Departments of Pathology.
Mount Sinai Health System, New York, NY.
Am J Surg Pathol. 2022 Dec 1;46(12):1659-1669. doi: 10.1097/PAS.0000000000001958. Epub 2022 Aug 29.
Pediatric thyroid carcinomas (TCs) are rare and mainly approached based on data extrapolated from adults. We retrospectively reviewed 222 pediatric TCs (patient age less than or equal to 21 y). Lymph node (LN) disease volume at presentation was considered high if the largest positive LN measured ≥1 cm and/or >5 LNs were positive. High-grade follicular cell-derived thyroid carcinoma (HGFCTC) were defined by the presence of marked mitotic count and/or tumor necrosis and considered as high-risk histology along with papillary thyroid carcinomas (PTC) diffuse sclerosing variant (DSV). Disease-free survival (DFS) was analyzed. LN involvement at presentation was significantly associated with male sex, larger tumor size, lymphatic invasion, positive surgical margins, and distant metastases at presentation. Five- and 10-year DFS was 84% and 77%, respectively. Only 1 patient with HGFCTC died of disease. Within PTC variants, PTC-DSV was associated with adverse histopathologic parameters and higher regional disease spread, unlike PTC tall cell variant which did not portend worse behavior. The presence of necrosis conferred worse DFS ( P =0.006), while increased mitotic activity did not. While the entire HGFCTC group did not correlate with outcome ( P =0.071), HGFCTC with necrosis imparted worse DFS ( P =0.006). When restricted to PTC-DSV and HGFCTC with necrosis, high-risk histologic classification emerged as an independent prognostic parameter of DFS ( P =0.020). The excellent prognosis of pediatric TCs differs from that of adult TCs showing similar histologic features. While neither increased mitotic activity nor PTC tall cell variant histology predict adverse outcome, PTC-DSV and tumors with necrosis constitute high-risk histologic variants with an increased risk of protracted disease.
儿童甲状腺癌（TC）较为罕见，主要基于成人数据进行推断。我们回顾性分析了 222 例儿童 TC（患者年龄≤21 岁）。如果最大阳性 LN 测量值≥1cm 和/或>5 个 LN 阳性，则认为淋巴结（LN）疾病体积大。高分化滤泡细胞来源甲状腺癌（HGFCTC）的定义为存在明显的有丝分裂计数和/或肿瘤坏死，并与甲状腺乳头状癌（PTC）弥漫硬化型（DSV）一起被认为是高危组织学类型。分析无病生存（DFS）。初诊时 LN 受累与男性、肿瘤较大、淋巴血管侵犯、阳性手术切缘和远处转移有关。5 年和 10 年 DFS 分别为 84%和 77%。仅有 1 例 HGFCTC 患者死于疾病。在 PTC 变异型中，PTC-DSV 与不良组织病理学参数和更高的区域疾病扩散相关，与 PTC 高细胞变异型不同，后者的行为并不差。坏死的存在预示着更差的 DFS（P=0.006），而有丝分裂活性的增加则没有。虽然整个 HGFCTC 组与预后无关（P=0.071），但伴有坏死的 HGFCTC 预示着更差的 DFS（P=0.006）。当仅限于 PTC-DSV 和伴有坏死的 HGFCTC 时，高危组织学分类成为 DFS 的独立预后参数（P=0.020）。儿童 TC 的良好预后与具有相似组织学特征的成人 TC 不同。虽然有丝分裂活性增加或 PTC 高细胞变异型组织学均不能预测不良结局，但 PTC-DSV 和伴有坏死的肿瘤构成了高危组织学变异型，具有延长疾病的风险。
