儿童后颅窝肿瘤的诊断算法：验证研究。
A Diagnostic Algorithm for Posterior Fossa Tumors in Children: A Validation Study.
机构信息
From the Division of Neuroradiology (C.A.P.F.A., J.S.M.-S. S.R.T., F.G.G., A.V.), Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Department of Radiology (C.d.C.L.), Hospital das Clinicas, Faculdade de Medicina de Sao Paulo, Sao Paulo.
出版信息
AJNR Am J Neuroradiol. 2021 May;42(5):961-968. doi: 10.3174/ajnr.A7057. Epub 2021 Mar 4.
BACKGROUND AND PURPOSE
Primary posterior fossa tumors comprise a large group of neoplasias with variable aggressiveness and short and long-term outcomes. This study aimed to validate the clinical usefulness of a radiologic decision flow chart based on previously published neuroradiologic knowledge for the diagnosis of posterior fossa tumors in children.
MATERIALS AND METHODS
A retrospective study was conducted (from January 2013 to October 2019) at 2 pediatric referral centers, Children's Hospital of Philadelphia, United States, and Great Ormond Street Hospital, United Kingdom. Inclusion criteria were younger than 18 years of age and histologically and molecularly confirmed posterior fossa tumors. Subjects with no available preoperative MR imaging and tumors located primarily in the brain stem were excluded. Imaging characteristics of the tumors were evaluated following a predesigned, step-by-step flow chart. Agreement between readers was tested with the Cohen κ, and each diagnosis was analyzed for accuracy.
RESULTS
A total of 148 cases were included, with a median age of 3.4 years (interquartile range, 2.1-6.1 years), and a male/female ratio of 1.24. The predesigned flow chart facilitated identification of pilocytic astrocytoma, ependymoma, and medulloblastoma tumors with high sensitivity and specificity. On the basis of the results, the flow chart was adjusted so that it would also be able to better discriminate atypical teratoid/rhabdoid tumors and medulloblastoma groups 3 or 4 (sensitivity = 75%-79%; specificity = 92%-99%). Moreover, our adjusted flow chart was useful in ruling out ependymoma, pilocytic astrocytomas, and medulloblastoma tumors.
CONCLUSIONS
The modified flow chart offers a structured tool to aid in the adjunct diagnosis of pediatric posterior fossa tumors. Our results also establish a useful starting point for prospective clinical studies and for the development of automated algorithms, which may provide precise and adequate diagnostic tools for these tumors in clinical practice.
背景与目的
原发性后颅窝肿瘤包括一大组具有不同侵袭性和短期及长期预后的肿瘤。本研究旨在验证基于先前发表的神经放射学知识制定的后颅窝肿瘤放射学决策流程图在儿童后颅窝肿瘤诊断中的临床实用性。
材料与方法
本研究为回顾性研究，在美国费城儿童医院和英国大奥蒙德街儿童医院 2 家儿科转诊中心开展（2013 年 1 月至 2019 年 10 月）。纳入标准为年龄小于 18 岁，经组织学和分子学证实为后颅窝肿瘤。排除术前无磁共振成像（MRI）且肿瘤主要位于脑干的患者。采用预先设计的、逐步的流程图评估肿瘤的影像学特征。采用 Cohen κ 检验评估读者间的一致性，并对每种诊断进行准确性分析。
结果
共纳入 148 例患者，中位年龄为 3.4 岁（四分位距，2.1-6.1 岁），男女比例为 1.24。预设计的流程图有助于高度敏感和特异地区分毛细胞星形细胞瘤、室管膜瘤和髓母细胞瘤。基于这些结果，对流程图进行了调整，使其能够更好地区分非典型畸胎瘤/横纹肌样瘤和髓母细胞瘤 3 或 4 级（敏感性=75%-79%；特异性=92%-99%）。此外，我们调整后的流程图有助于排除室管膜瘤、毛细胞星形细胞瘤和髓母细胞瘤。
结论
改良后的流程图提供了一种辅助诊断儿童后颅窝肿瘤的结构化工具。我们的研究结果还为前瞻性临床研究和自动化算法的开发提供了有用的起点，这些算法可能为临床实践中这些肿瘤提供准确、充分的诊断工具。
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