Metastatic low-grade inflammatory myofibroblastic tumor (IMT) in the central nervous system of a 29-year-old male patient.

Metastatic low-grade inflammatory myofibroblastic tumor (IMT) in the central nervous system of a 29-year-old male patient.
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一名 29 岁男性患者中枢神经系统转移性低度炎性肌纤维母细胞瘤 (IMT)。

DOI:
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发表时间:
2004
影响因子:
1.1
通讯作者:
H. Mehdorn
H. Mehdorn
中科院分区:
医学4区
文献类型:
--
作者:
A. Petridis;R. Hempelmann;H. Hugo;T. Eichmann;H. Mehdorn

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一例肌纤维肉瘤(IMT)的大脑和肺以及脊髓的描述。一名29岁男性患者出现发热(40 ℃)、不适、呕吐、脑膜炎和白细胞增多。电脑断层扫描显示左额叶出血。疑似出血性血管瘤并进行了手术。组织学检查不能明确诊断。在第一次出血完全恢复后8个月,患者右侧出现第二次颅内颞枕部出血,已手术切除。在大脑左侧顶叶白色物质中观察到新病变。怀疑是一个正在生长的海绵状血管瘤,计划切除病变。术前,患者出现咯血和发热。胸部X光显示左下叶肺部病变。在胸部CT中,在左肺下叶发现一个大肿瘤,并在纵隔发现一个囊性结构。此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,左顶叶病变已被切除。估计脑病变是肺IMT的转移。在进一步的临床病史中,患者发生了胸椎的大脊髓转移。本病例报告的肿瘤转移发展是不寻常的。目前对这些肿瘤的治疗包括完全肿瘤切除和进一步的临床控制。然而,由于本病例中某些病变的定位和延伸,完全切除是不可能的。化疗和放疗的作用尚未得到证实。患者因肺部恶化死亡。
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.