间变性淋巴瘤激酶（ALK）表达是小儿髓母细胞瘤WNT激活型的一种新型标志物，也是患者预后良好的一个指标。

ALK Expression Is a Novel Marker for the WNT-activated Type of Pediatric Medulloblastoma and an Indicator of Good Prognosis for Patients.

作者信息

Łastowska Maria, Trubicka Joanna, Niemira Magdalena, Paczkowska-Abdulsalam Magdalena, Karkucińska-Więckowska Agnieszka, Kaleta Magdalena, Drogosiewicz Monika, Tarasińska Magdalena, Perek-Polnik Marta, Krętowski Adam, Dembowska-Bagińska Bożenna, Grajkowska Wiesława, Pronicki Maciej, Matyja Ewa

机构信息

*Department of Pathology §Clinic of Oncology, The Children's Memorial Health Institute †Department of Experimental and Clinical Pathology, Mossakowski Medical Research Centre Polish Academy of Sciences, Warsaw ‡Clinical Research Centre ∥Department of Endocrinology, Diabetology and Internal Medicine, Medical University of Białystok, Białystok, Poland.

出版信息

Am J Surg Pathol. 2017 Jun;41(6):781-787. doi: 10.1097/PAS.0000000000000847.

DOI:10.1097/PAS.0000000000000847

PMID:28338501

Abstract

ALK gene rearrangements were identified in a variety of cancers, including neuroblastoma, where the presence of ALK expression is associated with adverse prognosis. ALK mutations have recently been found in the pediatric brain tumor medulloblastoma, and microarray data indicate that ALK is highly expressed in a subset of these tumors. Therefore, we investigated whether ALK expression correlates with transcriptional profiles and clinical features of medulloblastoma. Tumors from 116 medulloblastoma patients were studied at diagnosis for the detection of ALK expression at the RNA level by an application of NanoString technology and at the protein level by immunohistochemistry using antibody ALK clone D5F3. The results indicate that ALK expression, at both the RNA and the protein levels, is strongly associated with the WNT-activated type of tumors and therefore may serve as a useful marker for the detection of this type of medulloblastoma. Importantly, ALK protein expression alone is also an indicator of good prognosis for medulloblastoma patients.

摘要

在多种癌症中都发现了ALK基因重排，包括神经母细胞瘤，其中ALK表达的存在与不良预后相关。最近在儿童脑肿瘤髓母细胞瘤中发现了ALK突变，微阵列数据表明ALK在这些肿瘤的一个亚组中高度表达。因此，我们研究了ALK表达是否与髓母细胞瘤的转录谱和临床特征相关。对116例髓母细胞瘤患者的肿瘤在诊断时进行研究，通过应用NanoString技术在RNA水平检测ALK表达，并使用抗体ALK克隆D5F3通过免疫组织化学在蛋白质水平检测ALK表达。结果表明，RNA和蛋白质水平的ALK表达均与WNT激活型肿瘤密切相关，因此可能作为检测此类髓母细胞瘤的有用标志物。重要的是，单独的ALK蛋白表达也是髓母细胞瘤患者预后良好的指标。