style=\"fixed-case\">ICCH<\/jats:styled-content>. Moreover, our findings support a pathogenetic relationship between telocyte hyperplasia and both <jats:styled-content style=\"fixed-case\">IFP<\/jats:styled-content>s and <jats:italic><jats:styled-content style=\"fixed-case\">PDGFRA<\/jats:styled-content><\/jats:italic>\u2010mutant <jats:styled-content style=\"fixed-case\">GIST<\/jats:styled-content>s. We propose the term \u201ctelocytoma\u201d for defining <jats:styled-content style=\"fixed-case\">IFP<\/jats:styled-content>, as it conveys both the pathogenetic (neoplastic) and histotypic (\u201ctelocytary\u201d) essence of this tumour, unlike <jats:styled-content style=\"fixed-case\">IFP<\/jats:styled-content>, which rather evokes an inflammatory\u2010hyperplastic lesion.<\/jats:p>","DOI":"10.1111\/jcmm.13748","type":"journal-article","created":{"date-parts":[[2018,8,17]],"date-time":"2018-08-17T12:15:01Z","timestamp":1534508101000},"page":"4856-4862","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Telocytes are the physiological counterpart of inflammatory fibroid polyps and <i><scp>PDGFRA<\/scp><\/i>\u2010mutant <scp>GIST<\/scp>s"],"prefix":"10.1111","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9089-5084","authenticated-orcid":false,"given":"Riccardo","family":"Ricci","sequence":"first","affiliation":[{"name":"Department of Pathology Universit\u00e0 Cattolica del Sacro Cuore Fondazione Policlinico Universitario \u201cA. Gemelli\u201d Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Cristina","family":"Giustiniani","sequence":"additional","affiliation":[{"name":"Department of Pathology Universit\u00e0 Cattolica del Sacro Cuore Fondazione Policlinico Universitario \u201cA. Gemelli\u201d Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Gessi","sequence":"additional","affiliation":[{"name":"Department of Pathology Universit\u00e0 Cattolica del Sacro Cuore Fondazione Policlinico Universitario \u201cA. Gemelli\u201d Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Lanza","sequence":"additional","affiliation":[{"name":"Department of Pathology Universit\u00e0 Cattolica del Sacro Cuore Fondazione Policlinico Universitario \u201cA. 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