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伴有1p和19q缺失的脊髓少突胶质细胞瘤并表现为大脑少突胶质细胞增生症

Spinal cord oligodendroglioma with 1p and 19q deletions presenting with cerebral oligodendrogliomatosis.

作者信息

Guppy Kern H, Akins Paul T, Moes Gregory S, Prados Michael D

机构信息

Department of Neurosurgery, The Kaiser Permanente Medical Group, and University of California, San Francisco, Sacramento, California 95825-2115, USA.

出版信息

J Neurosurg Spine. 2009 Jun;10(6):557-63. doi: 10.3171/2009.2.SPINE08853.

DOI:10.3171/2009.2.SPINE08853
PMID:19558288
Abstract

Oligodendroglioma of the spinal cord is a rare tumor that most often presents with spinal cord symptoms. The authors present a case of spinal cord oligodendroglioma that was associated with cerebral rather than spinal cord symptoms. A 30-year-old woman developed nausea, vomiting, and severe headaches. Magnetic resonance imaging of the brain showed meningeal enhancement. The patient underwent a craniotomy with biopsies of the meninges and brain. The biopsy findings revealed an abnormal arachnoid thickening without tumor cells. The patient later developed hydrocephalus and underwent shunt placement. Cerebrospinal fluid cytological findings were negative for tumor cells or infection. She was found to have a cervical cord lesion at C3-4 that was initially nonenhancing but later enhanced after Gd administration. Biopsy of the cord lesion with partial resection showed a WHO Grade II oligodendroglioma with 1p and 19q deletions determined by fluorescence in situ hybridization. Neurooncological treatment with tumor radiation and temozolomide (Temodor) resulted in improvement in radiographic findings, symptoms, and long-term survival. This paper presents an extensive review of the literature, which revealed only 2 other reported cases of cerebral symptoms in adults that preceded spinal cord symptoms in a patient with oligodendroglioma of the spinal cord. It is also the first reported case of oligodendrogliomatosis due to a cervical spinal cord oligodendroglioma with 1p and 19q deletions.

摘要

脊髓少突胶质细胞瘤是一种罕见肿瘤,多数情况下表现为脊髓症状。本文作者报告了一例脊髓少突胶质细胞瘤,该病例最初表现为脑部而非脊髓症状。一名30岁女性出现恶心、呕吐及严重头痛。脑部磁共振成像显示脑膜强化。患者接受了开颅手术,对脑膜和脑进行活检。活检结果显示蛛网膜异常增厚但无肿瘤细胞。患者随后出现脑积水并接受了分流手术。脑脊液细胞学检查结果显示未发现肿瘤细胞或感染迹象。发现她在C3 - 4水平有颈髓病变,最初无强化,但给予钆剂后出现强化。对脊髓病变进行活检并部分切除,结果显示为世界卫生组织(WHO)II级少突胶质细胞瘤,通过荧光原位杂交检测发现有1p和19q缺失。采用肿瘤放疗和替莫唑胺(泰道)进行神经肿瘤学治疗后,影像学表现、症状及长期生存率均有所改善。本文对文献进行了广泛回顾,发现仅有另外2例成人少突胶质细胞瘤患者在出现脊髓症状之前先出现脑部症状的报道。这也是首例因颈髓少突胶质细胞瘤伴1p和19q缺失导致的少突胶质细胞瘤病的报道。

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Disseminated leptomeningeal tumour mimicking a subarachnoid haemorrhage.酷似蛛网膜下腔出血的播散性软脑膜肿瘤。
Neuroradiol J. 2019 Feb;32(1):53-56. doi: 10.1177/1971400918793530. Epub 2018 Aug 9.
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A very rare spinal cord tumor primary spinal oligodendroglioma: A review of sixty cases in the literature.
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