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Bilateral Psammomatoid Ossifying Fibroma of Maxilla

机译:上颌骨双侧类窦性骨化性纤维瘤

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Ossifying-Fibroma (OF) is a benign neoplasm histopathologically composed of fibrocellularstroma and variable amount of mineralized material. Generally, these tumours in jaw bones are found as solitary lesions. Occurrence of these tumours as Multiple Ossifying Fibromas (MOF) in jaw bones is very rare. The studies reported till now have found an association between MOF and Hyperparathyroid Jaw Tumour Syndrome (HPT-JT). HPT-JT is always associated with the genetic mutation in tumour suppressor gene HRPT2 which encodes protein parafibromin. The simultaneous presentation of ossifying fibroma and other features of HPT-JT may or may not be present. Thus, the expression of parafibromin needs to be evaluated in MOF to rule out it?s association with HPT-JT. The present case of 24 years old female patient, showed bilateral maxillary Psammomatoid Juvenile Ossifying Fibroma (PsJOF). The patient was further investigated for association with HPT-JT. Other finding such as blood serum calcium and parathyroid assay were normal. A negative expression of parafibromin in the tumour tissue suggested an association with HPT-JT. Thus, evaluation of parafibromin can help in accurate diagnosis and treatment of MOF of jaws.
机译:骨化纤维瘤(OF)是一种良性肿瘤,在组织病理学上由纤维细胞基质和不同量的矿物质组成。通常,发现颌骨中的这些肿瘤是孤立的病变。颌骨多发性骨化性纤维瘤(MOF)导致这些肿瘤的发生非常罕见。迄今为止报道的研究发现,MOF与甲状旁腺功能亢进性颌骨肿瘤综合征(HPT-JT)之间存在关联。 HPT-JT始终与编码蛋白对纤维蛋白的肿瘤抑制基因HRPT2中的基因突变相关。可能同时或可能不会同时出现骨化性纤维瘤和HPT-JT的其他特征。因此,需要在MOF中评估副纤蛋白的表达以排除其与HPT-JT的关联。本例为24岁的女性患者,表现为双侧上颌类类滑膜少年骨化性纤维瘤(PsJOF)。进一步检查了患者与HPT-JT的关联。其他发现,例如血清钙和甲状旁腺检测正常。肿瘤组织中副纤蛋白的阴性表达提示与HPT-JT相关。因此,评估副纤蛋白有助于准确诊断和治疗颌骨的MOF。

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