Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, 1161 21st Avenue South, MCN CC3322, Nashville, TN, 37232-2561, USA.
Department of Otolaryngology - Head and Neck Surgery, Vanderbilt University Medical Center, Nashville, TN, USA.
Head Neck Pathol. 2022 Mar;16(1):229-235. doi: 10.1007/s12105-021-01343-3. Epub 2021 Jun 9.
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.
鼻窦胚胎性横纹肌肉瘤(SNTCS)是一种罕见的侵袭性恶性肿瘤,表现为恶性胚细胞瘤样、上皮和间叶成分的异质组合。其确切的组织发生尚不清楚,假说范围从真正的生殖细胞来源到多能干细胞起源。然而,尽管这种肿瘤具有多表型组织学,包括类似于附属生殖细胞肿瘤的频繁腺上皮、鳞状和神经外胚层分化,但 SNTCS 似乎与附属体腔瘤有一些不同。例如,与附属体腔瘤不同,SNTCS 从未被描述为混合生殖细胞瘤的组成部分。由于其罕见性和与其他鼻窦肿瘤的组织学重叠,准确识别 SNTCS 较为困难。在活检中问题更大,因为小样本中可能不存在所有成分。SNTCS 在鉴别诊断中也可能与其他肿瘤具有相似的染色模式。最近的一项研究发现单个 TCS 中存在核 β-连环蛋白表达,但这尚未在其他病例中得到证实。SALL-4,一种生殖细胞瘤的标志物,尚未被检查。我们对 7 例 SNTCS 和 19 例其他鼻窦肿瘤的全切片进行了β-连环蛋白和 SALL-4 免疫组织化学染色,以评估β-连环蛋白和 SALL-4 是否有助于建立 SNTCS 的诊断。注意了每个肿瘤的表达强度和染色百分比。对于 SNTCS,还记录了每个组织学成分(未成熟神经外胚层、上皮和间叶)内染色的分布。在任何 SNTCS 中均未发现核β-连环蛋白表达,所有病例均表现为膜表达(6 例)或细胞质和膜表达(1 例)。然而,SALL-4 免疫组织化学对 SNTCS 相对敏感(85.7%)和特异(89.5%)。SALL-4 表达也在 1 例低分化神经内分泌癌和 1 例鼻窦未分化癌中被识别。SALL-4 似乎有助于将 SNTCS 与其他高级别鼻窦肿瘤区分开来。