Gorlin-Goltz Syndrome and Neoplasms: A Case Study

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dc.contributor.authorLopes, Nilza N. F.
dc.contributor.authorCaran, Eliana M.
dc.contributor.authorLee, Maria Lucia
dc.contributor.authorSilva, Nasjla Saba
dc.contributor.authorRocha, Andre Caroli [UNESP]
dc.contributor.authorMacedo, Carla R. D.
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.date.accessioned2014-05-20T15:32:41Z
dc.date.available2014-05-20T15:32:41Z
dc.date.issued2010-12-01
dc.description.abstractGorlin 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. Objective: 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. Mild 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 and malignant neoplasias.en
dc.description.affiliationUniv Fed São Paulo, Med Sch São Paulo, Pediat Oncol Inst, GRAACC, São Paulo, SP, Brazil
dc.description.affiliationSão Paulo State Univ, Hosp Clin, São Paulo, Brazil
dc.description.affiliationUnespSão Paulo State Univ, Hosp Clin, São Paulo, Brazil
dc.format.extent203-206
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/21417126
dc.identifier.citationJournal of Clinical Pediatric Dentistry. Birmingham: Journal Pedodontics Inc, v. 35, n. 2, p. 203-206, 2010.
dc.identifier.issn1053-4628
dc.identifier.urihttp://hdl.handle.net/11449/41528
dc.identifier.wosWOS:000287680700015
dc.language.isoeng
dc.publisherJournal Pedodontics Inc
dc.relation.ispartofJournal of Clinical Pediatric Dentistry
dc.relation.ispartofjcr0.854
dc.relation.ispartofsjr0,469
dc.rights.accessRightsAcesso restrito
dc.sourceWeb of Science
dc.subjectGorlin syndromeen
dc.subjectodontogenic keratocystsen
dc.subjectbasal cell carcinomaen
dc.subjectmedulloblastomaen
dc.subjectacute myeloid leukemiaen
dc.titleGorlin-Goltz Syndrome and Neoplasms: A Case Studyen
dc.typeArtigo
dcterms.rightsHolderJournal Pedodontics Inc

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