Cancino Claudia Marcela H, Gaião Léonilson, Sant'Ana Filho Manoel, Oliveira Flavio Augusto Marsiaj
Porto Alegre, RS, Brazil.
J Contemp Dent Pract. 2007 May 1;8(4):67-73.
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).
NS is a dysmorphic disorder characterized by hypertelorism, short stature, congenital heart defects, short and webbed neck, skeletal anomalies, and bleeding diathesis.
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.
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.
本文旨在描述一例发生在一名14岁女孩身上的下颌骨多发性巨细胞病变，该女孩具有与努南综合征（NS）相关的表型特征。
NS是一种畸形疾病，其特征为眼距增宽、身材矮小、先天性心脏缺陷、短颈并伴有蹼状皮肤、骨骼异常和出血倾向。
一名先前被诊断为NS（散发病例）的14岁女孩，其下颌骨体部和升支出现多个透射性病变。
就临床行为和所描述的影像学特征而言，具有努南样表型的巨细胞病变可被视为一种类巨细胞性龈纤维瘤病。因此，无需手术干预，但在病变的控制和逐渐消退过程中，影像学随访和观察非常重要。
