초록
염증성 근섬유모세포종에 대한 정립된 치료 방침은 없다. 수술이 완치를 위한 치료로 알려져 있으나 조직 검사상 염증세포의 침윤이 있고 기존에 보고된 증례들을 바탕으로 하여 스테로이드 또는 비스테로이드성 염증약물도 하나의 치료 방침이 될 수 있겠다. 본 증례와 같이 급속히 진행하는 종양의 경우 연부 조직 육종에 준한 anthracycline 기반 항암 화학요법을 시행하는 것이 질병을 조절하고 생존기간의 연장하는 데에 적절한 선택이 될 수 있을 것으로 보이며 이것을 뒷받침하기 위한 추가 연구가 필요하겠다.
An inflammatory myofibroblastic tumor (IMT) is a rare disease entity, and the clinical characteristics range from indolent to aggressive forms. No established management for patients with unresectable or aggressive IMT is available. We report on a 62-year-old patient with aggressive IMT who achieved a durable partial response lasting 12 months after anthracycline-containing cytotoxic chemotherapy without corticosteroids. The patient was admitted for an evaluation of progressive weight loss and lower abdominal pain lasting for 2 weeks. Abdominopelvic computed tomography revealed a 10 cm sized heterogeneous mass in the mesentery that encased the superior mesenteric artery and a liver metastasis. The diagnosis of IMT was confirmed by percutaneous core needle biopsy of the mesenteric mass. Systemic chemotherapy was performed after confirming disease progression during a 1 month observation period. A partial response was obtained after two cycles of chemotherapy. Anthracycline-containing cytotoxic chemotherapy could be a treatment option for patients with aggressive IMT.