Mehta Bhakti, Mascarenhas Leo, Zhou Shengmei, Wang Larry, Venkatramani Rajkumar
1Division of Hematology/Oncology, Children's Hospital Los Angeles, Los Angeles, California, USA.
Pediatr Hematol Oncol. 2013 Oct;30(7):640-5. doi: 10.3109/08880018.2013.816810. Epub 2013 Aug 29.
Inflammatory myofibroblastic tumor (IMT) is a rare tumor of intermediate malignant potential that can occur anywhere in the body. Surgical resection is the principal treatment. We report on nine children diagnosed with IMT at our institution over a 10-year period. Presenting symptoms were reflective of tumor location. Complete surgical resection was curative. Local recurrence occurred in the presence of involved surgical margins. One patient with metastatic disease achieved long-term remission with chemotherapy alone. Severe inflammatory response and death occurred in one patient. The 3-year event free and overall survivals (OS) were 58 ± 20% and 89 ± 10% respectively.
炎性肌纤维母细胞瘤(IMT)是一种具有中等恶性潜能的罕见肿瘤,可发生于身体的任何部位。手术切除是主要的治疗方法。我们报告了在10年期间于我院诊断为IMT的9例儿童患者。呈现的症状反映了肿瘤的位置。完整的手术切除具有治愈性。手术切缘受累时会发生局部复发。1例转移性疾病患者仅通过化疗获得了长期缓解。1例患者发生了严重的炎症反应并死亡。3年无事件生存率和总生存率(OS)分别为58±20%和89±10%。