Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology
Oral and maxillofacial pathologyClinically aggressive central giant cell granulomas in two patients with neurofibromatosis 1
Section snippets
Case 1
In 1996, a 12-year-old boy presented with a large mass of the right palate. Intra-oral examination revealed significant palatal expansion (Fig. 1). The lesion crossed the midline, and extended to both the soft palate and the buccal vestibule. The extent of maxillary destruction was evident on coronal (Fig. 2, A) and axial (Fig. 2, B) CT scans.
The patient’s past medical history was significant for NF1, diagnosed when the patient was 3 years old. Besides multiple café-au-lait macules, axillary
Discussion
Central giant cell granuloma (CGCG) is a benign central lesion of bone, primarily involving the jaws, of variably aggressive nature characterized by aggregates of multinucleated giant cells in a background of cellular vascular fibrous connective tissue and spindle-shaped mononuclear stromal cells, often with extravasation of red blood cells.8 Currently there are no reliable criteria for correlating clinical aggressiveness with histological presentation.9 Recurrences are not uncommon, with an
Conclusion
We report 2 patients with NF1 who presented with central giant cell granulomas (CGCGs) of the jaws. In both cases, the clinical course was characterized by significant osseous destruction and recurrence.
Although the presence of CGCGs of the jaws in patients with NF1 could represent a coincidental association or a genetic linkage, we propose that this phenomenon is most likely related to NF1-mediated osseous dysplasia. Compared to normal bone, the Nf1-haploinsufficient bone in a patient with
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Cited by (32)
Multiple versus solitary giant cell lesions of the jaw: Similar or distinct entities?
2021, BoneCitation Excerpt :Nevertheless, the reports still provide pivotal information that may help to understand the pathogenesis of this condition. When limiting the search by excluding cases related to cherubism, only 77 cases with multifocal disease remain (Table 1) [3,10,106–151,399-401]. In 72 (94%) of these patients, multifocal GCLJ was accompanied by a diagnosis or strong clinical suspicion of either a RASopathy (Noonan syndrome (NS), Neurofibromatosis type 1 (NF1), Noonan syndrome with multiple lentigines (NSML), cardio-facio-cutaneous (CFC) syndrome), Schimmelpenning-Feuerstein-Mims syndrome, Jaffe-Campanacci syndrome, oculo-ectodermal syndrome or RAMON syndrome.
Recurrent multilocular mandibular giant cell granuloma in neurofibromatosis type 1: Evidence for second hit mutation of NF1 gene in the jaw lesion and treatment with curettage and bone substitute materials
2016, Journal of Cranio-Maxillofacial SurgeryCitation Excerpt :However, the follow-up time is too short to claim definite bone restoration in this individual. Indeed, the clinical course of NF1-associated GCG can be characterised by numerous recurrences despite sufficient mechanical curettage and surgical resection (Edwards et al., 2006). The rapid recurrence of GCG is a reason to seek out alternative strategies.
Aggressive giant cell lesion of the jaws: a review of management options and report of a mandibular lesion treated with denosumab
2015, Oral Surgery, Oral Medicine, Oral Pathology and Oral RadiologyNeurofibromatosis type 1 associated with bilateral central giant cell granuloma of the mandible
2011, Journal of Cranio-Maxillofacial SurgeryCitation Excerpt :The classification of the World Health Organization (Barnes et al., 2005) defined this entity as localized benign but sometimes aggressive osteolytic proliferation. Reports of CGCG in patients with NF1 have been described in the literature (Kerl and Schroll, 1971; Davis and Tideman, 1977; Van Damme and Mooren, 1994; Ardekian et al., 1999; Ruggieri et al., 1999; Krammer et al., 2003; de Lange and Van den Akker, 2005; Edwards et al., 2006). In the present report, a case of mandibular bilateral CGCG in a patient with NF1 was conservatively but successfully treated by adequate surgical curettage of mandibular bone lesions.
Central giant-cell granuloma in a patient with neurofibromatosis type 1: 7 years of follow-up
2024, Journal of the Korean Association of Oral and Maxillofacial SurgeonsSurgical management of mandibular and maxillary central giant cell granuloma
2023, Egyptian Journal of Otolaryngology