Von Hippel-Lindau病的影像学表现(附3例报告)

目的分析von Hipple-Lindau(VHL)病的影像学表现,进一步提高对本病的认识。方法分析3例家族性VHL病患者的影像学表现及临床病理资料并结合文献复习。结果 3例患者影像学检查均有中枢神经系统血管母细胞瘤及多发胰腺囊肿。患者均诊断肾囊肿及肾癌,2例患者诊断多发肾囊肿及多发肾癌,1例患者诊断单侧肾囊肿及单侧肾癌,同时合并左侧视网膜血管母细胞瘤。结论 VHL病累及全身多个系统,影像学表现多样化。对确诊患者应进行多学科的监测和随访。     

VHL综合征的影像学表现(附1例报道并文献复习)

目的探讨von Hippel-Lindau(VHL)综合征的影像学表现特征,提高对VHL综合征的认识。方法分析1例VHL综合征的临床及影像学资料,并复习相关文献,总结其特征。结果本例病变包括小脑及颈髓多发血管母细胞瘤、视网膜血管母细胞瘤、双肾囊肿、右肾癌、胰腺多发囊肿。结论 VHL综合征累及全身多个脏器,影像学表现多样,超声、CT及MRI在发现其各脏器病变方面具有各自的优势。     

VHL综合征的影像学表现

目的 :探讨VHL综合征的影像学特征,提高对VHL综合征的认识。方法 :回顾性分析7例经临床确诊为VHL综合征的CT及MRI资料,并参考复习相关文献总结其影像表现。结果:7例均出现中枢神经系统(CNS)血管母细胞瘤,6例伴双肾多发囊肿,4例伴双肾多发肾癌,6例伴胰腺多发囊肿,3例伴嗜铬细胞瘤,3例伴胰腺占位性病变,其中1例确诊为实性假乳头状瘤。结论:影像学检查是VHL综合征诊断与随访的主要手段,MRI在CNS病变诊断及随诊中具有显著优势,CT在腹部病变方面具有显著优势。     

以嗜铬细胞瘤为首诊的von Hippel-Lindau综合征患者的临床和分子生物学研究

目的：对1例临床诊断希佩尔-林道（von Hippel-Lindau,VHL）综合征的患者进行临床和分子生物学研究。方法全面收集患者病史资料、实验室检查及影像学资料,采集患者外周血提取DNA并通过PCR扩增产物直接测序明确有无突变。结果此患者血浆去甲肾上腺素及24 h尿去甲肾上腺素水平均明显升高,影像学提示右肾上腺占位,胰腺多发囊肿。有阳性家族史。基因诊断提示VHL基因3号外显子杂合突变。结论对临床上合并VHL综合征特征性临床表现、家族史明确的嗜铬细胞瘤患者行VHL基因检测有助于VHL综合征的诊治。     

颅内包虫病的CT诊断

目的探讨颅内包虫病的CT表现。方法分析经手术、病理证实的6例颅内包虫病的CT征象,其中单发脑内包虫囊肿3例,多发1例,硬膜外包虫囊肿1例,肝脑多发泡型棘球蚴病1例。结果颅内包虫病一般表现为典型囊肿性病变,轮廓清晰、锐利,CT值与脑脊液相似,囊内容物密度均一,囊壁无或轻度增强。脑泡型棘球蚴病无特异CT征象,有时表现酷似脑胶质瘤。结论CT是诊断颅内包虫病简捷、准确的影像学检查方法,密切结合临床资料正确诊断多无困难。     

成人胆总管囊肿合并胆道系统肿瘤的影像诊断

目的：总结成人胆总管囊肿釜并胆道系统肿瘤的影像学表现。方法：回顾分析15例经手术病理证实的成人先天性胆总管囊肿合并胆道系统肿瘤的影像资料，总结其影像学特征。结果：先天性胆总管囊肿合并胆管癌11例，其中5例为肝门部胆管癌，表现由肝门部肿块，具有特征性表现；其中6例为囊肿内合并肿瘤，表现为附壁小结节或乳头样突起。胆总管囊肿合并胆囊癌3例，表现为胆囊腔内软组织肿块或囊壁不规则增早，MRI上信号较结石略高．胆总管囊肿合并胰腺癌1例。15例中，胆总管囊肿同时合并胆道系统肿瘤、结石6例。结论：成人胆总管囊肿较易合并胆道肿瘤，CT、MRI检查时应该提高警惕，MRCP对囊肿显示清晰，准确，结合动态增强，可以提高肿瘤的检出率及定性。     

先天性支气管闭锁的影像学表现

目的分析先天性支气管闭锁（CBA）的影像学表现。资料与方法回顾性分析5例CBA的临床和影像学表现，并结合文献复习。5例均行X线及CT检查，1例行普通X线断层摄影，2例行MRI检查。结果X线胸片示3例肺门区肿块样阴影．1例局部肺野多发结节阴影，1例囊肿周围肺野过度透亮。胸部CT示5例黏液囊肿显示为多发分支状和棍棒状肿块或结节样阴影及周围肺野的过度充气改变。2例显示周围肺野支气管扩张改变。2例胸部MRI示囊肿T1、T2均为高信号。结论黏液囊肿和周围肺野的过度充气是CBA典型表现，CT对CBA的诊断优于其他影像学检查方法。     

淋巴管肌瘤病的胸腹盆部影像学表现

目的探讨淋巴管肌瘤病(LAM)的胸部及腹盆部影像学表现。资料与方法分析12例经病理证实的LAM患者胸部及腹盆部影像学表现。结果胸部CT表现为双肺多发直径约0.5～2 cm的无壁或薄壁的小囊腔,其中10例囊状影弥漫性分布,1例散在分布,1例仅有数个小囊腔;伴有气胸、胸腔积液7例;11例(92%)患者腹盆部出现异常,其中腹膜后多发淋巴管肌瘤4例(2例伴有腹腔及盆腔大量积液,其中1例伴有卵巢畸胎瘤、1例伴有双侧卵巢囊肿)、合并肝肾及盆腔多发血管肌脂瘤1例、合并肾血管肌脂瘤1例;此外7例中,子宫肌瘤2例,肝肾多发血管肌脂瘤伴子宫肌瘤、胆囊息肉伴子宫肌瘤、肝多发血管肌脂瘤伴子宫肌瘤、肝脏囊肿、腹腔积液各1例。结论 LAM是一种多系统的疾病,其胸部及腹盆部病变影像学有一定特征性。     

腮腺少见病变的影像学表现分析

目的:探讨腮腺少见病变的影像学表现,提高对其认识水平。方法:回顾性分析经临床及病理证实的38例腮腺少见病变的CT或MRI资料。结果:嗜酸细胞腺瘤向咽旁间隙生长是其特征性表现;基底细胞腺瘤有双期持续强化特点;肌上皮瘤和肌上皮癌影像表现无特异性;脂肪瘤可见脂肪成分;婴儿毛细血管瘤有血管流空信号;木村病表现为腮腺弥漫性肿大,累及皮肤及皮下组织;表皮样囊肿、皮样囊肿、潴留囊肿病灶内均无强化;钙化性上皮瘤常位于腮腺区外侧皮下,发生部位有一定特点;良性淋巴上皮病变表现为双侧腮腺弥漫性肿大伴多发囊性肿块或小结节;淋巴上皮癌表现为多发囊性肿块时与良性淋巴结上皮病变及转移性鳞癌鉴别困难;腮腺癌2例均为腺泡细胞癌;淋巴瘤常有原发淋巴瘤病史,伴全身多发淋巴结肿大;转移瘤有原发恶性肿瘤病史,影像学表现与原发肿瘤密切相关。结论:部分腮腺少见病变具有一定的影像学特点,有助于鉴别诊断,确诊仍依靠组织病理学和免疫组化。     

重症先天性肺囊性病的CT表现及护理

目的探讨重症先天性肺囊性病患儿的CT表现及护理要点。方法回顾性分析2017年1月～2019年10月收治经手术病理证实的重症先天性肺囊性病患儿17例的相关资料,应用描述性统计方法分析本病的CT特点及护理要点。结果影像学表现病灶呈单发囊肿6例,多发囊肿11例,囊内表现有空洞2例、液性6例、气液性9例,囊壁厚薄不一。单侧肺部一叶或多叶囊肿5例,双侧肺部一叶或多叶囊肿12例,肺部高密度影13例,纵膈向健侧移位6例。行单纯囊肿切除术1例、肺段楔形切除术8例、肺叶切除术8例,术后9～14天均痊愈出院。结论对于先天性肺囊性病的患儿,临床容易误诊,应根据其典型的影像学特征,尽早做出正确诊断与护理。     

关节旁骨内腱鞘囊肿的影像学诊断

目的 探讨关节旁骨内腱鞘囊肿的发病机制、临床表现及X线特征、诊断及鉴别诊断。资料与方法 回顾性分析经病理证实的11例关节旁骨内腱鞘囊肿的临床及影像学资料，其中5例位于膝关节附近，5例位于踝关节附近，1例位于肘关节，且均为单发。结果 11例X线表现：7例为单房性圆形或椭圆形密度减低区，其中2例与关节腔有一宽约1mm的通道；2例呈多房性囊状扩张，其内有粗细不等的间隔；另2例呈半圆形骨质缺损，开口朝向关节腔。11例均显示边界清楚，周围有宽约1mm的硬化边。结论 关节旁骨内腱鞘囊肿在临床和X线片上可早期诊断，但应与相关疾病鉴别，防止误诊。     

Solid variant of aneurysmal bone cysts in long tubular bones: giant cell reparative granuloma

OBJECTIVE: The purpose of this study was to determine the age distribution, location, and imaging features of histologically proven solid variants of aneurysmal bone cysts in long tubular bones. MATERIALS AND METHODS: We performed a retrospective review of imaging studies of histologically proven solid aneurysmal bone cysts in long bones between 1961 and 2001. There were 30 cases comprising 29 radiographic, six CT, and eight MR imaging examinations. The lesions were evaluated for bone involved, location within a long bone, matrix, size, soft-tissue mass, and MR imaging characteristics. The imaging findings were correlated with the histologic findings. RESULTS: The patients were 17 females and 13 males ranging in age from 2 to 58 years (mean, 18 years). The bones involved were the femur (n = 10), the ulna (n = 7), the tibia (n = 7), the humerus (n = 2), the radius (n = 2), and the fibula (n = 2). The lesions were five juxtaarticular, 13 metaphyseal, one diametaphyseal, and 11 diaphyseal. The location was eccentric in 20 cases, of which two were intracortical and two periosteal, and central in 10. Lesion size varied between 1 and 7 cm. Thirty-three percent of lesions were nonaneurysmal. Four lesions were mineralized. A soft-tissue mass was present in four cases. Four lesions showed a permeative-lytic pattern simulating a malignant process. Unusual findings included periosteal reaction and development of a solid aneurysmal bone cyst in a preexisting fracture. MR imaging showed solid elements in all cases and pronounced edema in 50% of cases. CONCLUSION: Solid aneurysmal bone cyst is a reactive nonneoplastic bone lesion with varied imaging characteristics; one third of lesions are nonaneurysmal.     

纵隔囊性病变的CT与MRI诊断

目的：探讨纵隔囊性病变的CT、MRI诊断及鉴别诊断。方法：回顾性总结30例经手术、病理确诊的纵隔囊性病变的CT及MRI表现，包括胸腺囊肿7例，囊性畸胎瘤8例，气管、支气管囊肿9例，食管囊肿4例，囊性淋巴管瘤2例。结果：不同纵隔囊性病变均有其较特定的发病部位。7例胸腺囊肿中6例位于前上纵隔的胸腺区，1例位于前纵隔中下部；8例囊性畸胎瘤主要位于前纵隔中部、大血管起始处；9例气管、支气管囊肿位于中上纵隔，气管、支气管右旁区；4例食管囊肿，其中3例位于后纵隔，1例位于中纵隔；2例囊性淋巴管瘤，其中1例为颈纵隔型，1例为颈一腋纵隔型。结论：纵隔囊性病变虽无明显特征性影像学表现，仔细观察CT与MRI征象特点，结合发病部位，对提高本病的诊断具有重要意义。     

Von Hippel-Lindau disease: strategies in early detection (renal-, adrenal-, pancreatic masses)

Von Hippel-Lindau disease (VHL) is a hereditary syndrome characterized by a predisposition for bilateral and multicentric retinal angiomas, hemangioblastomas in the central nervous system (CNS), renal cell carcinomas, pheochromocytomas, islet cell tumors of the pancreas, and endolymphatic sac tumors, as well as cysts in the kidney, pancreas, and epididymis. This review focuses on developments in imaging of renal, adrenal, and pancreatic masses in VHL. Radiology still has a central place in managing of VHL. Radiologists should therefore be aware of the importances of MRI, CT, and US compared with other radiodiagnostic tools for these three organs. Since a conservative approach to the treatment of VHL lesions is now becoming more widely accepted, ongoing follow-up by careful radiological screening with US, and especially with MRI, will play a central role in managing the disease. We also give an overview of recent advances in the molecular biology of VHL, because the combination of imaging with (presymptomatic) DNA analysis has made early detection and screening of lesions possible and led to a reduction in morbidity and mortality. Received: 31 December 1997; Revision received: 5 May 1998; Accepted: 10 June 1998     

Adrenal cysts: imaging and percutaneous aspiration

Six patients with primary adrenal cysts were encountered during a period of 3 years. Multiple imaging modalities were used to characterize these lesions, including magnetic resonance imaging in one case. Percutaneous aspiration was performed on four of the six cysts - in one case, in the operating room prior to excision of the cyst. Cholesterol was detected in four cysts and cortisol in one cyst. Cytologic findings from aspiration biopsy were benign in all five cases. Two cysts were removed, one of them after reaccumulation of the fluid after needle aspiration. The findings in this small series of adrenal cysts suggest that in certain cases, complete cyst aspiration, rather than surgical excision, may be carried out initially for diagnosis and management of indeterminate suprarenal cystic lesions and symptomatic cysts of the adrenal gland.     

卵巢良性囊性病变的CT、MRI表现

目的 分析卵巢良性囊性病变(BOCL)的CT和MRI表现,提高对其的影像诊断与鉴别诊断水平.方法 回顾分析48例经手术病理证实的BOCL的CT和MRI表现.35例行CT平扫,其中20例行CT增强检查;8例行MRI平扫,其中3例行MRI增强检查及扩散加权成像(DWI)检查;5例同时行CT、MRI检查.结果 囊肿11例(单纯性囊肿3例、黄体囊肿3例、子宫内膜异位囊肿5例),浆液性囊腺瘤10例13个病灶,黏液性囊腺瘤8例,囊性成熟型畸胎瘤9例10个病灶,卵巢甲状腺肿10例.BOCL的大小、形态、囊壁(厚度、有无壁结节)、密度或信号、强化程度和方式等特征有助于其诊断与鉴别诊断.结论 部分BOCL的CT、MRI表现具有一定特点,术前可做出正确诊断,为临床治疗方案的选择及预后判断提供一定的理论依据.     

支气管囊肿的影像诊断

目的探讨支气管囊肿的影像学表现。方法收集我院经手术、病理证实的32例支气管囊肿,对其影像学表现进行回顾性分析。结果纵隔型支气管囊肿4例,均为含液囊肿。肺内型支气管囊肿28例,含气液支气管囊肿22例,含气支气管囊肿6例,直径1~10cm,2例合并有脓胸,4例合并肺发育不全,1例合并有支气管胸膜瘘。支气管囊肿壁薄,呈圆形或类圆形,合并感染者周围有片状浸润影,壁模糊;多发者呈卷发状、蜂窝状或囊状阴影。结论支气管囊肿的影像学检查,首选普通X线,CT扫描可显著提高对复杂病变的显示效果。     

中肾管囊肿的CT与MRI诊断

目的：通过回顾分析手术病理证实的中肾管囊肿的CT或MRI表现,提高中肾管囊肿CT与MRI的影像诊断水平。方法：回顾分析25例手术病理证实的中肾管囊肿的CT或MRI表现,5例行CT平扫加增强,21例行MRI平扫加增强,其中1例同时行CT和MRI平扫加增强。结果：本组病例全部为女性,单发23例,多发2例,位于输卵管系膜内19例,其中双侧2例;子宫正后方1例,腹膜后3例,下腹部2例。CT表现为圆形或卵圆形,低密度薄壁囊性肿块,边界清楚,1例腹膜后中肾管囊肿壁见多发钙化并右肾下垂、旋转不良、右侧输尿管异位;MRI表现为长T1长T2薄壁囊性肿块,1例腹膜后中肾管囊肿后壁可见一小囊;增强扫描,4例位于输卵管系膜内中肾管囊肿表现为囊壁部分弧线状强化,其余病例囊壁及囊内未见强化。结论：中肾管囊肿有其典型的发病部位,结合临床,CT或MRI可以提示中肾管囊肿的诊断,显示钙化方面CT优于MRI,显示病灶囊变结构及与周围结构之间关系,MRI检查优于CT。     

前列腺巨大苗勒管囊肿的影像学表现

目的：探讨发生在前列腺的巨大苗勒管囊肿的影像学表现,提高对该病的认识。方法：回顾性分析4例经手术及病理证实的巨大前列腺苗勒管囊肿的影像学特点。结果：4例肿物均表现为下缘位于前列腺后正中线、向上突向膀胱正后方及两侧精囊之间的囊性肿物,边缘光滑,矢状面或冠状面呈倒置的水滴状（3例）或类圆形（1例）,囊壁均匀（3例）、略不均匀（1例）,囊内为液性成分,2例合并出血,增强扫描囊壁呈轻至中度强化,囊内容物不强化。结论：前列腺巨大苗勒管囊肿影像表现特征为囊肿位于膀胱直肠窝,下缘连于前列腺后正中线,矢状面或冠状面呈倒置的水滴状。     

Ga-68 Somatostatin Receptor PET/CT in von Hippel-Lindau Disease

Von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome with a variety of benign and malignant tumors such as retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renal cysts and tumors, pancreatic cysts and tumors, pheochromocytomas, and epididymal cystadenomas. Cross-sectional modalities (computed tomography and magnetic resonance imaging) as well as ultrasound play a major role in the initial evaluation and follow-up of the various manifestations of VHL disease. Ga-68-labeled somatostatin receptor analogs already have a significant role in the diagnosis, staging, and therapy management of neuroendocrine neoplasms and neural crest tumors. Herein, we report a case presenting a variety of malignancies in VHL and showing the usefulness of Ga-68 somatostatin receptor PET/CT as a one-stop-shop imaging modality in the management of VHL disease.