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[1例与唐氏综合征相关的基底节内胚窦瘤]

[A case of endodermal sinus tumor in the basal ganglia associated with Down's syndrome].

作者信息

Oshita N, Yamashita K, Gotou K, Nagata I, Ueda H, Mitani T

机构信息

Department of Neurosurgery, Yamaguchi University School of Medicine.

出版信息

No Shinkei Geka. 1993 Apr;21(4):345-9.

PMID:8474590
Abstract

A case of Down's syndrome accompanied by endodermal sinus tumor of the left basal ganglia is reported. She was referred to us because of right hemiparesis. A CT scan showed a calcified mass in the left basal ganglia, which was well enhanced on contrast CT. Emergency removal of the tumor was performed because of its rapid growth, intratumoral hemorrhage, and acute hydrocephalus. The histological diagnosis established after surgery was endodermal sinus tumor. Alphafetoprotein-positive cells were seen in the resected tumor by light microscopy. Postoperative irradiation was very effective, and the high level of serum alphafetoprotein decreased during the course of irradiation. The residual tumor, enhanced on contrast CT, was treated by administration of cisplatin and etoposide after irradiation. Cases of malignant tumors other than leukemia, which are associated with Down's syndrome, are extremely rate. To our knowledge, including three cases of germ cell tumor, there have been only nine cases of brain tumors associated with Down's syndrome reported previously.

摘要

报告了一例伴有左基底神经节内胚窦瘤的唐氏综合征病例。她因右侧偏瘫被转诊至我院。CT扫描显示左基底神经节有一钙化肿块,增强CT扫描显示其强化明显。由于肿瘤生长迅速、瘤内出血和急性脑积水,遂进行了急诊肿瘤切除。术后组织学诊断为内胚窦瘤。光镜下在切除的肿瘤中可见甲胎蛋白阳性细胞。术后放疗非常有效,放疗过程中血清甲胎蛋白水平降低。放疗后,对增强CT显示的残留肿瘤给予顺铂和依托泊苷治疗。与唐氏综合征相关的除白血病外的恶性肿瘤病例极为罕见。据我们所知,包括3例生殖细胞瘤在内,此前仅报道过9例与唐氏综合征相关的脑肿瘤病例。

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