General Surgery Division, Maria Vittoria Hospital, Turin, Italy.
Pediatric Endocrinology Division, Regina Margherita Children's Hospital, Turin, Italy.
Front Endocrinol (Lausanne). 2023 Mar 3;14:1126436. doi: 10.3389/fendo.2023.1126436. eCollection 2023.
Pediatric thyroid carcinoma represents about 4-5% of all pediatric carcinoma with an incidence of 0.5 cases/100,000, compared to 2-10/100000 cases in the adult population. The aim of this study is to present the experience of a reference adult endocrine surgery unit in charge of the treatment of pediatric thyroid diseases.
From January 2019 to September 2022, 25 patients, aged 5-17, underwent thyroid surgery. We analysed indications for surgery, use of intraoperative nerve monitoring (IONM), definitive histological examination, postoperative outcomes and risk factors related.
Surgical indication was performed for Graves' disease (27%) and for nodular pathology (73%): of these, four were malignant lesions (TIR4/TIR5), eight with indeterminate characteristics (TIR3A/TIR3B) and four characterized as benign (TIR1/TIR2). Total thyroidectomy (TT) was performed in 76% of cases, three of which were prophylactic for the activation of the RET gene mutation in MEN 2A. IONM was used in eight cases (32%), all patients aged 11 years or less. FNA's accuracy was 100% for lesions typified as benign and malignant (TIR1/TIR2 and TIR4/TIR5). The overall malignancy rate achieved was 40% and in the final histological examination 75% of the TIR 3B lesions were malignant. Six patients (24%) developed hypoparathyroidism in the first postoperative day, with normalization of calcium values within thirty days in 5 patients.
Pediatric thyroid nodules are rare and distinguished from adult thyroid disease by a worse prognosis and higher malignancy rates. Our work reports a much higher malignancy rate among indeterminate TIR 3B lesions than observed in the adult population and the three patients who underwent prophylactic total thyroidectomy for activating RET gene mutation had all a definitive histological diagnosis of medullary carcinoma. Post-surgical hypoparathyroidism is a common finding in these patients: in most cases the condition is transient and it benefits from supportive therapy. Intraoperative finding of a thinner recurrent laryngeal nerve in younger patients makes nerve isolation more difficult than in adult surgery: IONM is recommended in patients under 12. Pediatric thyroid surgery is challenging, we sustain it requires referral thyroid Centers for thyroid disease with highly skilled general endocrine surgeons.
小儿甲状腺癌约占所有小儿癌的 4-5%，发病率为每 10 万人 0.5 例，而成人发病率为每 100000 人 2-10 例。本研究旨在介绍一家负责治疗小儿甲状腺疾病的成人内分泌外科参考单位的经验。
自 2019 年 1 月至 2022 年 9 月，25 名年龄在 5-17 岁的患者接受了甲状腺手术。我们分析了手术适应证、术中神经监测（IONM）的应用、确定性组织学检查、术后结果和相关的危险因素。
手术适应证为格雷夫斯病（27%）和结节性病变（73%）：其中，4 例为恶性病变（TIR4/TIR5），8 例为不确定特征（TIR3A/TIR3B），4 例为良性病变（TIR1/TIR2）。76%的病例行全甲状腺切除术（TT），其中 3 例为 MEN 2A 中 RET 基因突变激活的预防性手术。IONM 用于 8 例（32%）患者，均为 11 岁或以下的患者。FNA 对典型良性和恶性病变（TIR1/TIR2 和 TIR4/TIR5）的准确率为 100%。总的恶性率为 40%，在最终的组织学检查中，75%的 TIR 3B 病变为恶性。6 名患者（24%）在术后第 1 天出现甲状旁腺功能减退症，5 名患者在 30 天内血钙值恢复正常。
小儿甲状腺结节少见，与成人甲状腺疾病不同，预后较差，恶性率较高。我们的工作报告了 TIR 3B 不确定病变的恶性率明显高于成人人群，而 3 例因 RET 基因激活而预防性行全甲状腺切除术的患者均明确诊断为甲状腺髓样癌。这些患者术后甲状旁腺功能减退症较为常见：大多数情况下为暂时性，且受益于支持治疗。术中发现年轻患者的喉返神经较薄，使神经隔离比成人手术更为困难：建议 12 岁以下患者使用 IONM。小儿甲状腺手术具有挑战性，我们认为需要向具有高技能普通内分泌外科医生的甲状腺疾病专业转诊中心转诊。
