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		<h1 class="catchCopy">病理診断のスタンダードをめざした腫瘍病理鑑別診断アトラス！</h1>
		

		<h1 class="copy">腫瘍病理鑑別診断アトラス</h1>
		<h1 class="title">NET・下垂体・副甲状腺・副腎<span class="version"></span></h1>
		

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					編集：笹野公伸（東北大学教授）
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					<span class="transparent">編集</span>　亀山香織（慶応義塾大学准教授）
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					監修：腫瘍病理鑑別診断アトラス刊行委員会
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		<div class="headers"><h3 class="current content">内容</h3><h3 class="preface">序文</h3><h3 class="indexes">主要目次</h3></div><section class="current content">内分泌臓器に発生する病変は，その病理組織形態所見が複雑で，病変も多岐にわたる．まさに，病理医と臨床医との間のクロストークが他の臓器よりも重要な領域といえる．本書では，WHO分類2017の改定内容を見据えて最新の知見を入れて解説する．本邦では数少ない内分泌疾患の病理および臨床双方の専門家の解説により，病理医がこれらの疾患の病理組織診断に遭遇した際の臨床側からのminimum requirementに応えられるようにした．</section><section class="preface">序文<br><br>　内分泌臓器に発生する病変はその病理組織形態所見が複雑で，病変も多岐にわたり，病理診断医にとり取っ付きにくい側面も否定できない．さらに，種々のホルモンを過剰合成，分泌する病変も少なくなく，一部の病変では病理組織鑑別診断に内分泌所見が時に重要な役割を果たすことから，病理診断医が鑑別診断にあたり，どこまでその患者の内分泌所見を必要とするのかというところも問題になる．特に最近の臨床内分泌学の進歩は目覚ましく，病理医と臨床医との間のクロストークが他の臓器病変よりも重要であることは言うまでもない．加えて，副甲状腺，副腎，下垂体疾患は診断，治療する施設が限られ，本邦では特に集約化が進んでいることから，他の臓器に比較して病理医が診断を経験する機会は決して多くない．このような背景から，どうしてもこれらの疾患の病理組織診断は敬遠される傾向があることは否めない．<br>　また，NET（neuroendocrine tumor）に関しても膵消化管に発生する，いわゆるGEPNET（gastroenteropancreatic NET）に関しては，WHO2010でかなりその概念，病理組織診断基準は整理されてきたが，NEC（neuroendocrine carcinoma）／ NET G3は1つの疾患として捉えてよいのかどうか，MANEC（mixed adenoneuroendocrine carcinoma）の概念など，これから整理していく必要のある課題が決して少なくはない．しかし，肺／呼吸器に発生するNET はWHO2015の改訂では依然としてカルチノイドという病名が用いられていて，病理医／臨床医双方とも注意が必要である．一方，GEPNETでは病理組織診断，特にKi67 labeling indexに基づくgrading，SSTR（somatostatin receptor）の発現動態などによって治療方針が大きく異なることから，病理医と臨床医とのクロストークも近年，その重要性はますます増してきている．今回のWHO2017では，膵臓のPNET（pancreatic NET）ではあるがNET G3，NECの概念がかなり整理されてきたので，本書ではこの最新の知見も取り入れて解説を加えた．また，副腎腫瘍に関してもWHO2017の改訂内容を反映して最新の知見を入れている．<br>　本書では各々の臓器に発生する病変に関して，「検鏡前の確認事項」「組織型と診断の実際」「鑑別ポイント」「臨床との連携」について，本邦では数少ないこれら内分泌疾患の病理組織診断および臨床の専門家が記載して，病理医がこれらの疾患の病理組織診断に遭遇した際における臨床側からのminimum requirementに応えられるようにした．<br><br>　平成29年3月<br><br>　笹野　公伸<br>　亀山　香織</section><section class="indexes">神経内分泌腫瘍neuroendocrine neoplasm（NEN）－総論－<br>　１．神経内分泌腫瘍とは？─その定義─<br>　２．神経内分泌腫瘍の命名の変遷<br>　３．神経内分泌腫瘍の概念─Karzinoidの誕生─<br>　４．Karzinoidは良性腫瘍なのか？─混乱，混乱，混乱の歴史─<br>　５．神経内分泌分化の同定方法の変遷<br>　６．神経内分泌腫瘍の臨床/生物学的悪性度の概念の変遷<br>　７．“Karzinoid/carcinoid”から“NET”“NEN”へ<br>　８．神経内分泌腫瘍の新しい病理学的分類の提唱へ<br>　９．GEP-NETのWHO2010分類<br>　10．GEP-NETのWHO2010分類の問題点<br>　11．神経内分泌腫瘍でのKi67標識率検討の長所と問題点<br>　12．NEC/NET G3は果たして同じ疾患群であるのかどうか？<br>　13．呼吸器原発の神経内分泌腫瘍の病理分類WHO2015─膵消化管原発の神経内分泌腫瘍との差異─<br>　14．WHO2017分類─P-NETにおける新しい分類─<br>　15．その他<br>１．消化管NET<br>第１部　検鏡前の確認事項 <br>　１．臨床情報の確認<br>　２．消化管NET の肉眼所見<br>　３．切り出し，標本作製の注意点<br>　４．本邦における消化管NET の傾向と特性<br>第２部　組織型の診断と実際 <br>　１．NETの組織像と特性<br>　２．低分化型NEC の組織像と特性<br>　３．WHO2010分類 <br>　４．WHO分類の次期改訂へ向けての動き<br>　５．MANECの組織像と特性<br>　６．NETのソマトスタチン受容体について<br>　７．TNM分類<br>第３部　鑑別ポイント<br>　１．食道<br>　２．胃<br>　３．十二指腸・Vater 乳頭NET<br>　４．空腸・回腸NET<br>　５．虫垂NET<br>　６．直腸NET，およびその他の大腸に発生するNET<br>第４部　臨床との連携 <br>　１．GI-NET G1/G2に対する治療戦略<br>　２．NECに対する治療戦略<br>　３．MANECに対する治療戦略<br>２．膵NET<br>第１部　検鏡前の確認事項 <br>　I．確認事項<br>　　１．WHO分類（グレード判定，TNM分類）<br>　　２．グレード判定<br>　　３．ホルモン産生腫瘍<br>　　４．遺伝性腫瘍症候群<br>　　５．遺伝子異常<br>　　６．診断に必要な免疫染色<br>　　７．レポート作成<br>　II．病理標本の取り扱い方<br>　　１．生検，細胞診標本<br>　　２．手術検体の取り扱い<br>第２部　組織型と診断の実際 <br>　I．神経内分泌腫瘍neuroendocrine tumor（NET）<br>　　１．定義・概念<br>　　２．臨床的事項<br>　　３．肉眼所見<br>　　４．組織学的所見<br>　　５．組織亜型<br>　　６．免疫組織化学的特徴<br>　　７．生検組織診・細胞診<br>　　８．電顕所見<br>　　９．非機能性・機能性腫瘍の特徴的な病理所見<br>　　10．遺伝性腫瘍症候群に合併する膵NETの特徴的な病理所見<br>　　11．悪性度に関わる因子<br>　II．NEC<br>　　１．定義・概念<br>　　２．臨床的事項<br>　　３．肉眼像<br>　　４．組織像<br>　　５．免疫組織化学的特徴<br>　　６．先行病変（組織発生）<br>　III．mixed adeno-neuroendocrine carcinoma（MANEC）<br>　　１．定義・概念<br>　　２．mixed ductal-neuroendocrine carcinoma<br>　　３．mixed acinar-neuroendocrine carcinoma<br>　IV．過形成性，前腫瘍性病変<br>　　１．nesidioblastosis<br>第３部　鑑別ポイント <br>　１．高分化神経内分泌腫瘍neuroendocrine tumor（NET）と組織学的類似性を示すSPN，腺房細胞癌との鑑別<br>　２．NETの亜型と鑑別疾患<br>　３．NETと膵管癌との鑑別<br>　４．臨床画像所見からのNETと鑑別疾患<br>　５．低分化神経内分泌癌neuroendocrine carcinoma（NEC）の鑑別疾患<br>　６．MANECと混同されやすい病態の鑑別（除外）診断<br>　７．原発部位の同定<br>第４部　臨床との連携<br>　１．画像診断と病理の連携<br>　２．EUS-FNAと病理の連携<br>３．肺・呼吸器NET<br>第１部　検鏡前の確認事項<br>　１．本項で扱う腫瘍<br>　２．全身および肺における神経内分泌腫瘍<br>　３．カルチノイドと神経内分泌癌の発生部位と症状<br>　４．カルチノイドと神経内分泌癌の性質の違い─混合型の存在，起源細胞，予後─<br>第２部　組織型と診断の実際 <br>　１．定義・概念<br>　２．WHO分類<br>　３．カルチノイド<br>　４．小細胞癌<br>　５．大細胞神経内分泌癌<br>　６．びまん性特発性肺神経内分泌細胞過形成<br>第３部　鑑別ポイント<br>　１．定型カルチノイドと異型カルチノイド<br>　２．異型カルチノイドと大細胞神経内分泌癌<br>　３．小細胞癌と大細胞神経内分泌癌<br>　４．小細胞癌と類基底細胞型扁平上皮癌<br>　５．紡錘細胞カルチノイドと小細胞癌<br>　６．小細胞癌とEwing 腫<br>第４部　臨床との連携 <br>　１．小細胞肺癌（SCLC）<br>　２．大細胞神経内分泌癌（LCNEC）<br>　３．カルチノイド <br>　４．症例提示<br>４．下垂体<br>第１部　検鏡前の確認事項<br>　１．下垂体とは<br>　２．下垂体腫瘍の分類<br>　３．下垂体腫瘍の免疫染色<br>　４．下垂体腺腫の悪性度<br>　５．その他の診断に必要な項目<br>第２部　組織型と診断の実際<br>　１．下垂体腺腫の概念<br>　２．GH-PRL-TSH産生腺腫：Pit-1系腺腫<br>　３．ACTH産生腺腫：Tpit系腺腫<br>　４．ゴナドトロピン産生腺腫：SF-1系腺腫<br>　５．特殊な腺腫<br>　６．その他の腫瘍の病理分類～囊胞性腫瘍と紡錘形細胞腫瘍<br>第３部　鑑別ポイント <br>　１．下垂体腺腫の血管構築と構造<br>　２．下垂体腺腫細胞の細胞質<br>　３．免疫染色の判定<br>第４部　臨床との連携<br>　１．鑑別診断<br>　２．術中迅速診断<br>　３．臨床に有用な下垂体腺腫の病理診断<br>　４．予後判定<br>　５．機能性腺腫・難治性下垂体腺腫における薬物感受性の予測<br>５．副甲状腺<br>第１部　検鏡前の確認事項 <br>　１．副甲状腺の発生<br>　２．副甲状腺の解剖・組織<br>第２部　組織型と診断の実際<br>　１．異所性副甲状腺<br>　２．パラサイロマトーシスparathyromatosis<br>　３．副甲状腺囊胞<br>　４．副甲状腺腺腫<br>　５．副甲状腺過形成<br>　６．家族性副甲状腺機能亢進症<br>　７．副甲状腺癌<br>　８．異型腺腫<br>第３部　鑑別ポイント<br>　１．腺腫か過形成か<br>　２．異所性副甲状腺腺腫か甲状腺腫瘍か<br>　３．囊胞の鑑別<br>第４部　臨床との連携<br>　１．原発性副甲状腺機能亢進症<br>　２．腎不全による続発性副甲状腺機能亢進症<br>６．副腎皮質<br>第１部　検鏡前の確認事項 <br>　１．病理医が病理組織診断の前に知っておくべき患者の診断前内分泌所見<br>　２．病理診断医が副腎皮質疾患の病理組織診断を行う際に臨床側に確認しておくべき，患者の内分泌所見以外の臨床情報<br>　３．病理診断医が理解しておくべき副腎皮質疾患の画像所見<br>　４．副腎皮質疾患摘出検体の標本提出方法<br>　５．副腎皮質疾患の摘出検体の診断で，通常の病理組織診断以外に何が必要になるのか？ 検体の処理は？<br>第２部　 組織型と診断の実際と鑑別ポイント <br>　I．非腫瘍性病変<br>　　１．組織型と診断<br>　　２．鑑別ポイント<br>　II．良性腫瘍<br>　　１．定義・概念<br>　　２．臨床的事項<br>　　３．肉眼所見<br>　　４．組織学的所見<br>　　５．鑑別上の問題点<br>　III．副腎皮質癌および2次性悪性腫瘍<br>　　１．副腎皮質腫瘍の良悪性の病理組織学的鑑別診断<br>　　２．副腎皮質癌と診断された症例の病理組織学的悪性度の鑑別診断<br>　　３．副腎に認められる悪性腫瘍が副腎皮質由来かどうかの鑑別診断<br>第３部　臨床との連携<br>　１．原発性アルドステロン症<br>　２．顕性副腎性Cushing 症候群<br>　３．副腎皮質癌<br>　４．副腎偶発腫<br>　５．サブクリニカル（不顕性）Cushing 症候群<br>７．副腎髄質腫瘍─パラガングリオーマ─<br>第１部　検鏡前の確認事項<br>第２部　組織型と診断の実際<br>　１．褐色細胞腫/傍神経節腫<br>　２．悪性褐色細胞腫/悪性傍神経節腫─PCC/PGLの悪性度─<br>　３．家族性・遺伝性褐色細胞腫<br>　４．神経芽腫群腫瘍と混成腫瘍<br>第３部　鑑別ポイント<br>第４部　臨床との連携 <br>　１．疫学<br>　２．術前診療のポイント<br>　３．治療<br>索引</section></article>
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