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Gorlin-Goltz Syndrome and Neoplasms: A Case Study

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Date
2010-12-01
Author
Lopes, Nilza Nelly Fontana [UNIFESP]
Caran, Eliana Maria Monteiro [UNIFESP]
Lee, Maria Lúcia de Martino [UNIFESP]
Silva, Nasjla Saba da [UNIFESP]
Rocha, Andre Caroli
Macedo, Carla R. D. [UNIFESP]
Type
Artigo
ISSN
1053-4628
Is part of
Journal Of Clinical Pediatric Dentistry
DOI
10.17796/jcpd.35.2.x01248284w166485
Metadata
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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.
Citation
Journal Of Clinical Pediatric Dentistry. Birmingham: Journal Pedodontics Inc, v. 35, n. 2, p. 203-206, 2010.
Keywords
Gorlin syndrome
odontogenic keratocysts
basal cell carcinoma
medulloblastoma
acute myeloid leukemia
URI
http://repositorio.unifesp.br/11600/44322
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