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先天性颅内畸胎瘤伴巨颅症及颅骨破裂。

Congenital intracranial teratoma with massive macrocephaly and skull rupture.

作者信息

Bolat Filiz, Kayaselcuk Fazilet, Tarim Ebru, Kilicdag Esra, Bal Nebil

机构信息

Department of Pathology, Başkent University Faculty of Medicine, Ankara, Turkey.

出版信息

Fetal Diagn Ther. 2008;23(1):1-4. doi: 10.1159/000109216. Epub 2007 Oct 9.

DOI:10.1159/000109216
PMID:17934288
Abstract

OBJECTIVE

Congenital intracranial tumors are rare and only account for 0.5-1.5% of all pediatric brain tumors. Teratoma is the most frequently encountered intracranial tumor at birth. Massive congenital intracranial teratoma is an extremely rare neoplasm with a poor prognosis. They grow rapidly and cause extensive destruction in the brain. Herein we report a massive intracranial teratoma causing skull rupture.

CASE REPORT

A fetus with a congenital intracranial teratoma presenting with a disproportionately enlarged head at 25 weeks of gestation is presented. Since it was the first admission of the mother to a medical expert for a prenatal examination, there was noprevious follow-up data. Prenatal ultrasonography demonstrated a huge, heterogeneous intracranial mass, and midline structures and ventricles could not be observed. No heartbeat was detected. Autopsy was perforated, and histopathologic examination of the samples taken from the intracranial mass revealed an immature teratoma.

CONCLUSION

Although congenital intracranial teratomas are rare, they may reach enormous sizes. Regular follow-up of the fetus may lead to early diagnosis of immature intracranial teratomas and prevent the mother from having further complications either due to intrauterine fetal death orpsychological trauma of giving birth to a heavily malformed baby.

摘要

目的

先天性颅内肿瘤较为罕见,仅占所有小儿脑肿瘤的0.5 - 1.5%。畸胎瘤是出生时最常遇到的颅内肿瘤。巨大先天性颅内畸胎瘤是一种极其罕见的肿瘤,预后较差。它们生长迅速,会对脑部造成广泛破坏。在此我们报告一例导致颅骨破裂的巨大颅内畸胎瘤。

病例报告

介绍一名患有先天性颅内畸胎瘤的胎儿,在妊娠25周时出现头部不成比例地增大。由于这是母亲首次因产前检查就诊于医学专家,所以没有先前的随访数据。产前超声检查显示颅内有一个巨大的、异质性肿块,无法观察到中线结构和脑室。未检测到心跳。进行了尸检,对从颅内肿块获取的样本进行组织病理学检查,结果显示为未成熟畸胎瘤。

结论

尽管先天性颅内畸胎瘤罕见,但它们可能长得非常大。对胎儿进行定期随访可能会早期诊断未成熟颅内畸胎瘤,并防止母亲因宫内胎儿死亡或生下严重畸形婴儿的心理创伤而出现进一步并发症。

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