Diagnostic utility of SOX10 to distinguish malignant peripheral nerve sheath tumor from synovial sarcoma, including intraneural synovial sarcoma.SOX10在鉴别恶性外周神经鞘瘤和滑膜肉瘤（包括神经内滑膜肉瘤）中的诊断价值Kang Y,Pekmezci M,Folpe AL,Ersen A,Horvai AE
AbstractSynovial sarcoma and malignant peripheral nerve sheath tumor pose a significant diagnostic challenge given similar histomorphology. The distinction is further complicated by similar immunophenotype and especially by occasional synovial sarcomas that present as intraneural tumors. Although the presence of a t(X;18) rearrangement or expression of TLE1 can help confirm the diagnosis of synovial sarcoma, negative results for these tests are not diagnostic of malignant peripheral nerve sheath tumor. The SOX10 transcription factor, a putative marker of neural crest differentiation, may have diagnostic utility in this differential, but immunohistochemical data are limited. The goal of the present study was to determine the diagnostic utility of SOX10 to discriminate between synovial sarcoma and malignant peripheral nerve sheath tumor. Forty-eight cases of malignant peripheral nerve sheath tumor, all from patients with documented neurofibromatosis, and 97 cases of genetically confirmed synovial sarcoma, including 4 intraneural synovial sarcomas, were immunohistochemically stained for SOX10. The stain was scored for intensity and fraction of cells staining. Thirty-two of 48 malignant peripheral nerve sheath tumors (67%) were SOX10-positive. The majority of malignant peripheral nerve sheath tumors showed ≥2 staining, but staining did not correlate with grade. By contrast, only 7/97 (7%) synovial sarcomas were SOX10-positive. Only three synovial sarcomas showed ≥2 staining but, importantly, two of these were intraneural synovial sarcoma. Therefore, SOX10 is a specific (93%), albeit not very sensitive (67%), diagnostic marker to support a diagnosis of malignant peripheral nerve sheath tumor over synovial sarcoma. Furthermore, the stain needs to be interpreted with caution in intraneural tumors in order to avoid a potential diagnostic pitfall. It remains to be determined whether SOX10-positive cells in intraneural synovial sarcoma represent entrapped Schwann cells, synovial sarcoma cells or both. 摘要滑膜肉瘤和恶性外周神经鞘瘤由于其相似的组织形态，给两者的诊断提出了巨大的挑战。由于两者相同的免疫表型，尤其是偶尔滑膜肉瘤表现为神经内肿瘤使得两者的鉴别更为困难。尽管 t(X;18)重排的出现或TLE1的表达有助于滑膜肉瘤的确诊，这些结果阴性并不能就诊断为恶性外周神经鞘瘤。SOX10转录因子，作为一个公认的神经嵴标记，在两者的鉴别中可能具有诊断价值，但其免疫组化资料有限。本研究旨在探讨SOX10在鉴别滑膜肉瘤和恶性外周神经鞘瘤中的诊断价值。对48例恶性外周神经鞘瘤（均来自伴明确多发性神经纤维瘤的患者），97例经基因水平证实的滑膜肉瘤、包括4例神经内滑膜肉瘤，进行了SOX10的免疫组化染色，依据染色强度及着色细胞比例进行评分。48例恶性外周神经鞘瘤中22例SOX10(67%)阳性表达，大部分恶性外周神经鞘瘤SOX10染色≥2 ，但染色与分级无关。相反，97例滑膜肉瘤中只有7例（ (7%)SOX10阳性表达。只有3例滑膜肉瘤SOX10染色≥2 ，但是，重要的是，其中两例是神经内滑膜肉瘤。因此，SOX10是一个特异性高(93%)，但敏感性较低(67%)的支持恶性外周神经鞘瘤而不是滑膜肉瘤的诊断标记。此外，有必要注意神经内肿瘤SOX10染色的解释以避免潜在的诊断陷阱。神经内滑膜肉瘤中的SOX10阳性细胞是陷入的施万细胞，还是滑膜肉瘤细胞，或是两者均有表达，还有待确定。 来源：华夏病理网 |
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