Gorlin-Goltz Syndrome and Neoplasms: A Case Study

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dc.contributor.authorLopes, Nilza Nelly Fontana [UNIFESP]
dc.contributor.authorCaran, Eliana Maria Monteiro [UNIFESP]
dc.contributor.authorLee, Maria Lúcia de Martino [UNIFESP]
dc.contributor.authorSilva, Nasjla Saba da [UNIFESP]
dc.contributor.authorRocha, Andre Caroli
dc.contributor.authorMacedo, Carla R. D. [UNIFESP]
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.date.accessioned2018-06-15T17:58:39Z
dc.date.available2018-06-15T17:58:39Z
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 Sao Paulo, Med Sch Sao Paulo, Pediat Oncol Inst, GRAACC, Sao Paulo, SP, Brazil
dc.description.affiliationSao Paulo State Univ, Hosp Clin, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Med Sch Sao Paulo, Pediat Oncol Inst, GRAACC, Sao Paulo, SP, Brazil
dc.description.sourceWeb of Science
dc.format.extent203-206
dc.identifierhttps://doi.org/10.17796/jcpd.35.2.x01248284w166485
dc.identifier.citationJournal Of Clinical Pediatric Dentistry. Birmingham: Journal Pedodontics Inc, v. 35, n. 2, p. 203-206, 2010.
dc.identifier.doi10.17796/jcpd.35.2.x01248284w166485
dc.identifier.issn1053-4628
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/44322
dc.identifier.wosWOS:000287680700015
dc.language.isoeng
dc.publisherJournal Pedodontics Inc
dc.relation.ispartofJournal Of Clinical Pediatric Dentistry
dc.rightsinfo:eu-repo/semantics/restrictedAccess
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.typeinfo:eu-repo/semantics/article
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