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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute.
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Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute.

机译：三级转诊神经病学研究所的神经元/神经胶质瘤肿瘤的病理频谱。

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Neuronal/glioneuronal tumors are uncommon neoplasms of the CNS with frequent association with refractory epilepsy. Reports documenting the entire spectrum of neuronal/glioneuronal tumors are scarce in the literature. Zulch et al. from Germany in a large series reported that neuronal/glioneuronal tumors accounted for 0.4% (38/9000 cases) of all brain tumors, with similar incidence reported from Japan (0.4%), with higher incidence from Korea (2.1%). However, data from the Indian subcontinent are lacking. We reviewed 244 cases of neuronal/glioneuronal tumors of the CNS diagnosed over the last decade at our Institute and they constituted 0.86% of all CNS tumors (244/28061) received in that period. Mean age at presentation was 25.06 years (range: 1-75 years) with male preponderance (M:F = 1.54 : 1). The majority occurred in third decade (76 cases, 31.4%), with only few cases occurring beyond fifth decade (13 cases, 5.3%). Ganglioglioma/gangliocytoma (94 cases, 38.52%) was the most frequent followed by central neurocytoma (86 cases, 35.24%), paraganglioma (32 cases, 13.52%), dysembryoplastic neuroepithelial tumors (DNET) (21 cases, 8.6%), desmoplastic infantile astrocytoma/desmoplastic infantile ganglioglioma (DIA/DIG) (6 cases, 2.45%), papillary glioneuronal tumor (PGNT) (3 cases, 1.22%) and rosette-forming glioneuronal tumor (RGNT) (1 case, 0.4%). Association with seizures was noted in 40.95% of cases. Glioneuronal tumors are an expanding group of tumors with varying spectra of morphologic patterns and biological behavior. An improved understanding has direct clinical implications for optimizing current treatments and developing novel therapeutic approaches. Although most glioneuronal tumors carry a favorable prognosis, other factors such as inaccessibility to surgical resection and rarely, malignant transformation, make it difficult to accurately predict the biological behavior based on histopathology alone. Reliable prognostic markers remain to be defined.
机译：神经元/神经胶质神经元肿瘤是中枢神经系统罕见的肿瘤，与难治性癫痫频繁相关。关于神经元/神经胶质神经瘤的整个频谱的文献报道很少。 Zulch等。来自德国的大量报告指出，神经元/神经胶质神经元肿瘤占所有脑肿瘤的0.4％（38/9000例），日本报道的发生率相似（0.4％），韩国报道的发生率较高（2.1％）。但是，缺乏来自印度次大陆的数据。我们回顾了过去十年来在我们研究所诊断的244例CNS神经元/神经胶质神经元肿瘤病例，它们占同期所有CNS肿瘤（244/28061）的0.86％。出现时的平均年龄为25.06岁（范围：1-75岁），男性占优势（男：女= 1.54：1）。多数发生在第三十年（76例，31.4％），只有很少的病例发生在第五十年之后（13例，5.3％）。神经胶质瘤/神经节细胞瘤最常见（94例，38.52％），其次是中枢神经细胞瘤（86例，35.24％），副神经节瘤（32例，13.52％），发育不良性神经上皮肿瘤（DNET）（21例，8.6％），增生婴儿星形细胞瘤/增生性婴儿神经节胶质瘤（DIA / DIG）（6例，2.45％），乳头神经胶质神经胶质瘤（PGNT）（3例，1.22％）和形成玫瑰花结神经胶质神经胶质瘤（RGNT）（1例，0.4％）。在40.95％的病例中发现与癫痫发作相关。神经胶质神经瘤是一组不断扩大的肿瘤，其形态学模式和生物学行为谱均不同。更好的理解对于优化当前治疗方法和开发新的治疗方法具有直接的临床意义。尽管大多数神经胶质神经瘤预后良好，但其他因素（如无法进行手术切除以及极少发生恶性转化）使仅凭组织病理学难以准确预测生物学行为。可靠的预后指标仍有待确定。

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