Petridis A K, Hempelmann R G, Hugo H H, Eichmann T, Mehdorn H M
Department of Neurosurgery, University of Kiel, Germany.
Clin Neuropathol. 2004 Jul-Aug;23(4):158-66.
A case of myofibrosarcoma (IMT) of the brain and lung as well as the spinal cord is described. A 29-year-old male patient presented with fever (40 degrees C), malaise, vomitus, meningism and leukocytosis. Computer tomography identified a bleeding in the left frontal lobe. A bleeding angioma was suspected and an operation was performed. The histological examination could not reveal an exact diagnosis. Eight months after complete recovery from the first bleeding, the patient had a second intracranial temporo-occipital bleeding on the right side which has been removed operatively. A new lesion was seen in the left parietal white matter of the brain. A growing cavernoma was suspected and resection of the lesion was planned. Pre-operatively the patient suffered from hemoptysis and fever. The X-ray of the chest showed a pulmonary lesion in the left lower lobe. In the CT of the chest a large tumor in the left lower lobe of the lung and additionally a cystic structure in the mediastinum was seen. The histological examination of this tumor identified an inflammatory myofibroblastic tumor (IMT). The left parietal lesion has been resected after the thoracic operation. The brain lesions were estimated to be metastases of the IMT of the lung. In the further clinical history the patient developed a large spinal cord metastasis of the thoracic spine. The metastatic development of the tumor reported in this case is unusual. The current therapy of these tumors consists of complete tumor resection and further clinical controls. However, due to the localization and the extension of some lesions in the present case, the complete resection has not been possible. There is no proven role of chemotherapy and radiation therapy. The patient died due to the pulmonary deterioration.
本文描述了一例发生于脑、肺及脊髓的炎性肌纤维母细胞瘤（IMT）。一名29岁男性患者出现发热（40摄氏度）、不适、呕吐、脑膜刺激征及白细胞增多。计算机断层扫描发现左额叶出血。怀疑为出血性血管瘤并进行了手术。组织学检查未能明确诊断。在首次出血完全康复8个月后，患者右侧颞枕部再次发生颅内出血并接受了手术切除。在左侧顶叶白质发现一个新病灶。怀疑为生长性海绵状血管瘤并计划切除该病灶。术前患者出现咯血和发热。胸部X线显示左下肺有一个肺部病灶。胸部CT显示左肺下叶有一个大肿瘤，此外在纵隔可见一个囊性结构。该肿瘤的组织学检查确定为炎性肌纤维母细胞瘤（IMT）。胸部手术后切除了左侧顶叶病灶。脑部病灶被认为是肺部IMT的转移灶。在后续临床过程中，患者胸椎出现了一个大的脊髓转移瘤。该病例中肿瘤的转移发展情况不常见。目前这些肿瘤的治疗方法包括完整切除肿瘤并进行进一步临床观察。然而，由于本病例中一些病灶的位置和范围，无法进行完整切除。化疗和放疗尚未被证实有作用。患者因肺部恶化死亡。
