整形８

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※初回から4か月後の画像　脂肪抑制、T2WI、T1WIの順に表示

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※脂肪抑制、T1WIの横断像、脂肪抑制の矢状断の順に表示

• 初回の検査時には右大腿背部に6*3*1㎝大の腫瘤を認め、T2WIや脂肪抑制画像で中～高信号、T1WIで低信号を認め、信号強度からはまずは神経鞘腫＋血種を疑う
• 4か月後の画像では、6*６*３cmと増大している
• その後の精査で悪性神経鞘腫と診断される
• 他、右大腿骨近位骨幹部に2cm大の骨嚢胞あり

【神経鞘腫/神経線維種】

・神経原性腫瘍は抹消神経（神経線維、神経鞘）由来と神経節および傍神経節に由来するものがある

・末梢神経由来の腫瘍は次の2つがある

1. 神経鞘腫：比較的大きな神経の神経鞘内に発生、被膜を持ち、細胞成分の多いAntoni A領域と細胞成分の少ないAntoni B領域から構成される
2. 単発、孤発が多いが、NF1（von Recklinghausen）病に合併する事は稀だが、NF2では多発する傾向がある
3. 神経線維種：皮膚や皮下などの小さな神経から発生し、紡錘形に発育し被膜を持たない事が多い
4. 腫瘍内に神経を含有する
5. 孤発性と多発性があり、後者は神経線維腫症（neurofibromatosis）と呼ばれる
6. 形態的には限局性と特殊型があり、特殊型には神経に沿って数珠状の形態や、びまん性のケースがある
7. 組織分類には、神経鞘腫（schwannoma）、神経線維種（neurofibroma）、悪性神経鞘腫（maligrant schwannoma）（いずれも神経線維/神経鞘由来）と、神経芽腫（neuroblastoma）、神経節神経芽細胞腫（ganglioneuroblastoma）、神経節細胞腫（ganglioneuroma）（いずれも神経節由来）がある
8. 傍神経節由来には組織分類に傍神経性腫瘍（paraganglioma）がある

・画像所見は、Target appearanceが有名で、神経鞘腫、神経線維種の両方に認められる事があると言われている

・その他には被膜の有無なども鑑別材料になる事がある

・びまん性の神経線維腫では、脂肪抑制画像で低～高信号で、やや不均一、造影効果は一般的に強い傾向がある

参考書籍：ジェネラリストを目指す人のための画像診断パワフルガイド 第2版