新生婴儿骶尾部畸胎瘤的诊治和预后——单中心104例分析

Diagnoses,managements and outcomes of sacrococcygeal teratoma in neonates:a report of 104 cases at a single center

目的分析总结新生婴儿骶尾部畸胎瘤诊治情况与长期预后。方法收集上海交通大学医学院附属新华医院小儿外科2000年9月至2021年9月间收治的104例年龄<3月龄的新生婴儿骶尾部畸胎瘤患儿的临床资料。男37例,女67例;平均孕期38.96周,平均出生体重3369 g。均在收住院后尽早行肿瘤切除及尾骨切除术,同时对肿瘤及其边缘进行病理活检。出院后门诊随访每1次,观察肿瘤标志物甲胎蛋白等指标及影像学复检,检查患儿生长发育、智力、大小便情况等。分类变量资料采用χ2检验。结果104例中,良性成熟型96例(92.31%),恶性8例(7.69%),包括2例卵黄囊瘤,均无转移发生。平均瘤体直径6.93 cm。按Altman分型,Ⅰ~Ⅱ型占82.69%(86/104)。产前诊断率32.69%(34/104),以孕15~39周产前筛查检出。58例合伴其他畸形,心血管畸形占91.38%(53/58),其次为泌尿系畸形8.62%(5/58)。患儿平均住院天数24.18 d。平均随访7.84年,随访率73.08%(76/104),无死亡病例,复发1例。智力差6.58%(5/76),排便异常21.05%(16/76),排尿异常2.63%(2/76)。结论新生婴儿骶尾部畸胎瘤以良性为主,部分合伴畸形,早期诊断特别是产前诊断对治疗方案、评估预后有利。手术治疗长期随访预后好,复发少。

Objective To explore the diagnosis,treatment and long-term prognosis of sacrococcygeal teratoma in neonates.Methods The relevant clinical data were retrospectively reviewed for 104 neonates of sacrococcygeal teratoma aged under 3 months from September 2000 to September 2021.There were 37 boys and 67 girls with an average gestational age of 38.96 weeks and an average birth weight of 3369 gram.Tumor and coccyx resections were performed with a pathological biopsy of tumor and its margin.After discharge,outpatient follow-ups were performed once every 3-6 months.Tumor marker(e.g.alpha-fetoprotein)and imaging re-examination were observed along with growth&development,intelligence,bowel and bladder function.Categorical data were processed by Chi-square test.Results Clinical types were benign mature(n=96,92.31%)and malignant(n=8,7.69%).The latter included yolk sac tumor(n=2)and none of them had metastasis.Average tumor diameter was 6.93 cm.According to the scheme of Altman classification,type Ⅰ-Ⅱ accounted for 82.69%(86/104).Prenatal diagnosis rate was 32.69%(34/104)and it was detected by prenatal screening at 15-39 weeks of gestation.And 58 cases were accompanied by other malformations,including cardiovascular malformations(91.38%,53/58)and urinary system malformations(8.62%,5/58).Average hospitalization stay was 24.18 days.Average follow-up time was 7.84 years with a follow-up rate of 73.08%(76/104).Without any mortality,there was only one recurrence.The outcomes were poor intelligence(6.58%,5/76),bowel dysfunction(21.05%,16/76)and urinary dysfunction(2.63%,2/76).Conclusion Sacrococcygeal teratoma is mostly benign in neonates.Some cases are accompanied by malformations.Early prenatal diagnosis is beneficial for treatment planning and prognostic evaluations.Surgery has decent long-term outcomes with a low recurrence rate.