Abstract
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.
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.