New York University Grossman School of Medicine, NY.
Department of Otolaryngology-Head and Neck Surgery, New York University Grossman School of Medicine, NY.
Surgery. 2021 Sep;170(3):736-742. doi: 10.1016/j.surg.2021.03.001. Epub 2021 Apr 8.
Unlike medullary thyroid carcinoma in adults, the vast majority of pediatric medullary thyroid carcinoma is hereditary. Pediatric medullary thyroid carcinoma is known to have different genetic alterations driving tumorigenesis, but it is not known if pediatric medullary thyroid carcinoma has different clinicopathologic features. This study aims to identify which pediatric medullary thyroid carcinoma patients might warrant elective neck dissection.
We selected all patients ages 0 to 19 diagnosed with clinically evident medullary thyroid carcinoma in the National Cancer Database between 2004 to 2016. Clinicopathologic factors, treatments, and outcomes were analyzed and compared between this cohort and adults (ages ≥20) with medullary thyroid carcinoma.
One hundred twenty-five pediatric medullary thyroid carcinoma (median age: 13) and 5,086 adult medullary thyroid carcinoma (median age: 57) patients were identified. Pediatric patients had smaller tumors (median diameter: 1.2 cm vs 2.0 cm; P < .001), lower rates of nodal metastases (n = 31, 36.9% vs 1,689, 50.4%; P = .02) but double the incidence of multifocal tumors (n = 70, 59.3%, vs 1,412, 29.9%; P < .001) compared with adults. Multifocal tumors conferred a significantly increased risk of nodal metastases in adult medullary thyroid carcinoma (64.4% vs 43.2%; P < .001) but not pediatric medullary thyroid carcinoma (37.7% vs 35.7%; P = .85). Nodal metastases were more frequent among older children (0-5 years: 0.0%, 6-12: 40.7%, 13-19: 41.7%; P = .04). However, rates of occult nodal metastases were similar between older children (6-19 years: n = 12, 21.4%) and adults (557, 25.8% P = .56).
Pediatric medullary thyroid carcinoma has lower rates of lymph node metastases compared with adults. The risk of nodal disease was low among the youngest children, but older children ages 6 to 19 were at considerable risk for occult metastases. These findings could guide clinicians in selecting pediatric patients considered for elective lymph node dissection.
与成人的髓样甲状腺癌不同，绝大多数儿童髓样甲状腺癌是遗传性的。已知儿童髓样甲状腺癌有不同的遗传改变驱动肿瘤发生，但尚不清楚儿童髓样甲状腺癌是否具有不同的临床病理特征。本研究旨在确定哪些儿童髓样甲状腺癌患者需要进行选择性颈清扫术。
我们从 2004 年至 2016 年国家癌症数据库中选择了所有诊断为临床明显髓样甲状腺癌的 0 至 19 岁患者。分析比较了该队列和成人（年龄≥20 岁）髓样甲状腺癌患者的临床病理因素、治疗方法和结果。
共确定了 125 例儿童髓样甲状腺癌（中位年龄：13 岁）和 5086 例成人髓样甲状腺癌（中位年龄：57 岁）患者。与成人相比，儿童患者肿瘤较小（中位直径：1.2 厘米 vs 2.0 厘米；P<.001），淋巴结转移率较低（n=31，36.9% vs 1689，50.4%；P=.02），但多灶性肿瘤的发生率增加一倍（n=70，59.3% vs 1412，29.9%；P<.001）。多灶性肿瘤在成人髓样甲状腺癌中显著增加了淋巴结转移的风险（64.4% vs 43.2%；P<.001），但在儿童髓样甲状腺癌中则不然（37.7% vs 35.7%；P=.85）。较大的儿童（0-5 岁：0.0%，6-12 岁：40.7%，13-19 岁：41.7%；P=.04）中淋巴结转移更为常见。然而，较大儿童（6-19 岁：n=12，21.4%）和成人（557，25.8%）中隐匿性淋巴结转移的发生率相似（P=.56）。
与成人相比，儿童髓样甲状腺癌的淋巴结转移率较低。在最小的儿童中，发生淋巴结疾病的风险较低，但 6 至 19 岁的较大儿童存在隐匿性转移的风险较大。这些发现可以指导临床医生选择需要进行选择性淋巴结清扫术的儿科患者。
