Abstract：
OBJECTIVE: The SOX10 transcription factor is important for the maturation of oligodendrocytes involved in central nervous system (CNS) myelination. Currently, very little information exists about its expression and potential use in CNS tumour diagnoses. The aim of our study was to characterize the expression of SOX10 in a large cohort of CNS tumours and to evaluate its potential use as a biomarker.
METHODS: We performed immunohistochemistry (IHC) for SOX10 and OLIG2 in a series of 683 cases of adult- and paediatric-type CNS tumours from different subtypes. The nuclear immunostaining results for SOX10 and OLIG2 were scored as positive (≥10% positive tumour cells) or negative.
RESULTS: OLIG2 and SOX10 were positive in diffuse midline gliomas (DMG), H3-mutant, and EZHIP-overexpressed. However, in all DMG, EGFR-mutant, SOX10 was constantly negative. In diffuse paediatric-type high-grade gliomas (HGG), all RTK1 cases were positive for both OLIG2 and SOX10. RTK2 cases were all negative for both OLIG2 and SOX10. MYCN cases variably expressed OLIG2 and were all immunonegative for SOX10. In glioblastoma, IDH-wildtype, OLIG2 was mostly positive, but SOX10 was variably expressed, depending on the epigenetic subtype. All circumscribed astrocytic gliomas were positive for both OLIG2 and SOX10 except pleomorphic xanthoastrocytomas, astroblastomas, MN1-altered, and subependymal giant cell astrocytomas. SOX10 was negative in ependymomas, meningiomas, pinealoblastomas, choroid plexus tumours, intracranial Ewing sarcomas, and embryonal tumours except neuroblastoma, FOXR2-activated.
CONCLUSIONS: To conclude, SOX10 can be incorporated into the IHC panel routinely used by neuropathologists in the diagnostic algorithm of embryonal tumours and for the subtyping of paediatric and adult-type HGG.
METHODS: We performed immunohistochemistry (IHC) for SOX10 and OLIG2 in a series of 683 cases of adult- and paediatric-type CNS tumours from different subtypes. The nuclear immunostaining results for SOX10 and OLIG2 were scored as positive (≥10% positive tumour cells) or negative.
RESULTS: OLIG2 and SOX10 were positive in diffuse midline gliomas (DMG), H3-mutant, and EZHIP-overexpressed. However, in all DMG, EGFR-mutant, SOX10 was constantly negative. In diffuse paediatric-type high-grade gliomas (HGG), all RTK1 cases were positive for both OLIG2 and SOX10. RTK2 cases were all negative for both OLIG2 and SOX10. MYCN cases variably expressed OLIG2 and were all immunonegative for SOX10. In glioblastoma, IDH-wildtype, OLIG2 was mostly positive, but SOX10 was variably expressed, depending on the epigenetic subtype. All circumscribed astrocytic gliomas were positive for both OLIG2 and SOX10 except pleomorphic xanthoastrocytomas, astroblastomas, MN1-altered, and subependymal giant cell astrocytomas. SOX10 was negative in ependymomas, meningiomas, pinealoblastomas, choroid plexus tumours, intracranial Ewing sarcomas, and embryonal tumours except neuroblastoma, FOXR2-activated.
CONCLUSIONS: To conclude, SOX10 can be incorporated into the IHC panel routinely used by neuropathologists in the diagnostic algorithm of embryonal tumours and for the subtyping of paediatric and adult-type HGG.
摘要：
目的：SOX10转录因子对于参与中枢神经系统（CNS）髓鞘形成的少突胶质细胞的成熟很重要。目前,关于其表达和在中枢神经系统肿瘤诊断中的潜在用途的信息很少。我们研究的目的是表征大量CNS肿瘤中SOX10的表达，并评估其作为生物标志物的潜在用途。
方法：我们在一系列683例不同亚型的成人和儿科型CNS肿瘤中对SOX10和OLIG2进行了免疫组织化学（IHC）。SOX10和OLIG2的核免疫染色结果评分为阳性（≥10％阳性肿瘤细胞）或阴性。
结果：OLIG2和SOX10在弥漫性中线胶质瘤（DMG）中呈阳性，H3-突变体,和EZHIP过度表达。然而,在所有DMG中，EGFR突变体，SOX10一直为负。在弥漫性儿科型高级别神经胶质瘤（HGG）中，所有RTK1病例的OLIG2和SOX10均为阳性.RTK2病例对OLIG2和SOX10均为阴性。MYCN病例可变表达OLIG2，并且对SOX10均免疫阴性。在胶质母细胞瘤中，IDH-野生型,OLIG2大部分是积极的，但是SOX10的表达方式不同，取决于表观遗传亚型。除多形性黄色星形细胞瘤外，所有局限性星形细胞胶质瘤的OLIG2和SOX10均为阳性，星形母细胞瘤,MN1-改变,室管膜下巨细胞星形细胞瘤.室管膜瘤中SOX10呈阴性,脑膜瘤,松果体母细胞瘤,脉络丛肿瘤,颅内尤因肉瘤,和胚胎性肿瘤，除了神经母细胞瘤，FOXR2激活。
结论：总而言之，SOX10可以纳入神经病理学家在胚胎性肿瘤的诊断算法以及儿科和成人型HGG亚型中常规使用的IHC组中。
方法：我们在一系列683例不同亚型的成人和儿科型CNS肿瘤中对SOX10和OLIG2进行了免疫组织化学（IHC）。SOX10和OLIG2的核免疫染色结果评分为阳性（≥10％阳性肿瘤细胞）或阴性。
结果：OLIG2和SOX10在弥漫性中线胶质瘤（DMG）中呈阳性，H3-突变体,和EZHIP过度表达。然而,在所有DMG中，EGFR突变体，SOX10一直为负。在弥漫性儿科型高级别神经胶质瘤（HGG）中，所有RTK1病例的OLIG2和SOX10均为阳性.RTK2病例对OLIG2和SOX10均为阴性。MYCN病例可变表达OLIG2，并且对SOX10均免疫阴性。在胶质母细胞瘤中，IDH-野生型,OLIG2大部分是积极的，但是SOX10的表达方式不同，取决于表观遗传亚型。除多形性黄色星形细胞瘤外，所有局限性星形细胞胶质瘤的OLIG2和SOX10均为阳性，星形母细胞瘤,MN1-改变,室管膜下巨细胞星形细胞瘤.室管膜瘤中SOX10呈阴性,脑膜瘤,松果体母细胞瘤,脉络丛肿瘤,颅内尤因肉瘤,和胚胎性肿瘤，除了神经母细胞瘤，FOXR2激活。
结论：总而言之，SOX10可以纳入神经病理学家在胚胎性肿瘤的诊断算法以及儿科和成人型HGG亚型中常规使用的IHC组中。
