惡性間質細胞瘤為存活率低的惡性疾病,病史詢問及早期診斷為預後的重要因子。影像學及病理學上有其重要的特色。分期部分包含影像分期及手術分期。治療方面以化學治療為主,手術治療及放射線治療已局部性侵犯腫瘤為主要選擇,但仍需要佐以化學治療。整體預後大體來說不甚理想,臨床上可用European Organisation for Research and Treatment of Cancer (EORTC)與the Cancer and Leukemia Group B (CALGB)兩種指標來做評估。
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