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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

摘要目的: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3 369 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。

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abstractsObjective: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 3 369 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.

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2024年45卷3期

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