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成人心室神经节母细胞瘤伴放疗成功治疗。

Ventricular ganglioneuroblastoma in an adult and successful treatment with radiotherapy.

机构信息

Department of Radiation Oncology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.

Department of Pathology, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.

出版信息

Balkan Med J. 2014 Jun;31(2):173-6. doi: 10.5152/balkanmedj.2014.13155. Epub 2014 Jun 1.

Abstract

BACKGROUND

Ganglioneuroblastomas (GNBs) are rare embryonic neoplasms in the spectrum of neuroblastic tumours and 80% of cases occur in the first decade. In adults, GNBs are usually located in the retroperitoneum, mediastinum and neck but intracranial GNBs are extremely rare.

CASE REPORT

A 34-year-old male applied to the Department of Neurology outpatient clinic with a two month history of headache and numbness in his legs. Detailed examinations and cranial CT were performed and showed a mass with a 5 cm diameter running from the third to the fourth ventricle. Referral to a neurosurgeon was performed for partial removal of the tumour, as the histological and immunohistochemical studies defined the diagnosis of GNB. Three months later, when the patient experienced dizziness, an MRI was performed, which showed a 4×3 cm ventricular mass, with hypointense characterisation in T1-weighted and hyperintense characteristics in T2-weighted and flair sequences. Afterwards, fractioned radiotherapy (54 Gy/30 fx) was chosen as the appropriate therapy. In the follow-up period, MRI was performed 3 months and 1 year after treatment, and revealed shrinkage of the tumour by at least 50%. Meanwhile the patient's post-irradiation course was favourable.

CONCLUSION

Data following the use of radiotherapy as treatment for intracranial GNB showing favourable results has been reported.

摘要

背景

神经节神经母细胞瘤(GNB)是神经母细胞瘤谱系中罕见的胚胎性肿瘤,80%的病例发生在第一个十年。在成人中,GNB 通常位于腹膜后、纵隔和颈部,但颅内 GNB 极为罕见。

病例报告

一名 34 岁男性因头痛和腿部麻木到神经内科门诊就诊,病史为两个月。进行了详细检查和头颅 CT,显示直径为 5 厘米的肿块从第三脑室延伸至第四脑室。由于组织学和免疫组织化学研究定义了 GNB 的诊断,因此转诊给神经外科医生进行部分肿瘤切除。三个月后,当患者出现头晕时,进行了 MRI 检查,显示脑室有 4×3 厘米的肿块,在 T1 加权像呈低信号,在 T2 加权像和 flair 序列呈高信号。随后,选择分次放疗(54 Gy/30 fx)作为合适的治疗方法。在随访期间,在治疗后 3 个月和 1 年进行了 MRI 检查,显示肿瘤至少缩小了 50%。同时,患者的放疗后过程良好。

结论

已有报道使用放疗治疗颅内 GNB 取得良好效果的数据。

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