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儿童小脑桥脑角髓母细胞瘤伴广泛结节形成:病例报告及文献复习

Cerebellopontine angle medulloblastoma with extensive nodularity in a child: case report and review of the literature.

作者信息

Noiphithak Raywat, Yindeedej Vich, Thamwongskul Chatchai

机构信息

Division of Neurosurgery, Department of Surgery, Thammasat University Hospital, Faculty of Medicine, Thammasat University, 95 Klongneung, Khlong Luang, Pathum Thani, 12120, Thailand.

Department of Pathology and Forensic Medicine, Faculty of Medicine, Thammasat University, Khlong Luang, Pathum Thani, Thailand.

出版信息

Childs Nerv Syst. 2017 May;33(5):839-842. doi: 10.1007/s00381-016-3325-6. Epub 2016 Dec 24.

Abstract

BACKGROUND

Cerebellar medulloblastomas (MBs) are one of the most common posterior fossa tumors in children but rarely occur in the cerebellopontine angle (CPA). Only 39 cases of CPA MBs were reported in the literature, and most of them were classic and desmoplastic MBs.

CASE PRESENTATION

A 22-month-old girl presented with progressive cerebellar ataxia. Magnetic resonance imaging showed a large tumor in the right CPA and obstructive hydrocephalus. Surgical resection was performed and achieved total tumor removal. Microscopic examination and immunohistochemical staining revealed the diagnosis of MB with extensive nodularity. The patient recovered from her symptoms during follow-up and was transferred for adjuvant chemotherapy.

CONCLUSION

MB should be considered as a differential diagnosis of a lesion in the CPA. The treatment and outcome of CPA MBs are similar to cerebellar MBs.

摘要

背景

小脑髓母细胞瘤(MBs)是儿童最常见的后颅窝肿瘤之一,但很少发生于桥小脑角(CPA)。文献中仅报道了39例CPA区MBs,其中大多数为经典型和促纤维增生型MBs。

病例介绍

一名22个月大的女孩出现进行性小脑共济失调。磁共振成像显示右侧CPA区有一个大肿瘤及梗阻性脑积水。进行了手术切除,实现了肿瘤全切。显微镜检查和免疫组化染色显示诊断为广泛结节型MB。患者在随访期间症状恢复,并转至辅助化疗。

结论

MB应被视为CPA区病变的鉴别诊断之一。CPA区MBs的治疗和结果与小脑MBs相似。

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