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首页> 外文期刊>European journal of cancer: official journal for European Organization for Research and Treatment of Cancer (EORTC) [and] European Association for Cancer Research (EACR) >Low- and high-grade esthesioneuroblastomas display a distinct natural history and outcome
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Low- and high-grade esthesioneuroblastomas display a distinct natural history and outcome

机译：低度和高度审美性神经母细胞瘤表现出明显的自然病史和预后

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Purpose: Esthesioneuroblastomas, also called olfactory neuroblastomas (ENB) represent a rare sinonasal neurectodermal tumour which prognostic factors are unsteadily described. Patients and methods: Clinical and pathological characteristics were analysed in patients treated at Gustave Roussy Institute between 1979 and 2009. Results: Out of 63 patients, 19 patients were reclassified and 44 patients were eligible for the analysis. Multivariate analysis revealed that T staging of the modified Dulguerov TNM staging and Hyams grade > III (that we termed high-grade ENB) were the only independent prognostic factors for overall survival (OS). As compared to patients with low-grade ENB (Hyams grade ≤ III), patients with high-grade ENB have higher T4 staging (p = 0.02), have frequent lymph node involvement (p = 0.009) and are more often unresectable (p = 0.005). Resected patients with high-grade ENB frequently displayed mainly leptomeningeal metastasis (n = 4/6) in contrast to patients with low-grade ENB who typically experience late loco-regional recurrence (n = 10/25). With a median follow-up of 9.6 years, median DFS and OS for resected low-grade ENB were 5.4 and 20.5 years, respectively. Conversely, median DFS and OS for high-grade ENB were 1.5 and 2.5 years, respectively. Conclusion: Low and high-grade ENB display distinct patterns at presentation and relapse, leading to different prognosis. Therefore, they may be regarded as distinct entities.
机译：目的：粘膜神经母细胞瘤，也称为嗅神经母细胞瘤（ENB），代表一种罕见的鼻窦神经直肠真皮瘤，其预后因素不稳定。患者和方法：分析了1979年至2009年在Gustave Roussy研究所接受治疗的患者的临床和病理特征。结果：在63例患者中，对19例患者进行了重新分类，其中44例符合条件。多变量分析显示，改良的Dulguerov TNM分期的T分期和Hyams等级> III（我们称为高等级ENB）是整体生存（OS）的唯一独立预后因素。与低度ENB患者（Hyms级≤III）相比，高度ENB患者的T4分期更高（p = 0.02），淋巴结受累频繁（p = 0.009），更经常不能切除（p = 0.005）。高位ENB的切除患者通常主要表现为软脑膜转移（n = 4/6），而低位ENB的患者通常会发生局部局部复发（n = 10/25）。中位随访期为9.6年，切除的低级ENB的中位DFS和OS分别为5.4年和20.5年。相反，高级ENB的中位DFS和OS分别为1.5年和2.5年。结论：低级和高级ENB在表现和复发时表现出不同的模式，导致不同的预后。因此，它们可以被视为不同的实体。

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