Gorlin-Goltz syndrome and neoplasms: a case study.
J Clin Pediatr Dent
; 35(2): 203-6, 2010.
Article
en En
| MEDLINE
| ID: mdl-21417126
UNLABELLED: 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. Bifid 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 nd malignant neoplasias.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Leucemia Promielocítica Aguda
/
Neoplasias Primarias Secundarias
/
Neoplasias del Tronco Encefálico
/
Meduloblastoma
/
Neoplasias Primarias Múltiples
Tipo de estudio:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
/
Screening_studies
Límite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
J Clin Pediatr Dent
Asunto de la revista:
ODONTOLOGIA
/
PEDIATRIA
Año:
2010
Tipo del documento:
Article
País de afiliación:
Brasil
Pais de publicación:
Singapur