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伴有少突胶质细胞瘤和原始神经外胚层肿瘤样成分的胶质母细胞瘤病例,生存9年

Glioblastoma with Both Oligodendroglioma and Primitive Neuroectodermal Tumor-Like Components in a Case with 9-Year Survival.

作者信息

Chen Ying-Tso, Hsu Shu-Shong, Yip Chi-Man, Lai Ping-Hong, Lee Huai-Pao

机构信息

Division of Neurosurgery, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

出版信息

Case Rep Surg. 2018 Jun 11;2018:1382680. doi: 10.1155/2018/1382680. eCollection 2018.

Abstract

INTRODUCTION

Glioblastoma multiforme (GBM), the most common primary malignant brain tumor in adults, is characterized by extensive heterogeneity in its clinicopathological presentation. A primary brain tumor with both astrocytic differentiation and neuronal immunophenotype features is rare. Here, we report a long-term survival patient who presented this rare form of GBM in the disease course.

PRESENTATION OF CASE

A 23-year-old woman, presenting with rapidly progressive headache and right-side weakness, was diagnosed with brain tumor over the left basal ganglion. She underwent the first craniectomy for tumor removal, and histopathology revealed classic GBM. Tumor recurrence occurred 8 years later. Another gross total resection was performed and pathology revealed GBM with the oligodendroglioma component (GBM-O). Due to disease progression, she received debulking surgery the following year. The third pathology revealed glioblastoma with primitive neuroectodermal tumor-like component (GBM-PNET).

DISCUSSION

GBM-PNETs are collision tumors with both neuronal and glial components. They are rare, and a few case reports have suggested that these tumors are associated with favorable outcomes but a higher risk of cerebrospinal fluid dissemination.

CONCLUSION

We report a patient who developed the distinct pathologic variants of classic GBM, GBM-O, and GBM-PNET, throughout the disease course. Young age, aggressive surgical resection, and pathologic and genetic features may have contributed to the long-term survival of the patient.

摘要

引言

多形性胶质母细胞瘤(GBM)是成人中最常见的原发性恶性脑肿瘤,其临床病理表现具有广泛的异质性。一种同时具有星形细胞分化和神经元免疫表型特征的原发性脑肿瘤较为罕见。在此,我们报告一名在病程中出现这种罕见GBM形式的长期存活患者。

病例介绍

一名23岁女性,因快速进展性头痛和右侧肢体无力就诊,被诊断为左侧基底节区脑肿瘤。她接受了首次开颅肿瘤切除术,组织病理学显示为经典GBM。8年后肿瘤复发。再次进行了全切除手术,病理显示为伴有少突胶质细胞瘤成分的GBM(GBM-O)。由于疾病进展,次年她接受了减瘤手术。第三次病理显示为伴有原始神经外胚层肿瘤样成分的胶质母细胞瘤(GBM-PNET)。

讨论

GBM-PNET是同时具有神经元和胶质成分的碰撞瘤。它们很罕见,少数病例报告表明这些肿瘤与较好的预后相关,但脑脊液播散风险较高。

结论

我们报告了一名在整个病程中出现经典GBM、GBM-O和GBM-PNET不同病理变体的患者。年轻、积极的手术切除以及病理和基因特征可能是该患者长期存活的原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0063/6016224/4a089520b7a8/CRIS2018-1382680.001.jpg

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