Outcome analysis of childhood pilocytic astrocytomas: a retrospective study of 148 cases at a single institution
Outcome analysis of childhood pilocytic astrocytomas: a retrospective study of 148 cases at a single institution
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DOI：
10.1111/nan.12013
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发表时间：
2013-10-01
影响因子：
5
通讯作者：
Figarella-Branger, D.
中科院分区：
文献类型：
--
作者：
Colin, C.;Padovani, L.;Figarella-Branger, D.
背景：毛细胞性星形细胞瘤(PAS)的特点是预后良好，尽管已有多个不良预后因素的报道。丝裂原活化蛋白激酶通路在肿瘤的发生发展中起着重要作用。目的：报道148例儿童前列腺癌的临床病理和生物学预后因素。方法：从患者档案和邮寄查询中收集临床资料。对病理标本进行集中复查。应用逆转录聚合酶链式反应(RT-PCR)对47例冰冻标本和23例福尔马林固定石蜡包埋组织进行荧光原位杂交分析KIAA1549：BRAF三种主要融合亚型。研究肿瘤部位、手术年龄、手术切除范围、组织学亚型和RT-PCR KIAA1549：BRAF融合对预后的影响。结果：恶性星形细胞瘤和下丘脑-交叉部位预后较差[P<；总生存期P<；0.001，无进展生存期P=0.001]。与其他患者相比，接受完全手术切除的患者有更好的OS(P=0.004)和更长的PFS(P<；0.001)。年龄也是OS的一个强有力的预后因素，但不是PFS。婴儿(<；1岁)和幼儿(<；3岁)的结果比其他人差得多(P<；0.001和P=0.004)。KIAA1549：BRAF融合状态不能预测结果。结论：本研究强调了PAS良好的预后因素，但H/C PA仍是一个预后较差的亚型，与年龄小、PMA变异和手术不彻底有关。搜索KIAA1549：建议在PA类型的肿瘤中进行BRAF融合，即使其对预后的影响尚不清楚。
Background: Pilocytic astrocytomas (PAs) are characterized by an excellent prognosis although several factors of adverse outcome have been reported. The mitogenactivated protein kinase pathway plays a major role in their tumorigenesis. Aim:To report a series of 148 PAs in children to define clinicopathological and biological prognostic factors. Methods:Clinical data were collected from patient files and mail inquiry. Pathological specimenswere centrally reviewed. The three major KIAA1549:BRAF fusion subtypes were analysed by reverse transcription polymerase chain reaction (RT-PCR) in a subset of 47 frozen cases and by fluorescence in situ hybridization on formalin-fixed paraffin-embedded tissue in 23 cases. Tumour location, age at surgery, extent of surgical removal, histological subtype and KIAA1549:BRAF fusion by RT-PCR were searched for prognostic significance. Results:Pilomyxoid astrocytoma (PMA) and the hypothalamo-chiasmatic (H/C) location were associated with aworse prognosis [P < 0.001 for overall survival (OS) and P = 0.001 for progression-free survival (PFS)]. Patients who underwent complete surgical excision had a better OS (P = 0.004) and a longer PFS (P < 0.001) than the others. Age was also a strong prognostic factor for OS but not for PFS. Infants (< 1 year) and young children (< 3 years) had a much worse outcome than the others (P < 0.001 and P = 0.004 respectively). KIAA1549:BRAF fusion status was not predictive of outcome. Conclusion: This study highlights the good prognostic factors of PAs but H/C PA remains a subgroup with dismal prognosis associated with young age, PMA variant and incomplete surgery. Search for KIAA1549:BRAF fusion in tumours with PA pattern is recommended even though the prognostic impact is still unclear.