{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T15:10:31Z","timestamp":1782486631874,"version":"3.54.5"},"reference-count":0,"publisher":"Sociedad Chilena de Pediatria","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rev Chil Pediatr"],"abstract":"<jats:p>El tumor de Bednar es un sarcoma de bajo grado, infrecuente, considerado como la variante pigmentada del dermatofibrosarcoma protuberans (DFSP).Objetivo: describir las caracter\u00edsticas cl\u00ednicas, histopatol\u00f3gicas, el tratamiento y la evoluci\u00f3n de un caso pedi\u00e1trico de esta infrecuente neoplasia.Caso Cl\u00ednico: escolar de 9 a\u00f1os que consult\u00f3 por una p\u00e1pula indurada asintom\u00e1tica, de dos a\u00f1os de evoluci\u00f3n en el dorso del cuarto dedo del pie izquierdo. La biopsia incisional de la lesi\u00f3n fue compatibles con un DFSP pigmentado. El estudio inmunohistoqu\u00edmico mostr\u00f3 positividad intensa para CD34 en toda la lesi\u00f3n, con factor XIIIa negativo. Se complement\u00f3 el estudio de la pieza histol\u00f3gica con citogen\u00e9tica molecular FISH para el gen PDGFB (22q13.1) el cual reflej\u00f3 un patr\u00f3n an\u00f3malo en las c\u00e9lulas tumorales, no as\u00ed en los melanocitos ni en la piel peritumoral. Se realiz\u00f3 cirug\u00eda microgr\u00e1fica de Mohs diferida con cobertura mediante sustituto d\u00e9rmico, sin recidiva ni recurrencia tumoral a los 5 a\u00f1os de seguimiento.Conclusiones: El DFSP pigmentado es un sarcoma de bajo grado, que muy infrecuentemente se presenta en pacientes pedi\u00e1tricos. Las variantes cl\u00e1sica y pigmentada deben ser sospechadas ante una lesi\u00f3n papulonodular \u00fanica, de crecimiento lento y progresivo, con presencia de c\u00e9lulas fusiformes con patr\u00f3n estoriforme en la biopsia y con estudio inmunohistoqu\u00edmico positivo para CD34. Es una entidad con buen pron\u00f3stico, con escaso riesgo de recurrencia y met\u00e1stasis, si se logra la realizaci\u00f3n de una extirpaci\u00f3n completa<\/jats:p>","DOI":"10.32641\/rchped.v91i1.1303","type":"journal-article","created":{"date-parts":[[2020,2,6]],"date-time":"2020-02-06T02:13:10Z","timestamp":1580955190000},"page":"99","source":"Crossref","is-referenced-by-count":12,"title":["Dermatofibrosarcoma protuberans pigmentado: descripci\u00f3n de un caso pedi\u00e1trico"],"prefix":"10.32641","volume":"91","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7609-2736","authenticated-orcid":false,"given":"LM","family":"Nieto-Benito","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatriz","family":"Berenguer-Fr\u00f6hner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronica","family":"Parra-Blanco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minia","family":"Campos-Dominguez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"17639","published-online":{"date-parts":[[2020,2,6]]},"container-title":["Revista Chilena de Pediatr\u00eda"],"original-title":[],"link":[{"URL":"http:\/\/www.revistachilenadepediatria.cl\/index.php\/rchped\/article\/viewFile\/1303\/1410","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.revistachilenadepediatria.cl\/index.php\/rchped\/article\/viewFile\/1303\/1410","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,12]],"date-time":"2021-04-12T18:28:09Z","timestamp":1618252089000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.revistachilenadepediatria.cl\/index.php\/rchped\/article\/view\/1303"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2,6]]},"references-count":0,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2020,2,6]]}},"URL":"https:\/\/doi.org\/10.32641\/rchped.v91i1.1303","relation":{},"ISSN":["0370-4106","0370-4106"],"issn-type":[{"value":"0370-4106","type":"electronic"},{"value":"0370-4106","type":"print"}],"subject":[],"published":{"date-parts":[[2020,2,6]]}}}