Castleman病所致的与副肿瘤性天疱疮相关的闭塞性细支气管炎的临床病理与影像

目的 探讨与Castleman病所致的副肿瘤性天疱疮（PNP）相关的闭塞性细支气管炎（BO）的临床，病理与影像学特征。方法 5例患者包括男3例。女2例，平均年龄21岁。4例BO患者经临床诊断，1例经病理证实，所有患者均行吸气相和呼气相X线胸片和高分辨率CT（HRCT）扫描检查。结果 5例患者分别在PNP出现1-8个月后继发BO症状，胸片4例表现为双肺透亮度增加和血管纹理稀疏等过度充气改变，HRCT显示为弥漫性空气潴留征者5例。双肺多个亚段及其远端较大的细支气管柱状扩张4例。小叶核心和支气管管壁增厚4例。纵隔积气3例。片状肺泡实变（磨玻璃影）2例。术后随访5个月至3年均预后不良，呈现不同程度的Ⅱ型呼吸衰竭症状。结论 BO是伴有PNP的Castleman病的少见而严重的合并症，是导致预后不良的主要原因。HRCT上，BO的HRCT最常见表现包括呼气相空气潴留征，支气管扩张和小叶核心或支气管壁增厚。     

原发性肠系膜巨大淋巴结增生症的MSCT表现与病理对照分析

目的 探讨原发性肠系膜巨大淋巴结增生症的多层螺旋CT(MSCT)特征。 方法 回顾性分析经手术及病理证实的8例肠系膜巨大淋巴结增生症患者的MSCT表现, 分析肿块的位置、形态、大小、边界、密度及增强特点。 结果 8例肠系膜巨大淋巴结增生症中, 7例为单发, 1例为多发, 共检出9个病灶, 均为透明血管型。病灶边界均清晰, 呈类圆形或浅分叶, CT平扫均为稍低密度影, 1例病灶中央区见钙化; 8例患者病灶均明显强化, 其中6例的7个病灶均匀强化, 2例不均匀强化。4例的5个病灶表现为一个较大病灶周围伴多个小结节; 8例患者病灶周围或病灶内均见粗大血管影。 结论 MSCT对巨大淋巴结增生症的诊断及鉴别诊断具有重要价值。     

Meningioangiomatosis: clinical, radiologic, and histopathologic correlation

Meningioangiomatosis (MA) is a rare, benign, hamartomatous lesion of the leptomeninges; MA has been considered to be a forme fruste of neurofibromatosis. A review of pathology records for patients with MA who were seen between 1970 and 1989 at the authors' institutions revealed four patients (three male and one female; aged 2.5-21.0 years; mean, 10.8 years) with a history of seizures but without the stigmata or family history of neurofibromatosis. Three patients had undergone either computed tomographic (CT) or magnetic resonance (MR) imaging studies. All patients had undergone craniotomies to obtain tissue for pathologic analysis; a peripheral, leptomeningeal lesion was found in all four patients. At CT in two patients, the lesions were most consistent with calcification. At T2-weighted MR imaging in one patient, the lesion demonstrated a hyperintense periphery with associated edema of the white matter. Histopathologic examination demonstrated characteristic features of MA--cortical meningovascular fibroblastic proliferation and leptomeningeal calcification. The accurate diagnosis of MA is important since MA is a benign, surgically correctable cause of seizures.     

胸腹部局灶型巨淋巴结增生症影像学表现

目的:探讨胸腹部局灶型巨淋巴结增生症(LCD)的影像学表现特征,提高诊断符合率.方法:回顾性分析经手术后病理证实的12例胸腹部LCD患者的影像学表现,其中男3例,女9例,胸、腹部各6例.结果:病理分型为透明血管型11例,浆细胞型1例.10例行CT平扫及增强扫描,胸、腹部各5例,CT平扫示病灶呈等或略低密度,2例密度均匀一致,7例病灶内见裂隙状、小片状非坏死性低密度影,4例病灶内或边缘见簇状、点状、小片状或中央放射状钙化,1例见多发囊变.胸、腹部各有1例伴有纵隔及后腹膜多发小淋巴结,且腹部病例伴有肝脾肿大.另有1例合并副肿瘤性天疱疮;胸部LCD动脉期平均强化幅度90～130HU;腹部LCD动脉期平均强化幅度50～80HU,其中1例静脉期强化程度最高,余腹部病例静脉期及延迟期强化程度呈逐渐下降趋势.2例行MRI平扫及增强扫描,胸、腹部各1例,病灶呈等或稍长T1、长T2信号,T2抑脂序列呈明显高信号,增强后病灶动脉期明显强化,静脉期及延迟期强化程度下降.结论:LCD的影像学表现具有一定的特征性,结合临床症状,可以提示该病可能,确诊依赖于手术和病理.     

Respiratory bronchiolitis-associated interstitial lung disease: radiologic features with clinical and pathologic correlation.

PURPOSE: The purpose of this work was to describe the radiographic and CT findings in patients with respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) and to correlate them with clinical, physiologic, and pathologic features. METHOD: RB-ILD was proved pathologically in all 21 patients. Sixteen (76%) patients were current smokers, and five (24%) patients were ex-smokers. The mean cigarette consumption was 38.7 pack-years. Chest radiographs and CT scans were semiquantitatively analyzed and correlated with clinical findings, physiologic measures, and a pathologic score of disease extent. RESULTS: The major radiographic findings were bronchial wall thickening in 16 patients (76%) and ground-glass opacity in 12 patients (57%). The predominant initial CT findings were central bronchial wall thickening (proximal to subsegmental bronchi) in 19 patients (90%), peripheral bronchial wall thickening (distal to subsegmental bronchi) in 18 patients (86%), centrilobular nodules in 15 patients (71%), and ground-glass opacity in 14 patients (67%), None of these CT findings had a significant zonal predominance. Other findings were upper lung predominant centrilobular emphysema (57%) and patchy areas of hypoattenuation (38%) with a lower lung predominance. Radiologic findings were similar in both current and ex-smokers. The amount of ground-glass opacity correlated inversely with arterial oxygen saturation ( r = -0.67, p = 0.04), and the areas of hypoattenuation correlated with alveolar-arterial oxygen gradient ( r = 0.56, p = 0.04). The extent of centrilobular nodules correlated with the extent of macrophages in respiratory bronchioles ( r = 0.53, p = 0.03) and with chronic inflammation of respiratory bronchioles ( r = 0.57, p = 0.02). The extent of ground-glass opacity correlated with the amount of macrophage accumulation in the alveoli and alveolar ducts ( r = 0.56, p < 0.01 and r = 0.54, p = 0.04, respectively). At follow-up CT after steroid treatment and smoking cessation, in nine patients, the extent of bronchial wall thickening, centrilobular nodules, and ground-glass opacity had decreased, but the areas of hypoattenuation had increased (p < 0.05). CONCLUSION: The CT findings of RB-ILD are centrilobular nodules, ground-glass opacity, and air trapping. These radiologic features, in patients with a history of heavy cigarette smoking, may differentiate RB-ILD from other interstitial lung diseases.     

Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

Objective To characterize the radiological and clinicopathologic features of cystic synovial sarcoma.Design and patients Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed.Results CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma.Conclusion All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging.     

Idiopathic multilocular thymic cyst: CT features with clinical and histopathologic correlation

OBJECTIVE: We performed this study to describe the CT features of idiopathic multilocular thymic cysts with clinical and histopathologic correlation. CONCLUSION: Multilocular thymic cysts typically manifest on CT as unilocular or multilocular cystic thymic masses, often with soft-tissue attenuation components. CT cannot be used to distinguish neoplastic from nonneoplastic soft-tissue components.     

原发性纵隔囊肿的影像学表现

目的分析纵隔囊肿的影像学特点，提高对不同类型囊肿不典型表现的认识和诊断水平。方法回顾性分析经手术病理证实的44例纵隔囊肿的CT和（或）MRI资料（其中28和26例分别行CT和MR检查，10例同时行CT和MR检查），分析其影像学表现。结果囊肿位于前、中、后纵隔者分别为13、18和11例，位于前中纵隔者2例。支气管囊肿和心包囊肿位于不典型部位者分别为7和5例。CT密度近似于水者12例，占42．9％（12／28）。MR T2WI病变均为等或高于脑脊液的长T2信号，1例水成像上信号有衰减。近似于脑脊液的长T1信号者8例，占30．8％（8／26）。MRI信号不均匀者5例，原因各异。误诊14例。结论后纵隔和前纵隔CT高密度和厚壁的囊肿易被误诊为肿瘤性病变；中纵隔气管旁的心包囊肿易被误诊为支气管囊肿。MRI有助于诊断CT高密度的纵隔囊肿。MRI信号不均匀或水成像上信号衰减足误诊的原因。     

Thoracic actinomycosis: CT features with histopathologic correlation

OBJECTIVE: Thoracic actinomycosis is a chronic suppurative pulmonary or endobronchial infection caused by Actinomyces israelii, a gram-positive anaerobic organism. We present the CT features of thoracic actinomycosis with histopathologic correlation. CONCLUSION: The typical CT feature of parenchymal actinomycosis is a chronic segmental air-space consolidation containing necrotic low-attenuation areas with frequent cavity formation. A broncholith can be secondarily infected with Actinomyces organisms, resulting in endobronchial actinomycosis. It usually manifests as a proximal endobronchial calcification associated with distal obstructive pneumonia.     

Severe acute respiratory syndrome: correlation between clinical outcome and radiologic features

PURPOSE: To evaluate whether there is a correlation between the clinical outcomes and radiologic features of severe acute respiratory syndrome (SARS). MATERIALS AND METHODS: The clinical, laboratory, and radiologic features of 138 patients with SARS were analyzed. Three radiologists in consensus retrospectively assessed the frontal chest radiographs obtained at presentation and during treatment (n = 2045) for the distribution (each lung was divided into upper, middle, and lower zones) and extent of lung parenchymal abnormality. Clinical end points included intensive care unit (ICU) admission and death. RESULTS: Thirty-six (26.1%) patients required ICU care, and eight (5.8%) died. The patients who required ICU care and/or died had more extensive consolidation on chest radiographs obtained initially (median percentage of consolidation, 3.30%, with interquartile range [IR] of 1.70%-8.78% vs 1.70% [IR, 0%-3.30%]; P < .001) and on day 7 after fever onset (median percentage of consolidation, 15.00% [IR, 6.48%-28.73%] vs 5.00% [IR, 2.50%-7.50%]; P < .001) than did surviving patients who did not require ICU care. Patients with involvement of more than one lung zone on initial and day 7 chest radiographs were more likely to require ICU care and/or die than were those with involvement of one or fewer zones (P < .001). Patients with bilateral pneumonic changes at presentation were more likely to have an adverse outcome than were those with unilateral pneumonia (P < .001). Involvement of more than one lung zone at baseline chest radiography was an independent predictor of ICU admission and/or death (odds ratio, 3.16; 95% confidence interval: 1.07, 9.32; P = .037) after adjustments for other significant factors (ie, patient age, and baseline neutrophil count and lactate dehydrogenase level). CONCLUSION: More extensive airspace disease at presentation is an independent predictor of adverse outcome in patients with SARS.     

Cervical Castleman disease: CT study with angiographic correlation

The computed tomographic (CT) appearance, angiographic appearance, and clinical features of a patient with cervical Castleman disease, an uncommon disease of benign lymph node hyperplasia, are reported. CT scans showed a densely enhancing cervical mass. On external carotid angiography, the mass was seen as hypervascular with a capillary blush. Differential diagnosis included carotid body chemodectoma, vagal neuroma, tumor of the salivary gland, tuberculous adenitis and other granulomatous diseases, inflammatory lymph nodes, metastatic disease, and lymphoma.     

肝脏原发性神经内分泌肿瘤影像学表现及病理对照

目的 探讨肝脏原发性神经内分泌肿瘤(PHNET)的CT、MRI表现及其病理基础.方法 收集经手术病理证实的PHNET 14例(14例均行CT检查,其中9例行MR检查),分析病灶的CT、MRI表现,探讨形成影像表现的病例基础.结果 PHNET呈单发(8例)或多发结节(5例),弥漫性全肝分布1例.CT平扫呈低密度,1例可见液-液平面病理为内部出血;动脉期呈结节状或环形强化,1例呈弥漫性强化,门静脉期或延迟期强化减低.MRI呈长T1长T2信号,出血呈短T1短T2信号,强化形式与CT相同.随病理分级的提高,病灶由G1单发实性-G2实性或囊实性-G3弥漫性分布或出现肝内转移.扩散加权成像(DWI)呈扩散受限改变.结论 CT、MRI能够显示出PHNET的影像学特殊表现和组织学特征.     

颈部Castleman病影像表现及病理对照

Castleman病(Castleman's disease,CD)又称巨大淋巴结增生症(giant lymph node hyperplasia,GLNH)、血管淋巴滤泡增生症、血管瘤样淋巴结增生症、淋巴样错构瘤等.CD是一种少见的、原因未明的、介于炎症及肿瘤之间的不典型淋巴组织增生性疾病,1954年由Castleman首先描述,并于1956年提出其特征~([1]).     

Caroli's disease: radiologic spectrum with pathologic correlation

OBJECTIVE: The purpose of our study was to describe the spectrum of radiologic and pathologic features of Caroli's disease. CONCLUSION: Caroli's disease and its complications have overlapping radiologic appearances that reflect the underlying pathology of fibrosis, ductal dilatation, cholangitis, stone formation, and malignancy.     

Uncommon adrenal masses: CT and MRI features with histopathologic correlation

Adrenal glands are common sites of diseases. With dramatically increased use of computed tomography (CT) and magnetic resonance (MR) imaging, more and more uncommon adrenal masses have been detected incidentally at abdominal examinations performed for other purposes. In this article, uncommon adrenal masses are classified as cystic masses (endothelial cysts, epithelial cysts, parasitic cysts, and pseudocysts), solid masses (ganglioneuroma, ganglioneuroblastoma, extramedullary plasmacytoma (EMP), neurilemmoma, and lymphoma), fat-containing masses (myelolipoma, teratoma), and infectious masses (tuberculoma), and the imaging features of these uncommon masses are demonstrated. Although most of these lesions do not have specific imaging features, some fat-containing masses and cystic lesions present with characteristic appearances, such as myelolipoma, teratoma, and hydatid. Combination with histopathologic characteristic of these uncommon masses of adrenal gland, radiological features of these lesions on CT and MR imaging can be accurately understood with more confidences. Moreover, CT and MRI are highly accurate in localization of uncommon adrenal masses, and useful to guide surgical treatments.     

Multiple biliary hamartomas: magnetic resonance features with histopathologic correlation

The purpose of this study was to report the magnetic resonance imaging (MRI) features of multiple biliary hamartoma (MBH) and to correlate them with histopathology. MRI features of 11 patients with MBH proven by histology were retrospectively reviewed and correlated to histopathology. MBH presented as multiple, tiny, and uniformly distributed lesions in all cases. All were hypointense on T1-weighted images and hyperintense on T2-weighted images relative to the liver parenchyma. Mural nodules were identified in 10 of 11 (91%) cases. They were isosignal on T1-weighted images, intermediate signal on T2-weighted images. Gadolinium-enhanced images showed mural nodule enhancement in 9 of 10 patients (90%) or a peripheral rim-like enhancement of the whole lesion in one case (9%). MBH were present in all liver specimens. In the six patients examined at MR cholangiography, the lesions lacked communication with the biliary tree. At histopathology, the mural nodule corresponded to an endocystic polypoid projection made of conjunctive septa. Three (27%) patients had associated focal nodular hyperplasia, and 1 (9%) had concomitant cholangiocarcinoma. MRI features allowed diagnosis of MBH with accuracy. Their recognition, especially the mural nodule, may help avoid misdiagnosis.     

Gastrointestinal stromal tumors: radiologic features with pathologic correlation.

Gastrointestinal stromal tumors (GISTs) are unique neoplasms that occur throughout the gastrointestinal tract, mesentery, omentum, and retroperitoneum. They are the most common mesenchymal neoplasm of the gastrointestinal tract and are defined by their expression of KIT (CD117), a tyrosine kinase growth factor receptor. The expression of KIT is important to distinguish GISTs from other mesenchymal neoplasms such as leiomyomas, leiomyosarcomas, schwannomas, and neurofibromas and to determine the appropriateness of KIT-inhibitor therapy. The series described herein was accumulated over 2 years and includes 64 pathologically proved GISTs (28 gastric, 27 small intestinal, six anorectal, one colonic, one esophageal, and one from the small bowel mesentery). Radiologic features of GISTs vary depending on tumor size and organ of origin. Since most GISTs arise within the muscularis propria of the stomach or intestinal wall, they most commonly have an exophytic growth pattern and manifest as dominant masses outside the organ of origin. Dominant intramural and intraluminal masses are less common radiologic manifestations. GISTs occurring in the gastrointestinal tract and mesentery characteristically have hemorrhage, necrosis, or cyst formation that appears as focal areas of low attenuation on computed tomographic images. Although the radiologic features of GISTs are often distinct from those of epithelial tumors, criteria to separate GISTs radiologically from other nonepithelial tumors have not yet been fully developed.     

Intestinal anisakiasis: clinical and radiologic features

We report the clinical and radiologic features of 12 patients with acute intestinal anisakiasis. Diagnosis of anisakiasis was made immunologically by positive antibody to Anisakis larvae and a recent history of raw fish intake. Severe abdominal pain was a major symptom in these patients-half experienced fluid levels indicating ileus on plain x-ray films of the abdomen. Radiologic findings included irregular thickening of the jejunum, ileum, or colon, with mucosal edema and luminal narrowing with dilatation of the proximal intestine. In two cases a thread-like filling defect suggesting a worm was visualized on the x-ray film. These findings were interpreted as anisakiasis. Although ileus developed in some patients, all were treated and cured completely without surgery.