Gorlin-Goltz Syndrome and Neoplasms: A Case Study

Date
2010-12-01Author
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
ArtigoISSN
1053-4628Is part of
Journal Of Clinical Pediatric DentistryDOI
10.17796/jcpd.35.2.x01248284w166485Metadata
Show full item recordAbstract
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 syndromeodontogenic keratocysts
basal cell carcinoma
medulloblastoma
acute myeloid leukemia
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