Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in so...Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in solitary or few, indolent and chronic, lesions of bone or other organs called eosinophilic granulomas. Calvarial LCH is quite rare and an underappreciated differential etiology of skull lesions. We present a most unusual case of a young child with hyperacutely symptomatic langerhans histiocytosis of the skull. Method: A 7-year-old male presented with a history of increasing (progressive) frontal headaches of 8 days duration, unaccompanied by associated nausea, vomiting, or diplopia. His only additional complaint was a hard bump on his forehead. MRI and CT done in the ER identified a right fronto-parietal lesion with associated skull erosion. Nuclear medicine and SPECT studies confirmed an erosive skull lesion without significant metabolic activity. A right frontal craniectomy and excision was performed. Results: A soft, rubbery well-circumcised mass coming from the diploic layer of the skull with involvement of bone was identified. The mass had eroded both the outer and inner table of the skull, and the involved area of the right frontal bone was resected. Intra-operative histo-pathologic analysis of the lesion revealed Langerhans cell histiocytosis without involvement of the dura. The patient experienced no neurological worsening as a result of the resection. He was discharged home in stable condition. Conclusion: LCH lesions of the skull are common findings, however, this focal hyperacute symptomatic presentation is most rare and should not deter us from anticipating an erosive bony tumor and planning timely surgical management.展开更多
目的:探讨颅骨腺泡状软组织肉瘤(Alveolar soft part sarcoma,ASPS)的影像学特征。资料与方法:回顾性分析8例经手术病理证实的颅骨ASPS的CT(n=8)、MRI(n=8)及MR静脉造影(MRV)(n=2)表现。结果:8例ASPS均以推压头皮软组织向外生长为主,向...目的:探讨颅骨腺泡状软组织肉瘤(Alveolar soft part sarcoma,ASPS)的影像学特征。资料与方法:回顾性分析8例经手术病理证实的颅骨ASPS的CT(n=8)、MRI(n=8)及MR静脉造影(MRV)(n=2)表现。结果:8例ASPS均以推压头皮软组织向外生长为主,向内则与硬脑膜分界不清。CT表现:6例原发性APSP呈溶骨性骨质破坏,见不均匀密度肿块,含斑片状残留骨质,边缘示不完整骨性薄壳;2例继发性ASPS呈溶骨性骨质破坏,肿块密度较均匀。MRI表现:6例原发性ASPS外观呈"蘑菇征",呈以等T_1、长T_2、高DWI信号为主的混杂信号,内部可见片状T_2WI高信号(n=6)、点条状低信号(n=4)或簇状血管影(n=2),MR增强示明显不均匀强化,并于肿瘤两侧出现"硬膜尾征";2例继发性ASPS呈卵圆形,呈均匀等T_1、等T_2信号,明显均匀强化;6例原发性ASPS的ADC值平均为2.19×10^(-4)mm^2/s,2例继发性ASPS的ADC值平均为1.76×10^(-4) mm^2/s。MRV表现:上矢状窦受压,肿块内见大量引流静脉。结论:颅骨ASPS具有特征性的影像表现,结合其临床、病理特征,有助于本病的诊断。展开更多
文摘Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in solitary or few, indolent and chronic, lesions of bone or other organs called eosinophilic granulomas. Calvarial LCH is quite rare and an underappreciated differential etiology of skull lesions. We present a most unusual case of a young child with hyperacutely symptomatic langerhans histiocytosis of the skull. Method: A 7-year-old male presented with a history of increasing (progressive) frontal headaches of 8 days duration, unaccompanied by associated nausea, vomiting, or diplopia. His only additional complaint was a hard bump on his forehead. MRI and CT done in the ER identified a right fronto-parietal lesion with associated skull erosion. Nuclear medicine and SPECT studies confirmed an erosive skull lesion without significant metabolic activity. A right frontal craniectomy and excision was performed. Results: A soft, rubbery well-circumcised mass coming from the diploic layer of the skull with involvement of bone was identified. The mass had eroded both the outer and inner table of the skull, and the involved area of the right frontal bone was resected. Intra-operative histo-pathologic analysis of the lesion revealed Langerhans cell histiocytosis without involvement of the dura. The patient experienced no neurological worsening as a result of the resection. He was discharged home in stable condition. Conclusion: LCH lesions of the skull are common findings, however, this focal hyperacute symptomatic presentation is most rare and should not deter us from anticipating an erosive bony tumor and planning timely surgical management.
文摘目的:探讨颅骨腺泡状软组织肉瘤(Alveolar soft part sarcoma,ASPS)的影像学特征。资料与方法:回顾性分析8例经手术病理证实的颅骨ASPS的CT(n=8)、MRI(n=8)及MR静脉造影(MRV)(n=2)表现。结果:8例ASPS均以推压头皮软组织向外生长为主,向内则与硬脑膜分界不清。CT表现:6例原发性APSP呈溶骨性骨质破坏,见不均匀密度肿块,含斑片状残留骨质,边缘示不完整骨性薄壳;2例继发性ASPS呈溶骨性骨质破坏,肿块密度较均匀。MRI表现:6例原发性ASPS外观呈"蘑菇征",呈以等T_1、长T_2、高DWI信号为主的混杂信号,内部可见片状T_2WI高信号(n=6)、点条状低信号(n=4)或簇状血管影(n=2),MR增强示明显不均匀强化,并于肿瘤两侧出现"硬膜尾征";2例继发性ASPS呈卵圆形,呈均匀等T_1、等T_2信号,明显均匀强化;6例原发性ASPS的ADC值平均为2.19×10^(-4)mm^2/s,2例继发性ASPS的ADC值平均为1.76×10^(-4) mm^2/s。MRV表现:上矢状窦受压,肿块内见大量引流静脉。结论:颅骨ASPS具有特征性的影像表现,结合其临床、病理特征,有助于本病的诊断。
