双肾巨大血管平滑肌脂肪瘤破裂出血1例

患者 男性,59岁.肉眼血尿伴左腰痛2年,突发左腰痛及腹痛,并出现肉眼血尿,伴血块1天.查体:患者神志清楚,腹部隆起,右上腹部可触及一肿物,有明显压痛,大小约30.0cm×18.0cm×15.0cm,上至肋弓下缘,左侧边缘位于前正中线,下至髂嵴,右侧边缘至腋中线;左上腹部可触及另一肿物,大小约10.0cm×9.0cm×8.0cm,上至肋弓下缘,左侧边缘位于腋中线,下至髂嵴,右侧边缘至腋前线;双合诊未触及肾下极,双肾区叩痛,双肋脊角压痛.     

结节性硬化症合并肝肾错构瘤1例

患者男,36岁.因腰痛、血尿1周而入院.患者智力差,癫痫,自儿童期即发现面部和头部长小瘤.查体:面颊部及鼻翼两侧可见多发绿豆样大小的丘疹,粉红色、质硬、互不融合.头颅CT示双侧侧脑室室管膜下见多个大小不等的圆形或不规则性的钙化结节,其中最大钙化结节1.2 cm× 1.2cm.     

典型结节性硬化症一例

病例资料患者,女,28岁,主诉腹部渐进性膨隆一年余,腰痛3个月入院。患者无腹痛、腹胀、恶心呕吐症状,大小便正常,入院前外院超声提示腹盆腔巨大占位,遂转入我院。入院后查体：神志清,发育正常,口鼻三角区多发淡棕色蜡状丘疹,呈蝶翼状分布,触之较硬,无瘙痒、疼痛感;腹部明显膨隆。血尿常规、肝肾功能正常。否认家族中有类似病史。     

结节性硬化症的CT诊断

目的：探讨结节性硬化症（TS）的CT诊断价值。方法：对7例结节性硬化症患者的CT图像及临床资料进行回顾性分析,7例均行头部CT平扫,3例也做了腹部CT平扫,其中1例腹部CT增强。结果：7例患者中有6例以癫痫、智力低下就诊,3例因精神异常就诊。CT均表现为双侧侧脑室室管膜下多发大小不等钙化结节影,5例脑皮层及皮层下钙化结节影,3例伴有双肾多发肾血管平滑肌脂肪瘤（angimyolipoma,AML）,1例伴有胼胝体发育不良（DCC）,1例伴有脾肿大。结论：结节性硬化症具有特征性的临床与CT表现,CT扫描对结节性硬化症的诊断具有很高的价值。     

结节性硬化症的骨骼影像学特征

目的探讨结节性硬化症（TSC）的骨骼影像表现特征。方法回顾性分析13例符合临床确诊标准的TSC影像资料。着重观察其骨骼影像表现。13例均行头颅CT扫描，7例行腹部CT检查。结果CT可见下列3种表现：（1）骨骼多发硬化性小结节13例：病变多发且分布较为均称，呈多发斑点状或小结节状硬化，边界清晰，硬化性小结节灶见于颅骨、骨盆、椎体及长骨骨端的松质区。部分病例呈现枕骨大孔周缘珠环征，颅顶部层面硬化小结节酷似“鳞甲征”。（2）牙质样骨增生或类骨纤维结构不良样改变：类骨纤维结构不良样改变7例，象牙质样椎弓征5例。（3）骨皮质增生改变：2例髂骨皮质对称骨膜新骨增生，形似“皮质双边征”，短管状骨一侧或双侧皮质增厚3例。5例复查病灶未见明显变化。特征性骨骼影像表现为同时出现椎体内多发硬化小结节伴象牙质样椎弓硬化；颅骨内多发硬化小结节伴局部类骨纤样改变等。结论TSC骨骼影像学表现颇具特征性，该表现可列为结节硬化症的临床诊断主要指标之一。     

结节性硬化症并发多器官损害的CT 影像学诊断

目的：探讨结节性硬化症并发多器官损害的CT诊断价值。方法回顾性分析6例结节性硬化症并发多器官损害的CT影像学特征。结果3例可见室管膜下多发钙化结节,1例可见巨细胞星形细胞瘤;5例可见双肾多发血管平滑肌脂肪瘤,1例可见双肾多发囊肿;2例出现肝脏多发错构瘤;2例出现肺淋巴管肌瘤病;1例出现骨骼斑点状硬化。结论 CT能够清晰显示病变的部位、大小、形态,是诊断结节性硬化症并发多器官损害的一种重要的检查方法。     

结节性硬化症的影像学检查

目的探讨结节性硬化症的影像学检查方法，提高放射科医师对本病的认识。方法对1例因外伤就诊的结节性硬化症病人进行了平片、CT检查及详细体检。结果该病人有面部血管纤维瘤、鲨鱼皮样斑、室管膜下结节钙化、皮层结节钙化、双肾错构瘤并左侧出血，确诊为结节性硬化症。结论放射科医师必要时应亲自观察、检查病人，以便得出有利于临床确诊、治疗的诊断和建议。     

结节性硬化症的MRI和CT诊断

目的 探讨结节性硬化症的颅内影像学征象。材料和方法：对5例临床确诊为结节性硬化症的病例进行头颅CT和MRI检查,对其影像学征象进行分析。结果 所有病例均表现为两侧侧脑室室管膜下多发结节状异常信号和高密度,其内多同时有钙化。其余脑实质及皮层下亦可见散在结节状和片状异常信号,合并有钙化。增强扫描,室管膜下结节有轻度增强。结论 MRI和CT检查能全面反映结节性硬化症的颅内病理改变,对于其临床诊断和中枢系统损害程度的评价有意义。     

肾血管脂肪瘤1例

患者男,41岁。发现左肾占位5天入院。外院B超示:左肾区高回声包块。查体:心肺未闻及明显异常,双肾区无叩击痛,无尿频尿急尿痛及肉眼血尿。血尿常规、肝肾功能未见明显异常。CT平扫:左肾可见较大类圆形脂肪密度肿块影,边界清楚,CT值约-115HU,其内见多条血管影。增强:可见左肾肿块内部分血管自左肾发出,脂肪成分未见强     

脑结节性硬化症的影像学征象分析

目的探讨脑结节性硬化症的影像学表现及诊断要点。方法回顾性分析18例脑结节性硬化症的CT与MRI影像学表现。结果所有病例均出现双侧侧脑室室管膜多发结节状钙化和异常信号，4例出现脑实质内异常结节或钙化，2例合并脑室内肿瘤，1例患儿合并脑裂畸形，1例合并肾脏错构瘤。MRI对皮层下非钙化结节的显示明显优于CT。结论CT和MRI能全面反映脑结节性硬化症的颅内病理变化，对临床不典型病例作出诊断，同时可发现合并的其他颅内病变。     

结节性硬化伴巨大肾血管平滑肌脂肪瘤2例

例1：女,36岁。偶发口角抽搐25年,近5年来感腹部包块渐大。查体：面部可见红色丘疹样红斑及扩张毛细血管,神智清醒,对答正常,智力无障碍,左右腹部触及巨大包块,移动性差,边界不清,血压120／70mmHg。实验室检查：血常规正常,尿素氮（BUN）及肌酐（CREA）正常;尿十项检验及定量尿沉渣检验正常。影像学表现：头颅CT增强扫描示左侧侧脑室前角近透明隔处一均匀强化结节,     

巨大肾血管平滑肌脂肪瘤自发性破裂大出血CT误诊4例分析

目的：探讨肾血管平滑肌脂肪瘤破裂出血手术前误诊的原因及CT手术前诊断该病的可能性。方法：复习手术与病理证实的4例术前CT误诊的巨大的肾血管平滑肌脂肪瘤伴破裂出血的病例。结果：巨大的肾血管平滑肌脂肪瘤可发生肿瘤内、肾筋膜囊内,盆腔膜后甚至腹腔内出血;仔细寻找肿块内代表脂肪成分的CT征是支持肾血管平滑肌脂肪瘤诊断的关键。误诊原因可能 与出血掩盖肿块内实际存在的脂肪征有关,也可能与将被挤到肿块的边缘的肿瘤内的脂肪结构误诊为肾筋膜囊内或肾窦内的脂肪有关,结论：巨大的肾血管平滑肌脂肪瘤可发生自发性破裂出血,手术前CT发现肿块内脂肪征是诊断的关键。     

结节性硬化症伴肝脏、双肾、胰腺错构瘤1例

患者男性,25岁。既往体健,否认家族遗传病史。无明显诱因发热,血压145／78mmHg,额部见高出皮面两处皮疹,不红,无痛及瘙痒,各浅表淋巴结未触及,肠鸣音正常,病理反射未引出。辅助检查：血常规WBC17×10^9/L,中性粒细胞81．4％;尿常规（＋＋）,红细胞（＋＋＋＋）;血生化正常。     

介入治疗肾动脉巨大假性动脉瘤1例

患者女,43岁。患者一天前无明显诱因开始出现右侧腰腹部疼痛,呈持续性,伴有阵发性加重的绞痛。无尿频,尿急,尿痛,无肉眼血尿。体检：T37.5℃,BP150／100mmHg。右下腹压痛,轻度反跳痛,腱反射（＋＋）。彩超提示：右肾囊肿。入院后给予低盐饮食,抗炎及抗凝治疗,腹痛缓解不明显,第二天突然出现血尿,全程肉眼血尿,     

Tuberous sclerosis complex: renal imaging findings

PURPOSE: To review the renal imaging findings and changes over time in a large series of young patients with tuberous sclerosis complex (TSC). MATERIALS AND METHODS: One hundred thirty-nine renal imaging studies (113 ultrasonographic scans, 15 computed tomographic scans, and 11 magnetic resonance images) were identified in 59 patients with TSC (mean age, 11.4 years; age range, 3 days to 36 years). There were 31 male and 28 female patients. Angiomyolipomas and cysts were identified and characterized according to the following features: number, unilateral versus bilateral, largest diameter, and change over time. In patients whose initial examination results were normal, the age at onset of lesions was noted. The chi(2) test was used to determine whether cysts and angiomyolipomas occurred in different subpopulations. RESULTS: Angiomyolipomas were identified in 47 (80%) patients and were too numerous to count in 36 (76%), focal in 38 (81%), and bilateral in 42 (89%). The mean largest diameter was 21 mm. Cysts were identified in 28 (47%) patients, 18 (64%) of whom had fewer than five cysts. Cysts were bilateral in 17 (61%) patients. The mean largest diameter was 20 mm. In patients with initially normal examination results, the mean age at presentation was 9.0 years for those with cysts and 9.2 years for those with angiomyolipomas. In 80 follow-up examinations, size and/or number increased in 32 (40%) angiomyolipomas and 21 (26%) cysts. Cysts and angiomyolipomas did not occur in significantly different subpopulations (P =.13). CONCLUSION: Both angiomyolipomas and cysts occur commonly in pediatric patients with TSC and tend to increase in size and number with increasing age. Angiomyolipomas are more common than cysts and tend to be numerous.     

肾血管平滑肌脂肪瘤伴肾静脉、下腔静脉及肺动脉栓塞1例

肾血管平滑肌脂肪瘤(AML)是一种常见的良性间叶源性肿瘤,但合并下腔静脉或肺动脉瘤栓罕见。本文报道了1例肾AML伴肾静脉、下腔静脉和肺动脉栓塞的患者。男,77岁,2020年5月因腹痛发现右肾AML,2020年7月无明显诱因出现胸闷、胸痛,CT、MRI、PET-CT影像学提示右肾占位,右肾静脉、下腔静脉和左肺动脉内多发脂肪密度为主的栓子。患者先后行肺动脉病损切除术和右肾切除术,病理证实右肾病变及肺动脉栓子分别为AML和瘤栓。     

Evaluation of renal angiomyolipoma by traditional and modern imaging in a case of tuberous sclerosis

Renal angiomyolipoma is known to affect mainly patients with tuberous sclerosis, although it can be found in a significant number of otherwise normal individuals. Few cases have been published in which angiomyolipoma and renal cell carcinoma occurred in the same kidney. In such cases the radiologist may be asked if it is possible to distinguish between the two tumors in the same kidney. The present case report describes systemic traditional and modern imaging of huge bilateral angiomyolipoma in tuberous sclerosis. The analysis of the data implies that, although the diagnosis of angiomyolipoma can be established with noninvasive techniques only, it is impossible to distinguish between the two mentioned tumors if they coexist in the same kidney, even if all available imaging techniques are used.     

巨大胃间质肉瘤合并坏死误诊1例

胃间质肉瘤合并坏死临床较少见,易误诊,现报告如下。患者男,43岁。因腹部包块、黑便、呕吐2周入院。患者于3月前出现上腹部饱胀不适,疼痛且进食后加重,伴恶心、呕吐,近2周发现黑便,呈柏油样,并感头昏无力。查体:慢性病容,贫血貌,未见胃肠型及蠕动波,上腹部可扪及约8.0cm×9.0cm的质硬包块,边界不清,表面光滑,活动度差,压痛。实验室检查:血红蛋白3.0g,白细胞8.0×109/L,中性0.7,淋巴0.3,大便潜血实验( )。X线检查:胃体小弯贲门部下方有一向外开口,钡剂由此进入一大小约2.5cm×10.0cm的囊腔内,囊壁僵硬欠规整,食道下端及贲门拉长变形,呈细长…