端粒酶表达作为神经母细胞瘤的补充预后因素。

Bahnassy Abeer A, Fawzy Mohamed, El-Wakil Mohamed, Al-Bolkainy Tarek M N, Kamel Mahmoud M, Abd El-Azim Hisham

The Departments of Pathology , NCI, Cairo University.

J Egypt Natl Canc Inst. 2010 Dec;22(4):191-200.

BACKGROUND

Neuroblastoma (NB) is an aggressive tumor of childhood with a highly heterogeneous course. Identification of standard prognostic factors has several limitations. Hence, there is an increasing demand to identify new prognostic factors and tools that help in risk stratification of patients for proper treatment.

OBJECTIVE

To assess the prognostic and predictive impact of quantitative telomerase expression in NB patients.

METHODS

We investigated quantitative telomerase expression by immunohistochemistry in 44 neuroblastoma patients. Results were correlated with standard prognostic factors, n-myc amplification by chromogen in situ hybridization (CISH) and patients' response to treatment and survival.

RESULTS

High telomerase expression and n-myc expression were reported in 52.3% and 56.8% ; respectively. There was a significant relation between telomerase expression and n-myc amplification. Stage 3 and 4 represented 91% of patients. Thirteen patients showed complete remission, 9 partial remission, 19 no response and 3 showed progressive disease. The median followup was 3 years with 80% overall and 72% progression free survival for the low teleomerase expression group; and 52.2% and 55.4% for high teleomerase expression group. High Telomerase expression by immunohistochemistry was significantly associated with n-myc amplification and with poor response to treatment with a trend toward lower overall and progression free survival.

CONCLUSIONS

Telomerase expression by immunohistochemistry is a simple potential tool for risk stratification of NB patients. CISH can serve as a readily available alternative simple tool, compared to FISH, in identifying neuroblastoma cases with abnormal n-myc gene copy number.

KEY WORDS

Telomerase expression- N-myc amplification- Prognosis- Neuroblastoma.

背景

神经母细胞瘤（NB）是一种侵袭性儿童肿瘤，病程高度异质性。标准预后因素的识别存在若干局限性。因此，越来越需要识别有助于对患者进行风险分层以实现恰当治疗的新预后因素和工具。

目的

评估NB患者中定量端粒酶表达的预后和预测影响。

方法

我们通过免疫组织化学研究了44例神经母细胞瘤患者的定量端粒酶表达。结果与标准预后因素、原位杂交显色法检测的n-myc扩增以及患者的治疗反应和生存情况相关。

结果

分别有52.3%和56.8%的患者报告有高端粒酶表达和n-myc表达。端粒酶表达与n-myc扩增之间存在显著关联。3期和4期患者占91%。13例患者完全缓解，9例部分缓解，19例无反应，3例疾病进展。中位随访时间为3年，低端粒酶表达组的总生存率为80%，无进展生存率为72%；高端粒酶表达组分别为52.2%和55.4%。免疫组织化学检测的高端粒酶表达与n-myc扩增显著相关，且与治疗反应差相关，总体生存率和无进展生存率有降低趋势。