UW Medicine, University of Washington School of Medicine, Seattle, Washington, U.S.A.
Division of Pediatric Otolaryngology, Seattle Children's Hospital, Seattle, Washington, U.S.A.
Laryngoscope. 2022 May;132(5):1132-1138. doi: 10.1002/lary.29926. Epub 2021 Oct 29.
OBJECTIVES/HYPOTHESIS: The diffuse sclerosing variant of papillary thyroid carcinoma (DSV) may be more aggressive than conventional well-differentiated non-DSV related papillary thyroid carcinomas (N-PTC).
Retrospective chart review.
Retrospective review of clinical outcomes for patients 21 years of age or younger who underwent initial surgery for PTC at a single institution from January 1, 2005 to April 1, 2020. Genomic analysis was performed using targeted next-generation sequencing. Data were analyzed using Fischer's exact test and Kaplan-Meier curve log-rank test.
Our cohort consisted of 72 patients, nine with DSV and 63 with N-PTC. Age at diagnosis was comparable (15.4 vs. 16.2 years, respectively, P = .46). DSV were more likely to be in the high-risk American Thyroid Academy pediatric risk group (100% vs. 41.3%, P = .004), to present with regional cervical lymph node metastases (100% vs. 60.3%, P = .036), and to present with distant metastases (67% vs. 22%, P = .005). No mortality seen in either group over 27.5 (interquartile range 14.8, 46.00) months average follow-up. Throughout the follow-up period, DSV were more likely to experience progression than N-PTC (hazard ratio = 5.7 [95% confidence interval 1.7-20.0; P = .0056]). In a subset of 19 patients with aggressive disease who had molecular testing as part of clinical care we detected RET fusions in nearly all DSV compared to a minority of N-PTC (83% vs. 15.4%, P = .0095).
Pediatric patients with DSV have more advanced disease at diagnosis and are more likely to experience progression of disease compared to patients with N-PTC. The prevalence of RET fusions in our cohort recapitulates the frequency of this alteration described in prior studies.
4 Laryngoscope, 132:1132-1138, 2022.
目的/假设：弥漫性硬化型甲状腺乳头状癌（DSV）可能比传统的分化良好的非-DSV 相关甲状腺乳头状癌（N-PTC）更具侵袭性。
回顾性图表回顾。
对 2005 年 1 月 1 日至 2020 年 4 月 1 日在一家机构接受初始手术治疗 PTC 的 21 岁或以下患者的临床结果进行回顾性分析。使用靶向下一代测序进行基因组分析。使用 Fisher 精确检验和 Kaplan-Meier 曲线对数秩检验进行数据分析。
我们的队列包括 72 名患者，其中 9 名患有 DSV，63 名患有 N-PTC。诊断时的年龄相似（分别为 15.4 岁和 16.2 岁，P=0.46）。DSV 更有可能处于高风险的美国甲状腺协会儿科风险组（100%对 41.3%，P=0.004），更有可能出现局部颈部淋巴结转移（100%对 60.3%，P=0.036），并且更有可能发生远处转移（67%对 22%，P=0.005）。在平均 27.5 个月（四分位距 14.8，46.00）的随访期间，两组均未见死亡。在整个随访期间，DSV 比 N-PTC 更有可能发生进展（风险比=5.7[95%置信区间 1.7-20.0；P=0.0056]）。在一组 19 名患有侵袭性疾病的患者中，作为临床护理的一部分进行了分子检测，我们在几乎所有的 DSV 中检测到了 RET 融合，而在少数 N-PTC 中则检测到了 RET 融合（83%对 15.4%，P=0.0095）。
与 N-PTC 相比，患有 DSV 的儿科患者在诊断时具有更晚期的疾病，并且更有可能经历疾病的进展。我们队列中 RET 融合的患病率与先前研究中描述的这种改变的频率相符。
4 Laryngoscope, 132:1132-1138, 2022.
