Bilateral Central Giant Cell Granuloma of the mandibular angle in three females from the same family
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Bilateral Central Giant Cell Granuloma of the mandibular angle in three females from the same family Simona Tecco1* , Silvia Caruso2, Alessandro Nota1,2, Pietro Leocata3, Gianluca Cipollone4, Roberto Gatto2 and Tommaso Cutilli5
Abstract In literature there are few reports about multiple CGCG. But this is the first report of bilateral CGCG of the mandibular angles in three females from the same family. This report describes three cases of females from the same family - a mother and two young daughters - with bilateral CGCG in their jaw angles. All the lesions were surgically removed and the histopathologic diagnosis was always identical: giant cell central granulomas, with patterns that were absolutely superimposable between them and with that of the mother. The hypothesis is that this presentation of CGCG may be defined as hereditary bilateral CGCG of the mandibular angles (or also, cherubism-like lesions). Keywords: Central Giant cells granuloma, Cherubism-like lesions, Case series
Main text Central giant cell granuloma (CGCG) is defined by the World Health Organization as an intraosseous lesion consisting of cellular fibrous tissue that contains multiple foci of haemorrhage, aggregations of multiple nucleated giant cells, and occasionally trabeculae of woven bone [1]. It is uncommon (7% of all benign jaw lesions), and the biologic behaviour ranges from quiescent to aggressive, with pain, root resorption and a tendency to recurrence after excision [1]. In the great part of cases, CGCG lesion is unilateral. Sometime the lesion is located in a mandibular angle. And very few rare cases are reported in literature of bilateral CGCG located at the two angles of the mandible [2–4]. A case of bilateral CGCG of the mandibular angle has been reported in a 12 years old female, and classified as idiopathic, as none of the family members of the young girl presented with a similar lesion [2]. Another sporadic case has been reported in an 18 years * Correspondence: [email protected]; [email protected] 1 Dental School, Vita-Salute San Raffaele University, via Olgettina 58, Milan, Italy Full list of author information is available at the end of the article
old girl, associated with neurofibromatosis type 1 [3]. Finally, another case of bilateral CGCG of the mandibular angle was reported in a 8 years old female with Noonan’s syndrome [4]. In this cases series, we describe the first report in literature of a repetitive bilateral CGCG of the two mandibular angles, in three females from the same family. These rare presentations of CGCG may be defined as hereditary bilateral CGCG of the mandibular angles or also cherubism-like lesions. In 1990, a 24-year-old young athlete was exposed to clinical observation at the maxillofacial surgery of the University of L’Aquila, central Italy, for the appearance of two osteolytic lesions at branches and mandibular angles (Fig. 1). These lesions appeared symmetrical to radiological examinations (Fig. 2a, b). The patient underwent surgical intervention and histological e
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