■在极少数报告中从理论上声称牙性囊肿(DC)与Gorlin-Goltz综合征(GGS)之间的关联,很少有临床基础。本报告的目的是介绍一例显示GGS特征的患者下颌骨中多个DC的独特病例。
方法:一名63岁的男性患者表现为下颌骨多个囊肿样病变,并伴有GGS的一些临床和放射学特征,并提高了牙源性角化囊肿(OKC)的悬浮。患者接受了这些囊肿的袋化和摘除术,组织病理学检查证实了DCs的诊断。
■在本报告中,患者出现与下颌骨中发现的多个单眼射线可透性病变相关的症状,临床和放射学特征高度怀疑OKC与GGS相关.然而,围手术期的发现增加了DC的怀疑,组织病理学证实了这一点。有趣的是,GGS是一种遗传性常染色体显性遗传疾病,由修补型肿瘤抑制基因(PTCH)的突变引起。先前的研究表明DC中的这种基因改变;这可能与本报告中发现的关联的发病机理有关。
结论:本报告介绍了一例显示GGS特征的患者下颌骨双侧DC。因此,该报告验证了DC和GGS之间非常罕见的关联。这可以帮助牙医和医生达到GGS的准确和早期诊断。
UNASSIGNED: The association between Dentigerous cysts (DCs) and Gorlin-Goltz syndrome (GGS) was claimed theoretically in a very few
reports, with very few clinical foundations. The aim of this report was to present a unique
case of multiple DCs in the mandible in a patient showing features of GGS.
METHODS: A 63-year-old male patient presented with multiple cyst-like lesions in the mandible associated with some clinical and radiological features of GGS, and that raised the suspension of odontogenic keratocyst (OKC). The patient underwent marsupialization and enucleation of these cysts, and the histopathological examination confirmed the diagnosis of DCs.
UNASSIGNED: In this report, the patient presented with symptoms related to multiple unilocular radiolucent lesions found in the mandible and the clinical and radiological features were highly suggestive of OKCs associated with GGS. However, the perioperative findings raised the suspicion of DCs, which was confirmed by histopathology. Interestingly, GGS is an inherited autosomal dominant disorder arising from mutations in the patched tumor suppressor gene (PTCH). Previous studies showed this gene alteration in DCs; this can possibly be implicated in the pathogenesis of the association found in this report.
CONCLUSIONS: This report presented a
case of bilateral DC in the mandible in a patient showing features of GGS. Therefore, this report verified the very rare association between DC and GGS. This may help dentists and physicians in reaching an accurate and early diagnosis of GGS.