悪性腫瘍34

【臨床症状】7歳 入浴中に右腕のしこりに気が付く

【問題】画像所見と診断名は?

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➡ MRI画像(造影)
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➡ CT画像
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    ▶答えはこちら
    • MRI画像で尺骨を中心とする比較的境界明瞭な軟部腫瘤を認める
    • T2WIで高信号、T1WIで低信号、造影効果を認める6×4.5cm大の腫瘤で、骨髄にも造影効果がある
    • CT画像でも軟部腫瘤が淡く造影されているのが確認できる
    • 生検を実施し、Ewing肉腫と診断された(病理組織の免疫染色でCD99陽性)
    ※上段左からT2WI、T1WI、T2-FS、下段左からCE冠状断、CE横断像
    • また単純写真では透瞭性骨破壊像と骨膜の肥厚像を認め、FDG-PET画像でも集積を認める
    • 遠隔転移は認めない
    • 化学療法を実施し、切除可能であれば切除する方針となった
    ※ 左から単純写真(非提示)、CT造影、MRI(T2-FS)、FDG-PET(非提示)

    【Ewing肉腫】

    ・Ewing肉腫Familyに分類される小円形細胞腫瘍の一つ

    ・Ewing肉腫Familyとは次の3つを総称したもので、共通の染色体異常が確認されている

    1. Ewing肉腫
    2. primitive neuroectodermal tumor:PNET(未分化外胚葉腫瘍)
    3. Askin腫瘍(胸壁原発のPNET)

    ・5~15歳の男児の長管骨、骨盤骨に好発し、稀に軟部組織にも発生する

    ・疼痛、発熱、WBC増加で発見される事が多い

    ・病期分類は、限局性(所属リンパ節を超えていないもの)と転移性に分けられる

    ・転移性では肺、骨、骨髄に転移しやすく、中枢神経系への転移は少ない

    ・単純X線では、浸透性発育を認め、骨融解像、骨膜変化、反応性骨形成などが見られ、onion skin状の骨膜反応は特徴的

    ・MRI造影では、軟部腫瘤の不均一な造影が確認できる

    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