1 横浜市立大学附属病院小児循環器科Department of Pediatric Cardiology, Yokohama City University School of Medicine ◇ Kanagawa, Japan
2 横浜市立大学附属病院心臓血管外科Cardiovascular Surgery, Yokohama City University School of Medicine ◇ Kanagawa, Japan
心臓原発の炎症性筋線維芽細胞腫（inflammatory myofibroblastic tumor: IMT）は極めてまれであり，臨床像もよく分かっていない．我々は嘔吐を主訴に受診した3か月の女児で，心エコー検査で右房内を占拠する巨大腫瘤と多量の心嚢液貯留が確認され，病理診断の結果より炎症性筋線維芽細胞腫と確定診断した症例を経験したので報告する．心タンポナーデへの進行が危惧されたため，緊急で腫瘍摘出術を施行した．本症例では血清IL-6値の上昇も認め，粘液腫と類似の臨床像を呈していた．IMTは悪性化の可能性も指摘されているため，右房内腫瘤の鑑別としてIMTも考慮する必要があり，その診断には病理所見が重要である．
Cardiac inflammatory myofibroblastic tumors are very rare, and the clinical features are not well understood. We present the case of a 3-month-old girl with a confirmed pathological diagnosis of cardiac inflammatory myofibroblastic tumor. She was initially brought to hospital due to vomiting. She exhibited elevated serum IL-6 levels and other clinical symptoms suggestive of myxoma. Echocardiogram confirmed the presence of a large tumor of the right atrium and abundant pericardial effusion. We were concerned about the possibility of cardiac tamponade and considered the case to be urgent; we therefore recommended surgical removal of the tumor. This patient is also at risk for malignant transformation or recurrence of the cardiac inflammatory myofibroblastic tumor and needs a long-term follow-up.
Key words: inflammatory myofibroblastic tumor; IL-6; pericardial effusion
© 2016 特定非営利活動法人日本小児循環器学会© 2016 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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This page was last modified on 2016-07-26T14:32:45.844+09:00
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