An Overview of Autosomal Dominant Tumour Syndromes with Prominent Features in the Oral and Maxillofacial Region

Jaw tumor in recurrent primary hyperparathyroidism: A case report

“…Hyperparathyroidism in these patients occurs in younger ages contrary to sporadic forms, also parathyroid adenomas are usually single in them, but may be multiple or likely to get recurrences [ 14 ].…”

“…Ossifying fibroma (OF) is a benign neoplasm in the craniofacial region which predominantly affects the mandible and is more common in women [ 12 ]. The OF lesion discrimination from the Brown tumor can be either difficult or impossible for clinical and radiological perspectives, and could be diagnosed only with no improvement after hyperparathyroidism correction and the absence of giant cell lesions in the pathology [ 14 ]. OF treatment includes complete surgical excision of bone tumor and grafting enucleated lesion [ 15 ].…”

Oral maxillary exostosis

“…1 Although theories regarding a possible role of a chronic periosteal inflammation have been suggested, the true etiology of OE remains still unclear. 1,2 Though the appearance reported here is typical of oral exostosis, benign and malignant bone tumors should be considered in the differential diagnosis when the lesion presents as solitary localized enlargement. In addition, such patients should be investigated for Garden's syndrome by an accurate familial anamnesis and subsequently by colonoscopy in doubtful cases, considering the high rate of malignant transformation of intestinal polyps.…”

“…Oral exostoses (OEs) are bony protuberances arising from the buccal or lingual cortical plates of the maxilla and/or the mandible, with a variable prevalence ranging from 8% to 51% in the maxilla and 6%‐32% in the mandible . Although theories regarding a possible role of a chronic periosteal inflammation have been suggested, the true etiology of OE remains still unclear . Though the appearance reported here is typical of oral exostosis, benign and malignant bone tumors should be considered in the differential diagnosis when the lesion presents as solitary localized enlargement.…”

Oral and skin manifestations of tuberous sclerosis complex

“…According to Gavren et al [3], tuberous sclerosis can be associated with cleft lip and palate, higharched palate, bifid uvula, and macroglossia. Cases of bony desmoid fibroids [6,13,17], odontogenic fibroids [10], and myxomas [10] have been reported, with sporadic cases of oral angiomyolipoma [12]. According to Barron et al [12], the treatment of intraosseous fibroblastic lesions is performed by curettage and enucleation.…”