Rathinam Sridhar, Kuntz Heinke, Panting Jonathan, Kalkat Maninder S
Regional Department of Thoracic Surgery, Birmingham Heartlands Hospital, Birmingham, UK.
Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):443-5. doi: 10.1510/icvts.2009.221945. Epub 2009 Dec 29.
Inflammatory myofibroblastic tumour (IMT) or inflammatory pseudotumour is a histologically distinctive lesion occurring primarily in the viscera and soft tissue of children and young adults. We report an unusual case of IMT which had undergone malignant transformation in the chest wall at the pacemaker site. A 64-year-old male presented with a history of high fever, loss of appetite and weight loss of three months duration. He had a dual chamber pacemaker reinserted in the left infraclavicular region in the previous year. This was followed by a gradually enlarging hard swelling at the insertion site. The CT-scan showed a soft tissue mass encasing the pacing box, without intrathoracic extension. The trucut biopsy was suspicious of soft tissue sarcoma. A well encapsulated hard mass, with pacemaker embedded within it was resected en-bloc ensuring wide resection margins. Histology revealed fascicles of spindle cell proliferation with prominent inflammatory component, occasional spindle cells with prominent nucleoli and scattered atypical mitotic figures, with areas of focal necrosis. The lesional cells were negative for CD21, smooth muscle actin, ckit, cytokeratins and anaplastic lymphoma kinase 1. A diagnosis of IMT with malignant transformation i.e. inflammatory fibrosarcoma was made. He had adjuvant radiotherapy and uneventful recovery.
炎性肌纤维母细胞瘤(IMT)或炎性假瘤是一种组织学上独特的病变,主要发生于儿童和青年的内脏及软组织。我们报告一例罕见的IMT病例,该病例在胸壁起搏器部位发生了恶性转化。一名64岁男性,有持续三个月的高热、食欲不振和体重减轻病史。他于前一年在左锁骨下区域重新植入了双腔起搏器。随后,植入部位出现逐渐增大的硬性肿块。CT扫描显示一个软组织肿块包绕起搏器盒,无胸腔内延伸。粗针活检怀疑为软组织肉瘤。完整切除一个包膜完整的硬性肿块,其中嵌入起搏器,确保手术切缘足够宽。组织学显示梭形细胞呈束状增生,伴有显著的炎症成分,偶见有明显核仁的梭形细胞和散在的非典型有丝分裂象,并有局灶性坏死区域。病变细胞CD21、平滑肌肌动蛋白、细胞角蛋白、细胞角蛋白和间变性淋巴瘤激酶1均为阴性。诊断为发生恶性转化的IMT,即炎性纤维肉瘤,并接受了辅助放疗,恢复顺利。