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多国回顾性中枢病理学神经母细胞瘤研究:在资源有限的环境下建立区域性病理学转诊中心的经验教训。

Multinational Retrospective Central Pathology Review of Neuroblastoma: Lessons Learned to Establish a Regional Pathology Referral Center in Resource-Limited Settings.

机构信息

From the Departments of Pathology (Santiago, Hayes).

St Jude Children's Research Hospital, Memphis, Tennessee; the Departments of Pathology (Polanco) and Oncology (Fuentes-Alabi).

出版信息

Arch Pathol Lab Med. 2021 Feb 1;145(2):214-221. doi: 10.5858/arpa.2019-0570-OA.

Abstract

CONTEXT.—: Several countries of the Central America and Caribbean region have been sharing regional neuroblastoma (NB) treatment guidelines. However, there is no standardization in the diagnosis, subclassification, or tumor biology to aid in the risk stratification of these patients.

OBJECTIVE.—: To examine the histology and assess the accuracy of the local pathology reports; to evaluate the usefulness of manual MYCN immunohistochemistry (IHC); and to use NB as a model to identify the needs to establish a central pathology review (CPR) program in this region.

DESIGN.—: A retrospective CPR of specimens derived from patients with a diagnosis of NB and treated under the regional NB guidelines between 2012 and 2017 was conducted, allowing for a comparison between local diagnoses and the CPR diagnoses. Manual MYCN IHC was performed in the confirmed NB specimens and the results compared with known fluorescence in situ hybridization or automated IHC results, when available.

RESULTS.—: The 156 specimens reviewed included 460 blocks and 183 original slides. Neuroblastoma was confirmed in 138 samples (88.5%), but low concordance rates for Shimada classification (n = 39; 25.0%), mitotic-karyorrhectic index (n = 4; 2.5%), and International Neuroblastoma Pathology Classification (n = 18; 11.5%) were noted. Manual MYCN IHC performed on 120 specimens showed conclusive results in 89.2% (28 positive, 23.4%; 79 negative, 65.8%) and questionable results in 10.8% (n = 13).

CONCLUSIONS.—: This retrospective CPR highlights the need for a CPR program to serve this region, to ensure correct diagnosis and subclassification of NB, and to provide manual MYCN IHC-with reflexing to fluorescence in situ hybridization, if questionable. This approach can further regional collaboration, enhance test utilization, and ultimately improve patients' outcomes.

摘要

背景

中美洲和加勒比地区的几个国家一直在共享区域性神经母细胞瘤(NB)治疗指南。然而,在这些患者的风险分层方面,尚无诊断、亚型分类或肿瘤生物学的标准化方法。

目的

检查组织学并评估当地病理报告的准确性;评估手动 MYCN 免疫组织化学(IHC)的有用性;并使用 NB 作为模型,确定在该地区建立中央病理审查(CPR)计划的需求。

设计

对 2012 年至 2017 年期间根据区域 NB 指南治疗的 NB 诊断患者的标本进行回顾性 CPR,允许比较当地诊断和 CPR 诊断。在确诊的 NB 标本中进行手动 MYCN IHC,并在有条件的情况下与已知的荧光原位杂交或自动 IHC 结果进行比较。

结果

共回顾了 156 例标本,包括 460 个块和 183 张原始载玻片。138 例(88.5%)标本证实为神经母细胞瘤,但 Shimada 分类(n=39;25.0%)、有丝分裂-核破裂指数(n=4;2.5%)和国际神经母细胞瘤病理分类(n=18;11.5%)的一致性率较低。对 120 例标本进行的手动 MYCN IHC 显示,89.2%(28 例阳性,23.4%;79 例阴性,65.8%)的结果明确,10.8%(n=13)的结果可疑。

结论

这项回顾性 CPR 强调了需要建立 CPR 计划,以确保正确诊断和 NB 的亚型分类,并提供手动 MYCN IHC-如果可疑,可进行荧光原位杂交。这种方法可以进一步促进区域合作,提高检测利用率,并最终改善患者的预后。

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