Gorlin-Goltz syndrome and neoplasms: a case study
Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a var...
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Veröffentlicht in: | The Journal of clinical pediatric dentistry 2010-12, Vol.35 (2), p.203-206 |
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creator | Lopes, Nilza N F Caran, Eliana M Lee, Maria Lucia Silva, Nasjla Saba Rocha, André Caroli Macedo, Carla R D |
description | Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome.
To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias. |
doi_str_mv | 10.17796/jcpd.35.2.x01248284w166485 |
format | Article |
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To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.</description><identifier>ISSN: 1053-4628</identifier><identifier>EISSN: 1557-5268</identifier><identifier>DOI: 10.17796/jcpd.35.2.x01248284w166485</identifier><identifier>PMID: 21417126</identifier><language>eng</language><publisher>United States</publisher><subject>Basal Cell Nevus Syndrome - pathology ; Brain Stem Neoplasms - radiotherapy ; Brain Stem Neoplasms - surgery ; Child ; Cranial Irradiation ; Dentistry ; Disease Progression ; Fatal Outcome ; Follow-Up Studies ; Humans ; Leukemia, Promyelocytic, Acute - pathology ; Leukemia, Radiation-Induced - pathology ; Male ; Medulloblastoma - radiotherapy ; Medulloblastoma - surgery ; Neoplasm Recurrence, Local - pathology ; Neoplasms, Multiple Primary - pathology ; Neoplasms, Second Primary - pathology ; Spine - radiation effects</subject><ispartof>The Journal of clinical pediatric dentistry, 2010-12, Vol.35 (2), p.203-206</ispartof><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c373t-7830e6720238532272d281d14fd793f8f68e4497b50280ef1636842a7f39d3b73</citedby><cites>FETCH-LOGICAL-c373t-7830e6720238532272d281d14fd793f8f68e4497b50280ef1636842a7f39d3b73</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21417126$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Lopes, Nilza N F</creatorcontrib><creatorcontrib>Caran, Eliana M</creatorcontrib><creatorcontrib>Lee, Maria Lucia</creatorcontrib><creatorcontrib>Silva, Nasjla Saba</creatorcontrib><creatorcontrib>Rocha, André Caroli</creatorcontrib><creatorcontrib>Macedo, Carla R D</creatorcontrib><title>Gorlin-Goltz syndrome and neoplasms: a case study</title><title>The Journal of clinical pediatric dentistry</title><addtitle>J Clin Pediatr Dent</addtitle><description>Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome.
To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.</description><subject>Basal Cell Nevus Syndrome - pathology</subject><subject>Brain Stem Neoplasms - radiotherapy</subject><subject>Brain Stem Neoplasms - surgery</subject><subject>Child</subject><subject>Cranial Irradiation</subject><subject>Dentistry</subject><subject>Disease Progression</subject><subject>Fatal Outcome</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Leukemia, Promyelocytic, Acute - pathology</subject><subject>Leukemia, Radiation-Induced - pathology</subject><subject>Male</subject><subject>Medulloblastoma - radiotherapy</subject><subject>Medulloblastoma - surgery</subject><subject>Neoplasm Recurrence, Local - pathology</subject><subject>Neoplasms, Multiple Primary - pathology</subject><subject>Neoplasms, Second Primary - pathology</subject><subject>Spine - radiation effects</subject><issn>1053-4628</issn><issn>1557-5268</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkMtKw0AUhgdRbK2-ggRcuEqdc-Z2oispWoWCG10P08wEWnIzk6D16Y1aXbg6_-K_HD7GLoDPwZhMX23z1s-FmuP8nQNKQpJvoLUkdcCmoJRJFWo6HDVXIpUaacJOYtxyzkkTP2YTBAkGUE8ZLJuu3NTpsin7jyTuat81VUhc7ZM6NG3pYhWvE5fkLoYk9oPfnbKjwpUxnO3vjL3c3z0vHtLV0_JxcbtKc2FEnxoSPGiDHAUpgWjQI4EHWXiTiYIKTUHKzKwVR-KhAC00SXSmEJkXayNm7PKnt-2a1yHE3labmIeydONjQ7SkCDMAVKPz5seZd02MXShs220q1-0scPuNzH4hs0JZtP-Rjenz_c6wroL_y_4yEp8ltGck</recordid><startdate>20101201</startdate><enddate>20101201</enddate><creator>Lopes, Nilza N F</creator><creator>Caran, Eliana M</creator><creator>Lee, Maria Lucia</creator><creator>Silva, Nasjla Saba</creator><creator>Rocha, André Caroli</creator><creator>Macedo, Carla R D</creator><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></search><sort><creationdate>20101201</creationdate><title>Gorlin-Goltz syndrome and neoplasms: a case study</title><author>Lopes, Nilza N F ; Caran, Eliana M ; Lee, Maria Lucia ; Silva, Nasjla Saba ; Rocha, André Caroli ; Macedo, Carla R D</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c373t-7830e6720238532272d281d14fd793f8f68e4497b50280ef1636842a7f39d3b73</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2010</creationdate><topic>Basal Cell Nevus Syndrome - pathology</topic><topic>Brain Stem Neoplasms - radiotherapy</topic><topic>Brain Stem Neoplasms - surgery</topic><topic>Child</topic><topic>Cranial Irradiation</topic><topic>Dentistry</topic><topic>Disease Progression</topic><topic>Fatal Outcome</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Leukemia, Promyelocytic, Acute - pathology</topic><topic>Leukemia, Radiation-Induced - pathology</topic><topic>Male</topic><topic>Medulloblastoma - radiotherapy</topic><topic>Medulloblastoma - surgery</topic><topic>Neoplasm Recurrence, Local - pathology</topic><topic>Neoplasms, Multiple Primary - pathology</topic><topic>Neoplasms, Second Primary - pathology</topic><topic>Spine - radiation effects</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Lopes, Nilza N F</creatorcontrib><creatorcontrib>Caran, Eliana M</creatorcontrib><creatorcontrib>Lee, Maria Lucia</creatorcontrib><creatorcontrib>Silva, Nasjla Saba</creatorcontrib><creatorcontrib>Rocha, André Caroli</creatorcontrib><creatorcontrib>Macedo, Carla R D</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>The Journal of clinical pediatric dentistry</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Lopes, Nilza N F</au><au>Caran, Eliana M</au><au>Lee, Maria Lucia</au><au>Silva, Nasjla Saba</au><au>Rocha, André Caroli</au><au>Macedo, Carla R D</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Gorlin-Goltz syndrome and neoplasms: a case study</atitle><jtitle>The Journal of clinical pediatric dentistry</jtitle><addtitle>J Clin Pediatr Dent</addtitle><date>2010-12-01</date><risdate>2010</risdate><volume>35</volume><issue>2</issue><spage>203</spage><epage>206</epage><pages>203-206</pages><issn>1053-4628</issn><eissn>1557-5268</eissn><abstract>Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. 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To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.</abstract><cop>United States</cop><pmid>21417126</pmid><doi>10.17796/jcpd.35.2.x01248284w166485</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Basal Cell Nevus Syndrome - pathology Brain Stem Neoplasms - radiotherapy Brain Stem Neoplasms - surgery Child Cranial Irradiation Dentistry Disease Progression Fatal Outcome Follow-Up Studies Humans Leukemia, Promyelocytic, Acute - pathology Leukemia, Radiation-Induced - pathology Male Medulloblastoma - radiotherapy Medulloblastoma - surgery Neoplasm Recurrence, Local - pathology Neoplasms, Multiple Primary - pathology Neoplasms, Second Primary - pathology Spine - radiation effects |
title | Gorlin-Goltz syndrome and neoplasms: a case study |
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