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对比增强模式可预测非WNT/SHH型髓母细胞瘤患者的不良生存情况。

Contrast enhancement pattern predicts poor survival for patients with non-WNT/SHH medulloblastoma tumours.

作者信息

Łastowska Maria, Jurkiewicz Elżbieta, Trubicka Joanna, Daszkiewicz Paweł, Drogosiewicz Monika, Malczyk Katarzyna, Grajkowska Wiesława, Matyja Ewa, Cukrowska Bożena, Pronicki Maciej, Perek-Polnik Marta, Perek Danuta, Dembowska-Bagińska Bożenna

机构信息

Department of Pathology, The Children's Memorial Health Institute, Av. Dzieci Polskich 20, 04-730, Warsaw, Poland,

出版信息

J Neurooncol. 2015 May;123(1):65-73. doi: 10.1007/s11060-015-1779-0. Epub 2015 Apr 11.

Abstract

Recent studies revealed the biological heterogeneity of medulloblastoma, with the existence of at least four groups which are associated with several clinical and morphological features. We investigated for further correlations between molecular types, location of tumours, their contrast enhancement pattern and survival of patients. Altogether 76 tumours were analyzed and molecular subtypes were identified by immunohistochemistry using representative antibodies, detection of chromosome 6 monosomy and CTNNB1 mutation. The site of the tumour was assessed on diagnosis using Magnetic Resonance images and intra-operative surgical reports. In addition, the gadolinium enhancement pattern was also investigated in pre-treatment tumours. Cerebellar hemispheric location was associated with SHH tumours (p < 0.001), as opposed to midline location being typical for WNT and non-WNT/SHH tumours. Remarkably, for patients with non-WNT/SHH tumours, the extensive gadolinium enhancement pattern (present in >75% of tumour volume) predicted worse OS and EFS than for those with none/weak or heterogeneous enhancement (>10-75% of tumour volume), (both p < 0.001). Our analysis indicates that distribution of the medulloblastoma tumours location is related to the biological characteristics of tumour. Importantly, the enhancement pattern of the tumour may be a clinically useful prognostic marker for patients with non-WNT/SHH medulloblastomas.

摘要

最近的研究揭示了髓母细胞瘤的生物学异质性,其至少存在四组,且与多种临床和形态学特征相关。我们进一步研究了分子类型、肿瘤位置、其对比增强模式与患者生存率之间的相关性。共分析了76个肿瘤,并通过使用代表性抗体的免疫组织化学、6号染色体单体检测和CTNNB1突变来鉴定分子亚型。利用磁共振图像和术中手术报告在诊断时评估肿瘤部位。此外,还研究了治疗前肿瘤的钆增强模式。小脑半球位置与SHH肿瘤相关(p < 0.001),而中线位置是WNT和非WNT/SHH肿瘤的典型特征。值得注意的是,对于非WNT/SHH肿瘤患者,广泛钆增强模式(肿瘤体积的>75%出现)比无/弱或异质性增强(肿瘤体积的>10 - 75%)的患者的总生存期(OS)和无进展生存期(EFS)更差(两者p < 0.001)。我们的分析表明,髓母细胞瘤肿瘤位置的分布与肿瘤的生物学特征相关。重要的是,肿瘤的增强模式可能是非WNT/SHH髓母细胞瘤患者临床上有用的预后标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78b5/4439433/c2f9efee92a3/11060_2015_1779_Fig1_HTML.jpg

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