Abstract：
Sinonasal teratocarcinosarcoma (SNTCS) is a rare, aggressive malignancy that displays a heterogeneous combination of malignant blastema-like, epithelial and mesenchymal components. Its exact histogenesis is unknown with hypotheses ranging from true germ cell derivation to origin from pluripotent stem cells. However, despite this tumor\'s multiphenotypic histology, which includes frequent glandular, squamous, and neuroectodermal differentiation similar to adnexal germ cell tumors, SNTCS appears to have some differences from adnexal teratomas. For example, unlike adnexal teratomas, SNTCS has never been described as a component in a mixed germ cell tumor. Accurate recognition of SNTCS is difficult due to its rarity and histologic overlap with other sinonasal tumors. It is even more problematic on biopsy, since not all elements may be present in small samples. SNTCS can also share similar staining patterns with other neoplasms in the differential diagnosis. A recent study found nuclear β-catenin expression in a single TCS, but this has yet to be confirmed in additional cases. SALL-4, a marker of germ cell tumors, has not been examined. We performed β-catenin and SALL-4 immunohistochemistry on whole sections of 7 SNTCS and 19 other sinonasal neoplasms to assess whether β-catenin and SALL-4 are of utility in establishing a diagnosis of SNTCS. Intensity of expression and percentage of staining was noted for each tumor. For SNTCS, distribution of staining within each histologic component (immature neuroectodermal, epithelial, and mesenchymal) was also documented. Nuclear β-catenin expression was not identified in any SNTCS, with all cases demonstrating membranous expression (6 cases) or cytoplasmic and membranous expression (1 case). SALL-4 immunohistochemistry, however, was relatively sensitive (85.7%) and specific (89.5%) for SNTCS. SALL-4 expression was also identified in one poorly differentiated neuroendocrine carcinoma and one case of sinonasal undifferentiated carcinoma. SALL-4 appears to have utility in distinguishing SNTCS from other high grade sinonasal tumors.
摘要：
鼻窦畸胎癌肉瘤（SNTCS）是一种罕见的，侵袭性恶性肿瘤，表现出恶性母细胞样的异质性组合，上皮和间质成分。从真正的生殖细胞衍生到多能干细胞的起源，其确切的组织起源尚不清楚。然而,尽管这种肿瘤的多表型组织学，其中包括频繁的腺体，鳞状,和神经外胚层分化类似于附件生殖细胞肿瘤，SNTCS似乎与附件畸胎瘤有一些差异。例如,不像附件畸胎瘤,SNTCS从未被描述为混合生殖细胞肿瘤中的组分。由于SNTCS的稀有性和与其他鼻窦肿瘤的组织学重叠，因此很难准确识别SNTCS。活检更有问题，因为并非所有元素都可能存在于小样本中。SNTCS在鉴别诊断中也可以与其他肿瘤共享相似的染色模式。最近的一项研究发现核β-连环蛋白在单个TCS中表达，但这还没有在其他病例中得到证实。SALL-4是生殖细胞肿瘤的标志物,尚未检查。我们对7个SNTCS和19个其他鼻窦肿瘤的整个切片进行了β-catenin和SALL-4免疫组织化学，以评估β-catenin和SALL-4在建立SNTCS诊断中是否有用。记录每个肿瘤的表达强度和染色百分比。对于SNTCS，染色在每个组织学成分中的分布（未成熟的神经外胚层，上皮，和间充质）也有记录。在任何SNTCS中均未发现核β-连环蛋白表达，所有病例均显示膜表达（6例）或细胞质和膜表达（1例）。SALL-4免疫组织化学，然而,对SNTCS相对敏感(85.7%)和特异性(89.5%)。在1例低分化神经内分泌癌和1例鼻腔鼻窦未分化癌中也发现了SALL-4的表达。SALL-4似乎在区分SNTCS与其他高级别鼻窦肿瘤方面具有实用性。
