Glioneuronal tumor with neuropil-like islands of the spinal cord with diffuse leptomeningeal neuraxis dissemination
Glioneuronal tumor with neuropil-like islands of the spinal cord with diffuse leptomeningeal neuraxis dissemination
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DOI：
10.1007/s11060-010-0505-1
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发表时间：
2011-09-01
影响因子：
3.9
通讯作者：
Scheithauer, Bernd W.
中科院分区：
文献类型：
--
作者：
Ruppert, Bree;Welsh, Cynthia T.;Scheithauer, Bernd W.
1例54岁白人女性，有1年左腿间歇性麻木病史，进展为双侧下肢感觉丧失，进一步包括振动和本体感觉受损。随后的胸椎磁共振成像（MRI）扫描显示了一个不均匀的，强烈增强的，位于中央的脊髓肿块，涉及T7到T10，伴随着前后脊髓表面向上和向后延伸的厚的线性增强。颈椎和腰椎MRI也显示弥漫性软脑膜疾病。脑部MRI显示左右侧裂、鞍上池和后颅窝的局灶性软脑膜增强;该模式与转移性疾病一致。患者接受了T6-T10椎板切除术，以进行肿瘤活检和减瘤。组织学检查显示WHO III级胶质神经元肿瘤伴玫瑰状神经纤维样岛。突触素和神经丝（NF）阳性染色的神经出现的组件内，而胶质细胞酸性蛋白（GFAP）免疫阳性是明显的星形细胞瘤的肿瘤组成部分。Ki-67标记指数为7%。这种肿瘤模式，现在被纳入2007年世界卫生组织（WHO）的中枢神经系统肿瘤分类中，作为间变性星形细胞瘤的模式变异（Kleihues et al. In：Louis et al.（eds）WHO classification of tumors of the central nervous system，2007），首次由Teo et al.在1999年在4个病例系列中描述。大多数随后报告的病例将其描述为大脑原发性肿瘤。在此，我们报告了一个独特的例子，脊髓胶质神经元肿瘤与神经纤毛样岛相关的软脑膜传播涉及整个颅脊髓轴。
A 54-year-old Caucasian female presented with a 1 year history of intermittent numbness of the left leg progressing to bilateral, lower extremity sensory loss that advanced to include impaired vibration and proprioception. The subsequent thoracic spine magnetic resonance imaging (MRI) scan revealed a heterogeneous, avidly enhancing, centrally situated spinal cord mass involving T7 through T10 in association with thick linear enhancement of the anterior and posterior cord surfaces extending both superiorly and inferiorly. Both the cervical and lumbar spine MRI demonstrated diffuse leptomeningeal disease as well. A brain MRI revealed focal leptomeningeal enhancement in the left and right sylvian fissures, the suprasellar cistern, and the posterior fossa; a pattern consistent with metastatic disease. The patient underwent a T6-T10 laminectomy for tumor biopsy and debulking. Histology revealed a WHO grade III glioneuronal tumor with rosetted neuropil-like islands. Synaptophysin and neurofilament (NF) positive staining was noted within the neural appearing component, whereas, glial fibrillary acidic protein (GFAP) immunopositivity was evident in the fibrillary astrocytoma component of the tumor. The Ki-67 labeling index was 7%. This tumor pattern, now included in the 2007 World Health Organization (WHO) classification of central nervous system tumours as a pattern variation of anaplastic astrocytoma (Kleihues et al. In: Louis et al. (eds) WHO classification of tumours of the central nervous system, 2007), was first described in a four-case series by Teo et al. in 1999. The majority of subsequently reported cases described them as primary tumors of the cerebrum. Herein, we report a unique example of a spinal glioneuronal tumor with neuropil-like islands with associated leptomeningeal dissemination involving the entire craniospinal axis.