Institute of Pathology, University Hospital Essen, University of Duisburg-Essen, Hufelandstr, 55, 45147, Essen, Germany.
Institute of Nuclear Medicine, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.
Endocr Pathol. 2020 Jun;31(2):101-107. doi: 10.1007/s12022-020-09608-3.
Strumal carcinoid is an extraordinary rare tumor of the ovary consisting of thyroid tissue intermixed with neuroendocrine tumor component. The cellular origin of strumal carcinoids has been an area of debate. There is also little data on detailed immunohistochemical and molecular characteristics of these neoplasms. For this reason, this series investigated the characteristics of a series of 13 strumal carcinoids using immunohistochemical markers and a 47-gene next-generation sequencing (NGS) solid tumor panel analysis. Both cellular components showed thyroglobulin expression in all tumors. TTF-1 expression was noted in both cellular components of 11 cases. Chromogranin A was positive in both components of most tumors (n = 12, 92.3% in the neuroendocrine component and n = 10, 76.9% in the thyroid follicular component). Synaptophysin stained the neuroendocrine component of all cases, and it was also identified in the follicular thyroid component of a single case. All tumors were negative for CDX2 and calcitonin. ISLET1 was positive in the neuroendocrine component of 8 cases (6.5%). With the exception of one case, all tumors were positive for SSTR2a. The tumors were associated with a low Ki67 labeling index. All cases were microsatellite stable and no pathogenic mutations were identified using a 47-gene NGS solid tumor analysis. This series underscored that strumal carcinoids are distinct neuroendocrine tumors. The synchronous expression for thyroid follicular epithelial and neuroendocrine differentiation biomarkers may suggest a precursor cell origin displaying mixed-amphicrine differentiation. While strumal carcinoids can be diagnosed by their typical morphology and immunohistochemical profile, frequent SSTR expression may serve as a potential theranostic biomarker in the management of affected patients. In addition, the absence of common driver mutations in the NGS solid tumor panel may suggest that these neoplasms seem to be genetically unrelated to follicular epithelial-derived thyroid tumors and potentially different than other commonly identified well-differentiated neuroendocrine neoplasms. Therefore, further studies focusing on molecular characteristics of this entity are still needed.
甲状腺滤泡性腺癌是一种罕见的卵巢肿瘤，由甲状腺组织和神经内分泌肿瘤成分混合而成。甲状腺滤泡性腺癌的细胞起源一直存在争议。关于这些肿瘤的详细免疫组织化学和分子特征的数据也很少。因此，本系列研究使用免疫组织化学标志物和 47 基因下一代测序 (NGS) 固体肿瘤面板分析对 13 例甲状腺滤泡性腺癌的特征进行了研究。所有肿瘤的两种细胞成分均表达甲状腺球蛋白。11 例肿瘤的两种细胞成分均表达 TTF-1。大多数肿瘤的神经内分泌成分（n=12，92.3%）和甲状腺滤泡成分（n=10，76.9%）的铬粒蛋白 A 均为阳性。突触素染色所有病例的神经内分泌成分，并且在单个病例的甲状腺滤泡成分中也发现了它。所有肿瘤均为 CDX2 和降钙素阴性。ISLET1 在 8 例（6.5%）神经内分泌成分中为阳性。除 1 例外，所有肿瘤均对 SSTR2a 呈阳性。肿瘤与低 Ki67 标记指数相关。所有病例均为微卫星稳定，并且使用 47 基因 NGS 固体肿瘤分析未鉴定出致病性突变。本系列强调甲状腺滤泡性腺癌是一种独特的神经内分泌肿瘤。甲状腺滤泡上皮和神经内分泌分化生物标志物的同步表达可能表明存在混合全能分化的前体细胞起源。虽然甲状腺滤泡性腺癌可以通过其典型的形态和免疫组织化学特征进行诊断，但频繁的 SSTR 表达可能成为影响患者管理的潜在治疗生物标志物。此外，NGS 固体肿瘤面板中没有常见的驱动突变表明这些肿瘤在遗传上与滤泡上皮来源的甲状腺肿瘤无关，并且与其他常见的分化良好的神经内分泌肿瘤不同。因此，仍需要进一步研究该实体的分子特征。
