Spinal cord glioneuronal tumor with neuropil-like islands with 1p/19q deletion in an adult with low-grade cerebral oligodendroglioma.
Spinal cord glioneuronal tumor with neuropil-like islands with 1p/19q deletion in an adult with low-grade cerebral oligodendroglioma.
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DOI：
10.1007/s11060-011-0760-9
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发表时间：
2012-04
影响因子：
3.9
通讯作者：
Iwamoto FM
中科院分区：
文献类型：
--
作者：
Fraum TJ;Barak S;Pack S;Lonser RR;Fine HA;Quezado M;Iwamoto FM
具有神经纤毛样岛的胶质神经元肿瘤（GTNI）被认为是星形细胞瘤的一种罕见变体，其特征在于表达神经元标记物的细胞离散聚集，这些标记物在GFAP阳性的胶质细胞背景中标点。在24例已发表的GTNI病例中，只有2例发生在成人脊髓;没有一例同时发生另一种CNS肿瘤;没有一例检测到典型的少突胶质细胞瘤1 p/19 q缺失。一名48岁男性，无明显既往病史，通过对患者全身强直阵挛性癫痫发作后发现的病灶进行立体定向活检，诊断为WHO II级少突胶质细胞瘤。通过FISH分析，该肿瘤表现出1 p/19 q缺失，存在于高达80%的少突胶质细胞瘤中。患者接受了14个月周期的替莫唑胺治疗，他的脑肿瘤有轻微反应。当患者随后报告下肢进行性感觉异常时，MRI显示胸脊髓的增强囊性肿瘤，通过组织学分析诊断为GTNI。通过FISH分析，该病变表现出相同的1 p/19 q缺失存在于并发脑少突胶质细胞瘤。这例脊髓GTNI与1 p/19 q缺失构成了脊髓GTNI在成人患者的第三次报告，GTNI在一个单独的中枢神经系统肿瘤的个人的第一次报告，和GTNI与1 p/19 q缺失的第一次报告。本病例建立了GTNI和少突胶质细胞瘤之间潜在的遗传亲缘关系，值得进一步研究。
Glioneuronal tumor with neuropil-like islands (GTNI) is considered a rare variant of astrocytoma, characterized by discrete aggregates of cells expressing neuronal markers that punctuate a GFAP-positive glial background. Of the 24 published GTNI cases, only two occurred in adult spinal cords; none occurred concurrent with another CNS tumor; and none of those tested exhibited the 1p/19q deletion typical of oligodendroglioma. A 48-year-old man without significant past medical history was diagnosed with a WHO grade II oligodendroglioma by stereotactic biopsy of a lesion discovered after the patient suffered a generalized tonic-clonic seizure. By FISH analysis, this tumor exhibited the 1p/19q deletion present in up to 80% of oligodendrogliomas. The patient received 14 monthly cycles of temozolomide, and his cerebral tumor had a minor response. When the patient subsequently reported progressive paresthesias of his lower extremities, an MRI revealed an enhancing, cystic tumor of the thoracic spinal cord that was diagnosed as GTNI by histological analysis. By FISH analysis, this lesion exhibited the same 1p/19q deletion present in the concurrent cerebral oligodendroglioma. This case of a spinal cord GTNI with 1p/19q deletions constitutes the third report of a spinal cord GTNI in an adult patient; the first report of a GTNI in an individual with a separate CNS neoplasm; and the first report of a GTNI with 1p/19q deletions. This case establishes a potential genetic kinship between GTNI and oligodendroglioma that warrants further investigation.
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影响因子：
--
作者：
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通讯作者：
Reddy, Kavita S.
影响因子：
3.3
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