皮膚神経線維腫の組織学的診断および分類における観察者間のばらつきと精度を評価する

Assessing interobserver variability and accuracy in the histological diagnosis and classification of cutaneous neurofibromass.

• Nicolas Ortonne
• Steven L Carroll
• Fausto J Rodriguez
• Douglas C Miller
• Rosalynn M Nazarian
• Jaishri O Blakeley
• Zachary B Madaj
• Sharad K Verma
• Anat Stemmer-Rachamimov

抄録

背景:

皮膚神経線維腫（cNF）は、神経線維腫症1型（NF1）患者において最も一般的な腫瘍であり、生活の質の低下と関連している。現在のところ、cNFを臨床的または病理組織学的に記述するための広く受け入れられた標準化された言語は存在しない。本研究の目的は、皮膚に関与する神経線維腫の記述および報告における病理医間の観察者間の一致を評価することである。

Background: Cutaneous neurofibromas (cNFs) are the most common tumors in people with neurofibromatosis type 1 (NF1) and are associated with reduced quality of life. There is currently no widely accepted standardized language for describing cNFs clinically or histopathologically. The objective of this study was to evaluate interobserver agreement across pathologists in describing and reporting of neurofibromas involving the skin.

方法:

Methods: Twenty-eight (H&E)-stained slides of cNF were scanned using an Aperio XT scanner. The digital images were reviewed by 6 pathologists, who entered free text of up to a 200 word description for each case into a REDcap database. Responses were analyzed for the most commonly used terms based on frequency, as well as agreement (reported as concordance) between reviewers.

結果:

病理医がcNFの組織学的分類に最も一般的に使用している用語のセットが、一致と不一致の領域とともに同定された。本研究では、皮膚に関与する神経線維腫のすべてが皮膚神経線維腫ではないこと、非定型的な特徴や異種要素の有無について、レビュアー間で強い合意があったことが示されている。同意が得られなかった分野としては、cNFのサブタイプ、伸展の定義および成長パターン、皮膚に関与する皮内成分のない叢状神経線維腫とcNFの区別などが挙げられる。

Results: A set of the terms most commonly used by pathologists for the histological classification of cNF along with areas of agreement and disagreement have been identified. The study shows that there was strong agreement across reviewers that not all neurofibromas involving the skin are cutaneous neurofibromas and regarding the presence or absence of atypical features and heterologous elements. Areas of less concordance were identified and include cNF subtypes, definition of extension and pattern of growth, as well as the distinction of a cNF from a plexiform without an intraneural component involving skin.

結論:

この研究は、堅牢な分類システムの開発と、皮膚神経線維腫の病理組織学的診断基準「ゴールドスタンダード」の考案に向けた第一歩である。

Conclusions: This work is the first step towards development of a robust classification system and devising "gold standard" histopathologic diagnostic criteria for cutaneous neurofibromas.

© The Author(s) 2019. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.