1 Endocrinology Unit, Department of Medicine, University of Padua , Padua, Italy .
2 Radiotherapy Unit, Veneto Institute of Oncology IOV-IRCCS , Padua, Italy .
Thyroid. 2018 Jan;28(1):96-103. doi: 10.1089/thy.2017.0248. Epub 2018 Jan 2.
The hobnail variant of papillary thyroid carcinoma (HPTC) has an aggressive behavior. The aims of this prospective study were to define the clinical/molecular characteristics of HPTC, and to compare them to those of conventional papillary thyroid carcinoma (PTC).
From 2010 to 2016, 25 cases of HPTC, characterized clinically and molecularly (BRAF, RAS, TERT promoter, and TP53 mutations), were compared to a series of 165 consecutive cases of PTC. All patients underwent total thyroidectomy and received radioactive iodine treatment. Follow-up was available for 19 HPTC patients.
Among the HPTC patients, 64% had a hobnail component ≥30%, and 64% had multifocal disease. The mean tumor size was 30 mm; 96% of tumors were angio-invasive; 68% were N1, and 12% were M1; 58% harbored the BRAF mutation, 12% had a mutation in the TERT promoter, 17% had a TP53 mutation, and not had a RAS mutation. At a mean follow-up of 39 months, 32% of patients had biochemical and/or structural disease. Tumor size was the only significant difference between patients with persistent disease and those with an excellent response (40 mm and 24 mm, respectively; p = 0.02). Compared to the PTC control group, the HPTC patients had larger tumors (30 mm vs. 16 mm; p < 0.001), more frequent lymph node involvement (68% vs. 38%; p = 0.01), and remote disease (16% vs. 3%; p < 0.0001), a similar prevalence of the BRAF mutation (58% vs. 59%), a higher prevalence of TP53 mutations (17% vs. 1%; p < 0.05), and a worse outcome (structural/biochemical disease: 32% vs. 9%; p < 0.0001).
HPTC is an aggressive variant, characterized by large tumor size, lymph node involvement, a tendency to metastasize, and a worse outcome.
钉突样甲状腺乳头状癌（HPTC）具有侵袭性的行为。本前瞻性研究的目的是定义 HPTC 的临床/分子特征，并将其与传统甲状腺乳头状癌（PTC）进行比较。
从 2010 年至 2016 年，25 例 HPTC 患者在临床上和分子上具有特征（BRAF、RAS、TERT 启动子和 TP53 突变），与 165 例连续 PTC 患者进行了比较。所有患者均接受全甲状腺切除术和放射性碘治疗。19 例 HPTC 患者可获得随访。
在 HPTC 患者中，64%有≥30%钉突样成分，64%有多灶性疾病。肿瘤平均大小为 30mm；96%的肿瘤为血管侵袭性；68%为 N1，12%为 M1；58%携带 BRAF 突变，12%的 TERT 启动子发生突变，17%的 TP53 突变，没有 RAS 突变。在平均随访 39 个月时，32%的患者有生化和/或结构疾病。肿瘤大小是持续性疾病和完全缓解患者之间的唯一显著差异（40mm 和 24mm，分别；p=0.02）。与 PTC 对照组相比，HPTC 患者的肿瘤更大（30mm 比 16mm；p<0.001），淋巴结受累更频繁（68%比 38%；p=0.01），远处转移更常见（16%比 3%；p<0.0001），BRAF 突变的发生率相似（58%比 59%），TP53 突变的发生率更高（17%比 1%；p<0.05），结局更差（结构/生化疾病：32%比 9%；p<0.0001）。
HPTC 是一种侵袭性变异型，其特征为肿瘤较大、淋巴结受累、转移倾向和预后较差。
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