Alpha-fetoprotein levels correlate with the pathologic grade and surgical outcomes of pediatric retroperitoneal teratomas.

Alpha-fetoprotein levels correlate with the pathologic grade and surgical outcomes of pediatric retroperitoneal teratomas.
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甲胎蛋白水平与小儿腹膜后畸胎瘤的病理分级和手术结果相关。

DOI:
10.1007/s00383-009-2321-2
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发表时间:
2009
影响因子:
1.8
通讯作者:
Stein,JamesE
Stein,JamesE
中科院分区:
医学3区
文献类型:
--
作者:
Hunter,CatherineJane;Ford,HenriR;Estrada,JoaquinJ;Stein,JamesE

文献摘要

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简介腹膜后畸胎瘤 (RT) 是一种罕见的肿瘤,占所有儿科畸胎瘤的 2-5%,占所有儿科腹膜后肿瘤的 10%。尽管甲胎蛋白 (AFP) 是公认的未成熟畸胎瘤肿瘤标志物,但由于该标志物的生理性升高,其对患有 RT 的新生儿和儿童的预后价值尚不清楚。本研究的目的是确定 AFP 水平是否与儿科 RT 的病理分级和患者结果相关。方法对我们机构 20 年来所见的所有 RT 进行回顾性分析。确定了 13 名 RT 患者;他们的 AFP 水平与肿瘤分级、患者年龄和结果相关。结果 12 名患者诊断时年龄不到 1 岁。有 9 名患者的 RT 不成熟,4 名患者的 RT 成熟。所有具有成熟 RT 的患者的 AFP 水平都在正常范围内,而九个不成熟 RT 的患者中有八个 AFP 水平升高。与成熟 RT 相比,无论年龄如何,III 级 RT 的 AFP 均有统计学显着性升高 (P = 0.036)。包括血管损伤、脓毒症和死亡在内的主要并发症在高级别 RT 中更为常见。结论我们得出的结论是,AFP 水平与肿瘤不成熟度相关,即使在小于 2 个月的患者中,也可以预测手术发病率。尽管手术切除高级别 RT 会增加发病率,但完全切除可使大多数患有这种罕见儿科肿瘤的婴儿长期无病生存。
IntroductionRetroperitoneal teratomas (RTs) are rare neoplasms that comprise 2–5% of all pediatric teratomas and 10% of all pediatric retroperitoneal tumors. Although alpha-fetoprotein (AFP) is a recognized tumor marker for immature teratomas, its prognostic value in neonates and children with RTs is unknown due to physiologic elevation of this marker. The aim of this study was to determine whether AFP level correlates with pathologic grade of pediatric RT and patient outcome.MethodsA retrospective analysis of all RTs seen at our institution over a 20-year period was performed. Thirteen patients with RTs were identified; their AFP levels were correlated with tumor grade, patient age, and outcome.ResultsTwelve patients were less than 1 year of age at diagnosis. There were nine patients with immature, and four with mature RTs. All patients with mature RTs had AFP levels within the normal range, while eight out of nine patients with immature RTs had elevated AFP levels. A statistically significant elevation in AFP was noted in grade III RTs compared with mature RTs (P= 0.036), regardless of age. Major complications including vascular injury, sepsis, and death were more prevalent in high-grade RTs.ConclusionWe conclude that AFP levels correlate with tumor immaturity and predict operative morbidity even in patients less than 2 months of age. Despite the increased morbidity associated with surgical excision of high-grade RTs, complete resection results in long-term disease-free survival in the majority of infants with this rare pediatric neoplasm.