中枢神经系统表面铁沉积症(superficial siderosis of the central nervous system, SSCNS)是由于慢性反复性出血进入脑脊液而导致过多的铁沉积在脑干、小脑、脊髓乃至部分脑神经表面所致的神经系统损害,较为罕见,肿瘤是出血源之一。目...中枢神经系统表面铁沉积症(superficial siderosis of the central nervous system, SSCNS)是由于慢性反复性出血进入脑脊液而导致过多的铁沉积在脑干、小脑、脊髓乃至部分脑神经表面所致的神经系统损害,较为罕见,肿瘤是出血源之一。目前国内在其诊治方面存在以下不足:认识较晚,诊断病例较少,长期随访资料匮乏,病因诊断不够重视,漏诊率较高,治疗水平较国外存在较大差距。SSCNS典型临床表现为:双侧进行性神经性耳聋、小脑共济失调和锥体束征阳性,称为SSCNS三联征,但同时具备三联征的患者较少,给及时诊断带来一定困难。MRI对确诊该病有重要意义,铁沉积在小脑、脑干、脊髓等表面呈特征性线状低信号,SE-T 2WI呈细线条,GE-T 2WI及磁敏感加权成像序列(susceptibility weighted imaging,SWI)呈显著粗线条。唯一有效的治疗方法是确定出血源并清除,MRI检查同样有助于寻找出血源,因此,如果考虑SSCNS,必须进行脑、脊柱MRI检查以寻找病因。本文报道1例37岁男性SSCNS患者,临床主要表现为共济失调、神经性耳聋和嗅觉减退,MRI表现有典型的中枢神经系统表面T2加权低信号,病因追查发现脊髓下端及马尾、终丝部巨大占位病变,经手术切除并组织病理学检查提示黏液乳头型室管膜瘤(myxopapillary ependymoma,MPE)。结合文献复习探讨了MPE继发SSCNS的机制,并对SSCNS的病因、临床表现、诊断以及治疗进行综述,以提高临床医师对该病的认识及诊断水平,尤其强调对该病病因的查找有利于治疗方案的准确制定。展开更多
We present morphological findings observed from the study of a myxopapillary ependymoma located in the III ventricle. This tumor, which is a rare occurrence at this level, was observed in a 73-year-old woman with no r...We present morphological findings observed from the study of a myxopapillary ependymoma located in the III ventricle. This tumor, which is a rare occurrence at this level, was observed in a 73-year-old woman with no relevant medical history who presented with obstructive hydrocephalus due to compression and blockage of the foramen of Monro. The good delineation of the lesion allowed its surgical excision, and histological examination of the material obtained after excisional biopsy revealed a glial neoplasm with a low degree of cytological malignancy that was strongly positive for glial fibrillary acidic protein (GFAP) as well as a myxopapillary architecture analogous to that observed in ependymomas of the filum terminale. Subsequent radiological examination allowed us to rule out a metastatic nature of the tumor, confirming its primary origin. We briefly discuss the case and review the main characteristics of this neoplasia, which is quite rare at the site identified in our patient.展开更多
BACKGROUND Myxopapillary ependymomas are rare spinal tumours.Although histologically benign,they have a tendency for local recurrence.CASE SUMMARY We describe a patient suffering from extra-and intradural myxopapillar...BACKGROUND Myxopapillary ependymomas are rare spinal tumours.Although histologically benign,they have a tendency for local recurrence.CASE SUMMARY We describe a patient suffering from extra-and intradural myxopapillary ependymoma with perisacral spreading.He was treated with subtotal resection and postoperative radiation therapy.After treatment,he experienced slight sphincter disorders and lumboischialgic pain with no motor or sensory disturbances.Eight months later,a tumour regression was documented.The patient is still followed-up regularly.CONCLUSION Lumbar myxopapillary ependymomas may present with lumbar or radicular pain,similar to more trivial lesions.Magnetic resonance imaging(MRI)is the primary modality for diagnosis.The treatment aim is to minimize both tumour and therapy-related morbidity and to involve different treatment modalities.展开更多
Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of pae...Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of paediatric brain tumors, and up to 33% of brain tumors in children under the age of three. Hydrocephalus is one of the complications, and it can be identified as progressive macrocephaly or increasing head circumference crossing percentiles, nausea, vomiting, poor appetite, irritability, and regression of developmental milestones.展开更多
文摘中枢神经系统表面铁沉积症(superficial siderosis of the central nervous system, SSCNS)是由于慢性反复性出血进入脑脊液而导致过多的铁沉积在脑干、小脑、脊髓乃至部分脑神经表面所致的神经系统损害,较为罕见,肿瘤是出血源之一。目前国内在其诊治方面存在以下不足:认识较晚,诊断病例较少,长期随访资料匮乏,病因诊断不够重视,漏诊率较高,治疗水平较国外存在较大差距。SSCNS典型临床表现为:双侧进行性神经性耳聋、小脑共济失调和锥体束征阳性,称为SSCNS三联征,但同时具备三联征的患者较少,给及时诊断带来一定困难。MRI对确诊该病有重要意义,铁沉积在小脑、脑干、脊髓等表面呈特征性线状低信号,SE-T 2WI呈细线条,GE-T 2WI及磁敏感加权成像序列(susceptibility weighted imaging,SWI)呈显著粗线条。唯一有效的治疗方法是确定出血源并清除,MRI检查同样有助于寻找出血源,因此,如果考虑SSCNS,必须进行脑、脊柱MRI检查以寻找病因。本文报道1例37岁男性SSCNS患者,临床主要表现为共济失调、神经性耳聋和嗅觉减退,MRI表现有典型的中枢神经系统表面T2加权低信号,病因追查发现脊髓下端及马尾、终丝部巨大占位病变,经手术切除并组织病理学检查提示黏液乳头型室管膜瘤(myxopapillary ependymoma,MPE)。结合文献复习探讨了MPE继发SSCNS的机制,并对SSCNS的病因、临床表现、诊断以及治疗进行综述,以提高临床医师对该病的认识及诊断水平,尤其强调对该病病因的查找有利于治疗方案的准确制定。
文摘We present morphological findings observed from the study of a myxopapillary ependymoma located in the III ventricle. This tumor, which is a rare occurrence at this level, was observed in a 73-year-old woman with no relevant medical history who presented with obstructive hydrocephalus due to compression and blockage of the foramen of Monro. The good delineation of the lesion allowed its surgical excision, and histological examination of the material obtained after excisional biopsy revealed a glial neoplasm with a low degree of cytological malignancy that was strongly positive for glial fibrillary acidic protein (GFAP) as well as a myxopapillary architecture analogous to that observed in ependymomas of the filum terminale. Subsequent radiological examination allowed us to rule out a metastatic nature of the tumor, confirming its primary origin. We briefly discuss the case and review the main characteristics of this neoplasia, which is quite rare at the site identified in our patient.
文摘BACKGROUND Myxopapillary ependymomas are rare spinal tumours.Although histologically benign,they have a tendency for local recurrence.CASE SUMMARY We describe a patient suffering from extra-and intradural myxopapillary ependymoma with perisacral spreading.He was treated with subtotal resection and postoperative radiation therapy.After treatment,he experienced slight sphincter disorders and lumboischialgic pain with no motor or sensory disturbances.Eight months later,a tumour regression was documented.The patient is still followed-up regularly.CONCLUSION Lumbar myxopapillary ependymomas may present with lumbar or radicular pain,similar to more trivial lesions.Magnetic resonance imaging(MRI)is the primary modality for diagnosis.The treatment aim is to minimize both tumour and therapy-related morbidity and to involve different treatment modalities.
文摘Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of paediatric brain tumors, and up to 33% of brain tumors in children under the age of three. Hydrocephalus is one of the complications, and it can be identified as progressive macrocephaly or increasing head circumference crossing percentiles, nausea, vomiting, poor appetite, irritability, and regression of developmental milestones.
