Gorlin-Goltz Syndrome and Neoplasms: A Case Study

Gorlin-Goltz Syndrome and Neoplasms: A Case Study

Author Lopes, Nilza Nelly Fontana Autor UNIFESP Google Scholar
Caran, Eliana Maria Monteiro Autor UNIFESP Google Scholar
Lee, Maria Lúcia de Martino Autor UNIFESP Google Scholar
Silva, Nasjla Saba da Autor UNIFESP Google Scholar
Rocha, Andre Caroli Google Scholar
Macedo, Carla R. D. Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Universidade de São Paulo (USP)
Abstract 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. 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.
Keywords Gorlin syndrome
odontogenic keratocysts
basal cell carcinoma
acute myeloid leukemia
Language English
Date 2010-12-01
Published in Journal Of Clinical Pediatric Dentistry. Birmingham: Journal Pedodontics Inc, v. 35, n. 2, p. 203-206, 2010.
ISSN 1053-4628 (Sherpa/Romeo, impact factor)
Publisher Journal Pedodontics Inc
Extent 203-206
Origin https://doi.org/10.17796/jcpd.35.2.x01248284w166485
Access rights Closed access
Type Article
Web of Science ID WOS:000287680700015
URI http://repositorio.unifesp.br/11600/44322

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