Gorlin-Goltz Syndrome and Neoplasms: A Case Study
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Data
2010-12-01
Autores
Lopes, Nilza N. F.
Caran, Eliana M.
Lee, Maria Lucia
Silva, Nasjla Saba
Rocha, Andre Caroli [UNESP]
Macedo, Carla R. D.
Título da Revista
ISSN da Revista
Título de Volume
Editor
Journal Pedodontics Inc
Resumo
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.
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Palavras-chave
Gorlin syndrome, odontogenic keratocysts, basal cell carcinoma, medulloblastoma, acute myeloid leukemia
Como citar
Journal of Clinical Pediatric Dentistry. Birmingham: Journal Pedodontics Inc, v. 35, n. 2, p. 203-206, 2010.