Liver tumors in neonates and very young infants: diagnostic pitfalls and therapeutic problems.
Liver tumors in neonates and very young infants: diagnostic pitfalls and therapeutic problems.
复制标题
新生儿和幼儿的肝脏肿瘤：诊断陷阱和治疗问题。
DOI：
10.1055/s-2008-1066170
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发表时间：
1995
期刊：
影响因子：
--
通讯作者：
H. Mildenberger
中科院分区：
文献类型：
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作者：
D. V. von Schweinitz;S. Glüer;H. Mildenberger
我们报告了 26 名患有各种肝脏肿瘤的 3 个月以下婴儿，这些婴儿自 1977 年以来在我们医院接受治疗和/或在德国儿童肝脏肿瘤合作研究 HB-89 中注册。其中 17 例患有婴儿血管内皮瘤 (inf HE)，7 例患有肝母细胞瘤 (HB)，1 例患有间质错构瘤 (mes H)，1 例患有神经母细胞瘤 IV-S 期 (Nbl)。 5 例 inf HE 病例出现羊水过多，其中 1 例与胎儿水肿相关。这导致4名婴儿早产。 7 名患有大 inf HE 的婴儿患有高输出量充血性心功能不全，另外 2 名婴儿还患有卡萨巴赫-梅里特综合征。除一名 HB 患者外，所有婴儿的血清甲胎蛋白均在正常年龄范围内。 Nbl 患者的神经元特异性烯醇化酶明显升高，1 名 inf HE 患者和 3 名 HB 患者的神经元特异性烯醇化酶略高于正常范围。其他肿瘤标志物和血小板计数并不代表诊断。此外，超声检查、CT 和血管造影等成像技术在区分肿瘤方面并不总是可靠。在 2 例病例中，最初的 HB 组织学诊断必须纠正为 inf HE。在 6 名儿童中，肿瘤最初是在错误的假设下进行治疗的：4 个肿瘤作为 HB 而不是 inf HE，1 个肿瘤作为 inf HE 而不是 Nbl IV-S，1 个肿瘤作为肝脏局灶性结节性增生而不是 HB。 7 例未切除的 inf HE（Dehner I 型）在 6 个月至 3 年后表现出自发消退。 1 例 inf II 型 HE 切除后复发两次。（摘要截短 250 字）
We report on 26 infants under 3 months of age with various liver tumors, who were treated in our hospital since 1977 and/or registered in the German Cooperative Pediatric Liver Tumor Study HB-89. 17 of these had an infantile hemangioendothelioma (inf HE), 7 a hepatoblastoma (HB), one a mesenchymal hamartoma (mes H) and one a neuroblastoma stage IV-S (Nbl). Polyhydramnios occurred in 5 cases of inf HE, in one associated with hydrops fetalis. This led to a preterm delivery of 4 infants. 7 infants with a large inf HE suffered from high output congestive heart insufficiency, 2 additionally from a Kasabach-Merritt-syndrome. Serum alpha-fetoprotein was within normal range of age in all infants except in one HB patient. Neuron-specific enolase was clearly elevated in the Nbl patient and slightly above normal range in one inf HE and 3 HB patients. Other tumor markers and platelet counts were not indicative of diagnosis. Also imaging techniques as ultrasonography, CT and angiography were not always reliable in differentiating the tumors. In 2 cases the initial histological diagnosis of an HB had to be corrected for an inf HE. In 6 children the tumors were initially treated under a wrong assumption: 4 tumors as an HB instead of an inf HE, one as an inf HE instead of a Nbl IV-S and one as a focal nodular hyperplasia of the liver instead of an HB. 7 not resected inf HEs (Dehner type I) showed spontaneous regression after 6 months to 3 years. One inf HE type II relapsed twice after resection.(ABSTRACT TRUNCATED AT 250 WORDS)