The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases
Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DF...
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| Veröffentlicht in: | British journal of dermatology (1951) 1998-10, Vol.139 (4), p.719-725 |
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| container_title | British journal of dermatology (1951) |
| container_volume | 139 |
| creator | Martin, L. Combemale, P. Dupin, M. Chouvet, B. Kanitakis, J. Bouyssou-Gauthier, M-L. Dubreuil, G. Claudy, A. Grimand, P.S.GRIMAND |
| description | Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid‐like plaques. We believe that DFSP in childhood is probably under‐estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment. |
| doi_str_mv | 10.1046/j.1365-2133.1998.02476.x |
| format | Article |
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It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid‐like plaques. We believe that DFSP in childhood is probably under‐estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.</description><identifier>ISSN: 0007-0963</identifier><identifier>EISSN: 1365-2133</identifier><identifier>DOI: 10.1046/j.1365-2133.1998.02476.x</identifier><identifier>PMID: 10025975</identifier><identifier>CODEN: BJDEAZ</identifier><language>eng</language><publisher>Oxford BSL: Blackwell Science Ltd</publisher><subject>Adolescent ; Adult ; Atrophy ; Biological and medical sciences ; Child, Preschool ; Dermatofibrosarcoma - genetics ; Dermatofibrosarcoma - pathology ; Dermatofibrosarcoma - surgery ; Dermatology ; Dermis - pathology ; Female ; Humans ; Infant ; Male ; Medical sciences ; Tumors of the skin and soft tissue. Premalignant lesions</subject><ispartof>British journal of dermatology (1951), 1998-10, Vol.139 (4), p.719-725</ispartof><rights>British Association of Dermatology</rights><rights>1998 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3836-841690c62f9b389efc951dac2859321b8799bd60a142aa204abd3759dd2e3143</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1046%2Fj.1365-2133.1998.02476.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1046%2Fj.1365-2133.1998.02476.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,777,781,1412,27905,27906,45555,45556</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=2424492$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10025975$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Martin, L.</creatorcontrib><creatorcontrib>Combemale, P.</creatorcontrib><creatorcontrib>Dupin, M.</creatorcontrib><creatorcontrib>Chouvet, B.</creatorcontrib><creatorcontrib>Kanitakis, J.</creatorcontrib><creatorcontrib>Bouyssou-Gauthier, M-L.</creatorcontrib><creatorcontrib>Dubreuil, G.</creatorcontrib><creatorcontrib>Claudy, A.</creatorcontrib><creatorcontrib>Grimand, P.S.GRIMAND</creatorcontrib><title>The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases</title><title>British journal of dermatology (1951)</title><addtitle>British Journal of Dermatology</addtitle><description>Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid‐like plaques. We believe that DFSP in childhood is probably under‐estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Atrophy</subject><subject>Biological and medical sciences</subject><subject>Child, Preschool</subject><subject>Dermatofibrosarcoma - genetics</subject><subject>Dermatofibrosarcoma - pathology</subject><subject>Dermatofibrosarcoma - surgery</subject><subject>Dermatology</subject><subject>Dermis - pathology</subject><subject>Female</subject><subject>Humans</subject><subject>Infant</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Tumors of the skin and soft tissue. Premalignant lesions</subject><issn>0007-0963</issn><issn>1365-2133</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1998</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpNkd9v0zAQxy3ExLrBv4D8gHhLOP-IEyPxAB0bTBVIUyUeeLAujqO6JHVnp9D990vWUu3JJ_nzPd3dhxDKIGcg1Yd1zoQqMs6EyJnWVQ5clirfvyCz08dLMgOAMgOtxDm5SGkNwAQU8IqcMwBe6LKYkd_LlaM4xLBdeUv_YvS4GWhoaeNij0NofR1DwmhDj3Qbw7CrXcRNon5D7cp3zSqE5iNFGt02xKdk8ntqMbn0mpy12CX35vhekuX11-X8W7b4efN9_nmRWVEJlVWSKQ1W8VbXotKutbpgDVpeFVpwVlel1nWjAJnkiBwk1o0oC9003AkmxSV5f2g7jne_c2kwvU_WdR1uXNglozRTZVXACL49gru6d43ZRt9jfDD_rzEC744AJotdOy5qfTpxXHIpNR-xTwfsn-_cw7M2ZpJj1mZyYCYHZpJjnuSYvflyezVVYz475H0a3P6Ux_jHqHLczPz6cWPuQMyvFss7o8Ujgf6RKQ</recordid><startdate>199810</startdate><enddate>199810</enddate><creator>Martin, L.</creator><creator>Combemale, P.</creator><creator>Dupin, M.</creator><creator>Chouvet, B.</creator><creator>Kanitakis, J.</creator><creator>Bouyssou-Gauthier, M-L.</creator><creator>Dubreuil, G.</creator><creator>Claudy, A.</creator><creator>Grimand, P.S.GRIMAND</creator><general>Blackwell Science Ltd</general><general>Blackwell</general><scope>BSCLL</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>199810</creationdate><title>The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases</title><author>Martin, L. ; Combemale, P. ; Dupin, M. ; Chouvet, B. ; Kanitakis, J. ; Bouyssou-Gauthier, M-L. ; Dubreuil, G. ; Claudy, A. ; Grimand, P.S.GRIMAND</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3836-841690c62f9b389efc951dac2859321b8799bd60a142aa204abd3759dd2e3143</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1998</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Atrophy</topic><topic>Biological and medical sciences</topic><topic>Child, Preschool</topic><topic>Dermatofibrosarcoma - genetics</topic><topic>Dermatofibrosarcoma - pathology</topic><topic>Dermatofibrosarcoma - surgery</topic><topic>Dermatology</topic><topic>Dermis - pathology</topic><topic>Female</topic><topic>Humans</topic><topic>Infant</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Tumors of the skin and soft tissue. Premalignant lesions</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Martin, L.</creatorcontrib><creatorcontrib>Combemale, P.</creatorcontrib><creatorcontrib>Dupin, M.</creatorcontrib><creatorcontrib>Chouvet, B.</creatorcontrib><creatorcontrib>Kanitakis, J.</creatorcontrib><creatorcontrib>Bouyssou-Gauthier, M-L.</creatorcontrib><creatorcontrib>Dubreuil, G.</creatorcontrib><creatorcontrib>Claudy, A.</creatorcontrib><creatorcontrib>Grimand, P.S.GRIMAND</creatorcontrib><collection>Istex</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of dermatology (1951)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Martin, L.</au><au>Combemale, P.</au><au>Dupin, M.</au><au>Chouvet, B.</au><au>Kanitakis, J.</au><au>Bouyssou-Gauthier, M-L.</au><au>Dubreuil, G.</au><au>Claudy, A.</au><au>Grimand, P.S.GRIMAND</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases</atitle><jtitle>British journal of dermatology (1951)</jtitle><addtitle>British Journal of Dermatology</addtitle><date>1998-10</date><risdate>1998</risdate><volume>139</volume><issue>4</issue><spage>719</spage><epage>725</epage><pages>719-725</pages><issn>0007-0963</issn><eissn>1365-2133</eissn><coden>BJDEAZ</coden><abstract>Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid‐like plaques. We believe that DFSP in childhood is probably under‐estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.</abstract><cop>Oxford BSL</cop><pub>Blackwell Science Ltd</pub><pmid>10025975</pmid><doi>10.1046/j.1365-2133.1998.02476.x</doi><tpages>7</tpages></addata></record> |
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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete; Oxford University Press Journals All Titles (1996-Current) |
| subjects | Adolescent Adult Atrophy Biological and medical sciences Child, Preschool Dermatofibrosarcoma - genetics Dermatofibrosarcoma - pathology Dermatofibrosarcoma - surgery Dermatology Dermis - pathology Female Humans Infant Male Medical sciences Tumors of the skin and soft tissue. Premalignant lesions |
| title | The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases |
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