Gorlin-goltz syndrome and neoplasms: A case study

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dc.contributor.authorLopes, Nilza
dc.contributor.authorCaran, Eliana
dc.contributor.authorLee, Maria Lucia
dc.contributor.authorSilva, Nasjla Saba
dc.contributor.authorRocha, André Caroli [UNESP]
dc.contributor.authorMacEdo, Carla
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionUniversidade Estadual Paulista (UNESP)
dc.date.accessioned2022-04-28T22:36:57Z
dc.date.available2022-04-28T22:36:57Z
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. 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 and malignant neoplasias.en
dc.description.affiliationPediatric Oncology Institute - GRAACC Medical School of São Paulo Federal University of São Paulo, SP
dc.description.affiliationOral and Maxillo-Facial Surgeon at the Hospital das Clínicas São Paulo State University
dc.description.affiliationUnespOral and Maxillo-Facial Surgeon at the Hospital das Clínicas São Paulo State University
dc.format.extent203-206
dc.identifierhttp://dx.doi.org/10.17796/jcpd.35.2.x01248284w166485
dc.identifier.citationJournal of Clinical Pediatric Dentistry, v. 35, n. 2, p. 203-206, 2010.
dc.identifier.doi10.17796/jcpd.35.2.x01248284w166485
dc.identifier.issn1053-4628
dc.identifier.scopus2-s2.0-79952930232
dc.identifier.urihttp://hdl.handle.net/11449/226280
dc.language.isoeng
dc.relation.ispartofJournal of Clinical Pediatric Dentistry
dc.sourceScopus
dc.subjectacute myeloid leukemia
dc.subjectbasal cell carcinoma
dc.subjectGorlin syndrome
dc.subjectmedulloblastoma
dc.subjectodontogenic keratocysts
dc.titleGorlin-goltz syndrome and neoplasms: A case studyen
dc.typeArtigo

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