Department of Oral Pathology, Peking University School and Hospital of Stomatology, 22 South Avenue Zhongguancun, Haidian District, Beijing, 100081, PR China.
National Engineering Laboratory for Digital and Material Technology of Stomatology, Peking University School and Hospital of Stomatology, Beijing, PR China.
Mod Pathol. 2022 Oct;35(10):1334-1340. doi: 10.1038/s41379-022-01103-w. Epub 2022 Jun 7.
Chronic diffuse sclerosing osteomyelitis of the mandible (DSOM) and fibrous dysplasia (FD) are distinct lesions with overlapping clinicopathological features that complicate their diagnosis. This study aimed to evaluate the efficacy of GNAS mutation analysis in differentiating between these two conditions. DNA samples from patients with DSOM (n = 35) and FD (n = 29) were collected to analyze the presence of GNAS mutations in exons 8 and 9, the two previously reported hotspot regions, using polymerase chain reaction and direct sequencing. Twenty-four of 29 patients (83%) with FD showed missense mutations in codon 201 in exon 8, whereas no mutation was detected in exon 9. No mutations were found in any of the 35 cases with DSOM. We also identified one case with an uncertain diagnosis due to overlapping clinicopathological features of DSOM and FD. A Q227H mutation was detected in this case, that confirmed the diagnosis of FD. Taken together, the findings indicate that mutational analysis of the GNAS is a reliable approach to differentiate between DSOM and FD of the jaw.
下颌慢性弥漫性硬化性骨髓炎(DSOM)和纤维结构不良(FD)是两种具有重叠临床病理特征的不同病变,这使得它们的诊断变得复杂。本研究旨在评估 GNAS 突变分析在区分这两种疾病方面的疗效。收集了 35 例 DSOM 患者和 29 例 FD 患者的 DNA 样本,使用聚合酶链反应和直接测序分析了外显子 8 和 9 中 GNAS 突变的存在情况,这两个外显子是之前报道的两个热点区域。29 例 FD 患者中有 24 例(83%)在外显子 8 的 201 密码子中显示错义突变,而在外显子 9 中未检测到突变。在 35 例 DSOM 患者中均未发现突变。我们还发现了一例由于 DSOM 和 FD 的临床病理特征重叠而具有不确定诊断的病例。在该病例中检测到了 Q227H 突变,从而确诊为 FD。综上所述,研究结果表明,GNAS 的突变分析是区分颌骨 DSOM 和 FD 的可靠方法。