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桥小脑角原始神经外胚层肿瘤酷似三叉神经鞘瘤。

Cerebellopontine angle primitive neuroectodermal tumor mimicking trigeminal schwannoma.

作者信息

Khan Saad Akhtar, Ujjan Badar Uddin, Salim Adnan, Shamim Shahzad

机构信息

Department of Neurosurgery, Aga Khan University Hospital, Karachi, Pakistan.

Dow Medical College, Dow University of Health Sciences, Karachi, Pakistan.

出版信息

Surg Neurol Int. 2016 Jan 20;7:7. doi: 10.4103/2152-7806.174599. eCollection 2016.

Abstract

BACKGROUND

Primitive neuroectodermal tumors (PNETs) comprise a group of aggressive, poorly differentiated embryonal tumors occurring in central nervous system as well as in peripheral locations. Primary cerebellopontine angle (CPA) PNET is an extremely rare entity. It is important to have knowledge of this pathology and to be able to differentiate it from other commonly occurring CPA tumors, such as vestibular and trigeminal schwannomas. This distinction is essential because of the difference in the overall treatment plan and prognosis.

CASE DESCRIPTION

This report describes a case of a young male presenting with diplopia and numbness of face; magnetic resonance imaging showed a CPA mass. With a provisional diagnosis of trigeminal schwannoma, the patient underwent surgery. Histopathology provided a diagnosis of PNET.

CONCLUSION

We discuss the importance of recognizing this rare condition and how this entity differs from the commonly occurring tumors.

摘要

背景

原始神经外胚层肿瘤(PNETs)是一组侵袭性强、分化差的胚胎性肿瘤,可发生于中枢神经系统以及外周部位。原发性小脑桥脑角(CPA)PNET极为罕见。了解这种病理情况并能够将其与其他常见的CPA肿瘤(如前庭和三叉神经鞘瘤)区分开来非常重要。由于整体治疗方案和预后存在差异,这种区分至关重要。

病例描述

本报告描述了一名年轻男性出现复视和面部麻木的病例;磁共振成像显示CPA有肿块。初步诊断为三叉神经鞘瘤,患者接受了手术。组织病理学诊断为PNET。

结论

我们讨论了认识这种罕见疾病的重要性以及该实体与常见肿瘤的不同之处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e4a7/4743274/d8f028635af2/SNI-7-7-g001.jpg

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