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Published on: February 8, 2019
Giant cell lesions with a Noonan-like phenotype: a case report
Claudia Marcela H Cancino1, Léonilson Gaião, Manoel Sant'Ana Filho
1Porto Alegre, RS, Brazil. marcelaforerocancino@hotmail.com
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.