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[幕上原始神经外胚层肿瘤:1例手术病例报告]

[Supratentorial primitive neuroectodermal tumor: report of a surgical case].

作者信息

Omae Tomoya, Takahashi Masataka, Sasajima Toshio, Sugawara Taku, Kinouchi Hiroyuki, Higashiyama Naoki, Mizoi Kazuo

机构信息

Department of Neurosurgery, Akita University School of Medicine, Akita-city, Japan.

出版信息

No Shinkei Geka. 2004 Jun;32(6):619-25.

Abstract

We reported a rare case of supratentorial primitive neuroectodermal tumors (sPNET) and reviewed the literature. A 15-year-old girl presented with a one-month history of headache and vomiting. CT scans showed a huge, solid and cystic tumor with calcification, occupying the left anterior cranial fossa. The solid portion of the tumor was hypointense on T1-weighted images, slightly hyperintense on T2-weighted images, hyperintense on diffusion- weighted images, isointense on fluid-attenuated inversion recovery (FLAIR) images, and strongly enhanced after administration of contrast medium. The expansile tumor had a broad attachment to the dura matter of the anterior cranial fossa. The patient underwent an uneventful extirpation of the tumor. Microscopically, the solid tumor contained small, round poorly-differentiated cells with pleomorphic nuclei and brisk mitotic activity. The tumor cells were immunoreactive for synaptophysin and GFAP, whereas lack of MIC2 gene product expression was confirmed using the monoclonal antibody 12E7. The MIB-1-positive rate was 43%. The histological diagnosis was sPNET. Postoperatively, a dose of 30.6Gy was administrated to the whole brain and a boost of 19.6Gy to the T2-hyperintensity lesion. The patient successively received a dose of 30.4Gy to the whole spine and Linac stereotactic radiotherapy with a marginal dose of 16.8Gy at the tumor bed. Three months after radiotherapy the patient received chemotherapy using carboplatin and etoposide. Follow-up MR images showed no evidence of recurrent tumor 5 months after the radiochemotherapy. On the basis of MR findings on both diffusion-weighted and FLAIR images, preoperative diagnosis of sPNET may be important for choosing appropriate therapeutic strategies for this tumor.

摘要

我们报告了一例罕见的幕上原始神经外胚层肿瘤(sPNET)并复习了相关文献。一名15岁女孩有1个月的头痛和呕吐病史。CT扫描显示一个巨大的实性和囊性肿瘤伴钙化,占据左前颅窝。肿瘤的实性部分在T1加权图像上呈低信号,在T2加权图像上略呈高信号,在扩散加权图像上呈高信号,在液体衰减反转恢复(FLAIR)图像上呈等信号,注射造影剂后明显强化。肿瘤呈膨胀性生长,与前颅窝硬脑膜广泛相连。患者肿瘤切除过程顺利。显微镜下,实性肿瘤包含小的、圆形的低分化细胞,细胞核多形性,有活跃的有丝分裂活性。肿瘤细胞对突触素和GFAP免疫反应阳性,而使用单克隆抗体12E7证实缺乏MIC2基因产物表达。MIB-1阳性率为43%。组织学诊断为sPNET。术后,全脑给予30.6Gy剂量照射,T2高信号病变给予19.6Gy的追加剂量照射。患者随后接受全脊柱30.4Gy剂量照射及直线加速器立体定向放射治疗,肿瘤床边缘剂量为16.8Gy。放疗3个月后患者接受卡铂和依托泊苷化疗。放化疗后5个月的随访MR图像显示无肿瘤复发迹象。基于扩散加权和FLAIR图像的MR表现,sPNET的术前诊断对于选择该肿瘤合适的治疗策略可能很重要。

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