PDF(Pubmed)
Abstract：
Paget\'s disease, the leading cause of skull sclerosis, is often under-diagnosed on imaging with tumefactive soft issue extension being mistaken for other intracranial findings. A 71-year-old female with past medical history of hypertension, chronic obstructive pulmonary disease, transient ischemic attack, 7 pack year smoke history, and alcohol abuse experienced an episode of bilateral upper extremity weakness, left arm numbness, left hand clumsiness, and word deficits that resolved within 20 minutes. Head computed tomography showed a right convexity mass measuring 6 mm with slight mass effect on the right cerebral hemisphere but no midline shift. She also had a sclerotic calvarium with focal erosions, periosteal reaction and scalp edema with no evidence of acute infarct, significant stenosis, occlusion, and aneurysm of the major intracranial arteries. Additional magnetic resonance imaging was ordered. The pattern of sclerosis of the right hemicalvarium extending into the left hemicalvarium and areas of abnormal bony texture and enhancement where sclerosis had not occurred suggested this to be the sclerotic phase of Paget\'s Disease. Additionally, the enhancing soft tissues on either side of the right hemicalvarium and overlying the posterior left parietal bone were thought to represent benign tumefactive soft tissue or pseudotumor. Tumefactive lesions often present a differential dilemma that is best resolved through a multi-disciplinary approach with extensive review on clinical and imaging findings. Tumefactive soft tissue extension related to Paget\'s disease of the skull has not been described in the literature and our case study highlights the importance of considering this entity on one\'s differential for patients presenting with an extra-axial lesion.
摘要：
佩吉特病,颅骨硬化的主要原因,通常在影像学检查中被诊断不足，具有复杂性的软问题扩展被误认为是其他颅内发现。一名71岁女性，既往有高血压病史，慢性阻塞性肺疾病,短暂性脑缺血发作,7包年吸烟史，酗酒经历了双侧上肢无力的发作，左臂麻木,左手笨拙，和单词赤字在20分钟内解决。头部计算机断层扫描显示右凸肿块6mm，对右脑半球有轻微的肿块影响，但无中线移位。她还患有硬化性颅骨并伴有局灶性糜烂，骨膜反应和头皮水肿，没有急性梗塞的证据，显著狭窄，遮挡,颅内主要动脉的动脉瘤.订购了其他磁共振成像。右半卵巢的硬化模式延伸到左半卵巢以及未发生硬化的异常骨性和增强区域，表明这是Paget病的硬化期。此外,右半皮质两侧的增强软组织和覆盖左后顶骨的增强软组织被认为代表良性肿瘤软组织或假瘤。结核性病变通常存在不同的困境，最好通过多学科方法解决，并对临床和影像学发现进行广泛审查。文献中尚未描述与Paget颅骨疾病相关的组织学软组织扩张，我们的案例研究强调了在表现为轴外病变的患者中考虑该实体的差异的重要性。
