Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years’ experience of a reference centre in Spain
Background Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center. Materials and methods We retrospectively included pati...
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| Veröffentlicht in: | Clinical & translational oncology 2019-11, Vol.21 (11), p.1573-1577 |
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| creator | Castelo, B. Viñal, D. Maseda, R. Ostios, L. Sánchez, D. García-Salvatierra, B. Escámez, M. J. Martínez-Santamaría, L. Del Río, M. Mora-Rillo, M. Vilches, Y. Beato, M. J. López Gutiérrez, J. C. Romero, N. Santos, C. Miranda, J. de Lucas, R. |
| description | Background
Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center.
Materials and methods
We retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018.
Results
Fourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2–29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6–242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94–11.61).
Conclusions
cSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients. |
| doi_str_mv | 10.1007/s12094-019-02073-3 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2191011162</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2191011162</sourcerecordid><originalsourceid>FETCH-LOGICAL-c347t-f73768d753c4ae5e209c5e937bf86bc5b796d4dcf2805cb6384707b4cbd30acf3</originalsourceid><addsrcrecordid>eNp9UUFu1TAQtRCIlsIFWCAv2QTGcRIn7FBVClIlFoDEznKcSesqsVNPUpEd12DXQ3ACjsJJcJoPS1Yz43nvjWceY88FvBIA6jWJHJoiA9FkkIOSmXzAjkXVNJmEsnx4yKGovx6xJ0TXkF4rIR6zIwl1VSTOMft5NrkORxeGcLly4zvuzbxEM_ArR3OIKw89t8tsPIaFON0sZtwSi8PArYnW-TAa7jyPaJHI3SLvVppjmK6c5bipxzEMKzni7TIMgQyfzOzQz_SG5_DrbkUT6ff3Hxy_TRhTw-I21CTFHuN9aRM64jbl02Scf8oe9WYgfHaIJ-zLu7PPp--zi4_nH07fXmRWFmrOeiVVVXeqlLYwWGK6li2xkart66q1Zauaqis62-c1lLatZF0oUG1h206Csb08YS933SmGmwVp1qOjbfP9GjoXjQAhRJUnaL5DbQxE6ed6im40cdUC9OaW3t3SyS1975aWifTioL-0I3b_KH_tSQC5Ayi1_CVGfR2W6NPO_5P9AyOCpvs</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2191011162</pqid></control><display><type>article</type><title>Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years’ experience of a reference centre in Spain</title><source>MEDLINE</source><source>SpringerLink Journals</source><creator>Castelo, B. ; Viñal, D. ; Maseda, R. ; Ostios, L. ; Sánchez, D. ; García-Salvatierra, B. ; Escámez, M. J. ; Martínez-Santamaría, L. ; Del Río, M. ; Mora-Rillo, M. ; Vilches, Y. ; Beato, M. J. ; López Gutiérrez, J. C. ; Romero, N. ; Santos, C. ; Miranda, J. ; de Lucas, R.</creator><creatorcontrib>Castelo, B. ; Viñal, D. ; Maseda, R. ; Ostios, L. ; Sánchez, D. ; García-Salvatierra, B. ; Escámez, M. J. ; Martínez-Santamaría, L. ; Del Río, M. ; Mora-Rillo, M. ; Vilches, Y. ; Beato, M. J. ; López Gutiérrez, J. C. ; Romero, N. ; Santos, C. ; Miranda, J. ; de Lucas, R.</creatorcontrib><description>Background
Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center.
Materials and methods
We retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018.
Results
Fourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2–29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6–242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94–11.61).
Conclusions
cSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.</description><identifier>ISSN: 1699-048X</identifier><identifier>EISSN: 1699-3055</identifier><identifier>DOI: 10.1007/s12094-019-02073-3</identifier><identifier>PMID: 30864020</identifier><language>eng</language><publisher>Cham: Springer International Publishing</publisher><subject>Adolescent ; Adult ; Brief Research Article ; Carcinoma, Squamous Cell - epidemiology ; Carcinoma, Squamous Cell - etiology ; Carcinoma, Squamous Cell - mortality ; Carcinoma, Squamous Cell - therapy ; Epidermolysis Bullosa Dystrophica - complications ; Epidermolysis Bullosa Dystrophica - mortality ; Female ; Humans ; Lung Neoplasms - secondary ; Male ; Medicine ; Medicine & Public Health ; Neoplasm Recurrence, Local ; Oncology ; Prognosis ; Retrospective Studies ; Skin Neoplasms - epidemiology ; Skin Neoplasms - etiology ; Skin Neoplasms - mortality ; Skin Neoplasms - therapy ; Spain - epidemiology ; Time Factors ; Young Adult</subject><ispartof>Clinical & translational oncology, 2019-11, Vol.21 (11), p.1573-1577</ispartof><rights>Federación de Sociedades Españolas de Oncología (FESEO) 2019</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c347t-f73768d753c4ae5e209c5e937bf86bc5b796d4dcf2805cb6384707b4cbd30acf3</citedby><cites>FETCH-LOGICAL-c347t-f73768d753c4ae5e209c5e937bf86bc5b796d4dcf2805cb6384707b4cbd30acf3</cites><orcidid>0000-0002-9905-3270</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s12094-019-02073-3$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s12094-019-02073-3$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,777,781,27905,27906,41469,42538,51300</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30864020$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Castelo, B.</creatorcontrib><creatorcontrib>Viñal, D.</creatorcontrib><creatorcontrib>Maseda, R.</creatorcontrib><creatorcontrib>Ostios, L.</creatorcontrib><creatorcontrib>Sánchez, D.</creatorcontrib><creatorcontrib>García-Salvatierra, B.</creatorcontrib><creatorcontrib>Escámez, M. J.</creatorcontrib><creatorcontrib>Martínez-Santamaría, L.</creatorcontrib><creatorcontrib>Del Río, M.</creatorcontrib><creatorcontrib>Mora-Rillo, M.</creatorcontrib><creatorcontrib>Vilches, Y.</creatorcontrib><creatorcontrib>Beato, M. J.</creatorcontrib><creatorcontrib>López Gutiérrez, J. C.</creatorcontrib><creatorcontrib>Romero, N.</creatorcontrib><creatorcontrib>Santos, C.</creatorcontrib><creatorcontrib>Miranda, J.</creatorcontrib><creatorcontrib>de Lucas, R.</creatorcontrib><title>Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years’ experience of a reference centre in Spain</title><title>Clinical & translational oncology</title><addtitle>Clin Transl Oncol</addtitle><addtitle>Clin Transl Oncol</addtitle><description>Background
Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center.
Materials and methods
We retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018.
Results
Fourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2–29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6–242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94–11.61).
Conclusions
cSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Brief Research Article</subject><subject>Carcinoma, Squamous Cell - epidemiology</subject><subject>Carcinoma, Squamous Cell - etiology</subject><subject>Carcinoma, Squamous Cell - mortality</subject><subject>Carcinoma, Squamous Cell - therapy</subject><subject>Epidermolysis Bullosa Dystrophica - complications</subject><subject>Epidermolysis Bullosa Dystrophica - mortality</subject><subject>Female</subject><subject>Humans</subject><subject>Lung Neoplasms - secondary</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Neoplasm Recurrence, Local</subject><subject>Oncology</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Skin Neoplasms - epidemiology</subject><subject>Skin Neoplasms - etiology</subject><subject>Skin Neoplasms - mortality</subject><subject>Skin Neoplasms - therapy</subject><subject>Spain - epidemiology</subject><subject>Time Factors</subject><subject>Young Adult</subject><issn>1699-048X</issn><issn>1699-3055</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9UUFu1TAQtRCIlsIFWCAv2QTGcRIn7FBVClIlFoDEznKcSesqsVNPUpEd12DXQ3ACjsJJcJoPS1Yz43nvjWceY88FvBIA6jWJHJoiA9FkkIOSmXzAjkXVNJmEsnx4yKGovx6xJ0TXkF4rIR6zIwl1VSTOMft5NrkORxeGcLly4zvuzbxEM_ArR3OIKw89t8tsPIaFON0sZtwSi8PArYnW-TAa7jyPaJHI3SLvVppjmK6c5bipxzEMKzni7TIMgQyfzOzQz_SG5_DrbkUT6ff3Hxy_TRhTw-I21CTFHuN9aRM64jbl02Scf8oe9WYgfHaIJ-zLu7PPp--zi4_nH07fXmRWFmrOeiVVVXeqlLYwWGK6li2xkart66q1Zauaqis62-c1lLatZF0oUG1h206Csb08YS933SmGmwVp1qOjbfP9GjoXjQAhRJUnaL5DbQxE6ed6im40cdUC9OaW3t3SyS1975aWifTioL-0I3b_KH_tSQC5Ayi1_CVGfR2W6NPO_5P9AyOCpvs</recordid><startdate>20191101</startdate><enddate>20191101</enddate><creator>Castelo, B.</creator><creator>Viñal, D.</creator><creator>Maseda, R.</creator><creator>Ostios, L.</creator><creator>Sánchez, D.</creator><creator>García-Salvatierra, B.</creator><creator>Escámez, M. J.</creator><creator>Martínez-Santamaría, L.</creator><creator>Del Río, M.</creator><creator>Mora-Rillo, M.</creator><creator>Vilches, Y.</creator><creator>Beato, M. J.</creator><creator>López Gutiérrez, J. C.</creator><creator>Romero, N.</creator><creator>Santos, C.</creator><creator>Miranda, J.</creator><creator>de Lucas, R.</creator><general>Springer International Publishing</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-9905-3270</orcidid></search><sort><creationdate>20191101</creationdate><title>Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years’ experience of a reference centre in Spain</title><author>Castelo, B. ; Viñal, D. ; Maseda, R. ; Ostios, L. ; Sánchez, D. ; García-Salvatierra, B. ; Escámez, M. J. ; Martínez-Santamaría, L. ; Del Río, M. ; Mora-Rillo, M. ; Vilches, Y. ; Beato, M. J. ; López Gutiérrez, J. C. ; Romero, N. ; Santos, C. ; Miranda, J. ; de Lucas, R.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c347t-f73768d753c4ae5e209c5e937bf86bc5b796d4dcf2805cb6384707b4cbd30acf3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Brief Research Article</topic><topic>Carcinoma, Squamous Cell - epidemiology</topic><topic>Carcinoma, Squamous Cell - etiology</topic><topic>Carcinoma, Squamous Cell - mortality</topic><topic>Carcinoma, Squamous Cell - therapy</topic><topic>Epidermolysis Bullosa Dystrophica - complications</topic><topic>Epidermolysis Bullosa Dystrophica - mortality</topic><topic>Female</topic><topic>Humans</topic><topic>Lung Neoplasms - secondary</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Neoplasm Recurrence, Local</topic><topic>Oncology</topic><topic>Prognosis</topic><topic>Retrospective Studies</topic><topic>Skin Neoplasms - epidemiology</topic><topic>Skin Neoplasms - etiology</topic><topic>Skin Neoplasms - mortality</topic><topic>Skin Neoplasms - therapy</topic><topic>Spain - epidemiology</topic><topic>Time Factors</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Castelo, B.</creatorcontrib><creatorcontrib>Viñal, D.</creatorcontrib><creatorcontrib>Maseda, R.</creatorcontrib><creatorcontrib>Ostios, L.</creatorcontrib><creatorcontrib>Sánchez, D.</creatorcontrib><creatorcontrib>García-Salvatierra, B.</creatorcontrib><creatorcontrib>Escámez, M. J.</creatorcontrib><creatorcontrib>Martínez-Santamaría, L.</creatorcontrib><creatorcontrib>Del Río, M.</creatorcontrib><creatorcontrib>Mora-Rillo, M.</creatorcontrib><creatorcontrib>Vilches, Y.</creatorcontrib><creatorcontrib>Beato, M. J.</creatorcontrib><creatorcontrib>López Gutiérrez, J. C.</creatorcontrib><creatorcontrib>Romero, N.</creatorcontrib><creatorcontrib>Santos, C.</creatorcontrib><creatorcontrib>Miranda, J.</creatorcontrib><creatorcontrib>de Lucas, R.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical & translational oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Castelo, B.</au><au>Viñal, D.</au><au>Maseda, R.</au><au>Ostios, L.</au><au>Sánchez, D.</au><au>García-Salvatierra, B.</au><au>Escámez, M. J.</au><au>Martínez-Santamaría, L.</au><au>Del Río, M.</au><au>Mora-Rillo, M.</au><au>Vilches, Y.</au><au>Beato, M. J.</au><au>López Gutiérrez, J. C.</au><au>Romero, N.</au><au>Santos, C.</au><au>Miranda, J.</au><au>de Lucas, R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years’ experience of a reference centre in Spain</atitle><jtitle>Clinical & translational oncology</jtitle><stitle>Clin Transl Oncol</stitle><addtitle>Clin Transl Oncol</addtitle><date>2019-11-01</date><risdate>2019</risdate><volume>21</volume><issue>11</issue><spage>1573</spage><epage>1577</epage><pages>1573-1577</pages><issn>1699-048X</issn><eissn>1699-3055</eissn><abstract>Background
Cutaneous squamous cell carcinoma (cSCC) is the leading cause of death in patients with recessive dystrophic epidermolysis bullosa (RDEB). We provide the management and prognosis of cSCC in RDEB patients at a Spanish reference center.
Materials and methods
We retrospectively included patients with RDEB attended in La Paz University Hospital from November 1988 to October 2018.
Results
Fourteen patients developed at least one cSCC. Tumors were predominantly well differentiated. Nearly half of the tumors have recurred. Median time to first recurrence was 23.4 months (95% CI: 17.2–29.5). Five patients have developed distant metastases. Median overall survival (mOS) was 136.5 months since the diagnosis of the first cSCC (95% CI: 30.6–242.3). When distant metastases occurred, mOS was 6.78 months (95% CI: 1.94–11.61).
Conclusions
cSCC is a life-threatening complication of RDEB patients. Although tumors are usually well differentiated, they tend to relapse. This is the first Spanish report of cSCC arising in RDEB patients.</abstract><cop>Cham</cop><pub>Springer International Publishing</pub><pmid>30864020</pmid><doi>10.1007/s12094-019-02073-3</doi><tpages>5</tpages><orcidid>https://orcid.org/0000-0002-9905-3270</orcidid></addata></record> |
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| ispartof | Clinical & translational oncology, 2019-11, Vol.21 (11), p.1573-1577 |
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| subjects | Adolescent Adult Brief Research Article Carcinoma, Squamous Cell - epidemiology Carcinoma, Squamous Cell - etiology Carcinoma, Squamous Cell - mortality Carcinoma, Squamous Cell - therapy Epidermolysis Bullosa Dystrophica - complications Epidermolysis Bullosa Dystrophica - mortality Female Humans Lung Neoplasms - secondary Male Medicine Medicine & Public Health Neoplasm Recurrence, Local Oncology Prognosis Retrospective Studies Skin Neoplasms - epidemiology Skin Neoplasms - etiology Skin Neoplasms - mortality Skin Neoplasms - therapy Spain - epidemiology Time Factors Young Adult |
| title | Epidemiology and natural history of cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa patients: 20 years’ experience of a reference centre in Spain |
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