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VOLUME 8 , ISSUE 4 ( May, 2007 ) > List of Articles

RESEARCH ARTICLE

Giant Cell Lesions with a Noonan-like Phenotype: A Case Report

Manoel Sant'Ana Filho, Claudia Marcela H. Cancino, Léonilson Gaião, Flavio Augusto Marsiaj Oliveira

Citation Information : Filho MS, Cancino CM, Gaião L, Oliveira FA. Giant Cell Lesions with a Noonan-like Phenotype: A Case Report. J Contemp Dent Pract 2007; 8 (4):67-73.

DOI: 10.5005/jcdp-8-4-67

License: CC BY-NC 3.0

Published Online: 01-05-2007

Copyright Statement:  Copyright © 2007; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Aim

The purpose of this article is to describe a case of multiple giant cell lesions of the mandible that occurred in a 14-year-old girl with phenotypic characteristics associated with Noonan Syndrome (NS).

Background

NS is a dysmorphic disorder characterized by hypertelorism, short stature, congenital heart defects, short and webbed neck, skeletal anomalies, and bleeding diathesis.

Report

A 14-year-old girl with a previous diagnosis of NS (sporadic case) presented with multiple radiolucent lesions in the body and ramus of her mandible.

Summary

In terms of clinical behavior and the described radiographic characteristics, giant cells lesions with Noonan-like phenotype can be considered a form of cherubism. Therefore, surgical intervention is not necessary, but radiographic follow-up and observation is very important during the control and gradual regression of the lesions.

Citation

Cancino CMH, Gaião L, Sant'Ana Filho M, Oliveira FAM. Giant Cell Lesions with a Noonan-like Phenotype: A Case Report. J Contemp Dent Pract 2007 May;(8)4:067-073.


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  1. Aspects of Noonan Syndrome. Cedars-Sinai Medical Center: Information Sheet. 2002;http://www.noonansyndrome.org/graham.html.
  2. Noonan Syndrome revisited. J Pediatr. 1999;135(6):667-8.
  3. Noonan Syndrome: a review. Am J Med Genet. 1985;21:493-506.
  4. HA. The Ullrich-Noonan syndrome (Turner Phenotype). Am J Dis Child. 1974;127:48-55.
  5. Mapping a gene for Noonan syndrome to the long arm of chromosome 12. Nat Genet. 1994;8:357-60.
  6. Noonan Syndrome. eMedicine Journal. 2001;2(10):http://http:www.emedicine.com/ped/topic1616.htm.
  7. The first Noonan Syndrome gene: PTPN11, which encodes the protein tyrosine phosphatase SHP-2. Pediatric Res. 2002;52(4):471.
  8. Associated noncardiac malformations in children with congenital heart disease. J Pediatr. 1963;63:468-70.
  9. Noonan-Like/Multiple giant cell lesion syndrome. Am J Med Genet. 1991;40:159-66.
  10. Polyarticular pigmented villonodular synovitis. Am J Roentgenol. 1981;136:821-3.
  11. Central Giant Cell lesions of the jaws: a clinicopathologic study. J Oral Maxillofac Surg. 1986;44:708-13.
  12. The Noonan syndrome/cherubism association. Oral Surg Oral Med Oral Pathol. 1989;67:698-705.
  13. Cherubism in a patient with Noonan Syndrome: Report of a case. J Oral Maxillofac Surg. 1991;49:1014-18.
  14. Multiple central giant cell lesions with a Noonan-like phenotype. Oral Surg Oral Med Oral Pathol. 1993;76:601-7.
  15. Patologia oral e maxilofacial. 2 ed. Guanabara Koogan: Rio de Janeiro; 2004.
  16. Cherubism: a report on three cases. Br J Oral Surg. 1978;16(1):47-56.
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