Computed tomography of xanthogranulomatous pyelonephritis

The computed tomography findings in two cases of xanthogranulomatous pyelonephritis are presented. There was diffuse involvement of the kidney in one case, with correlation with the pathologic specimen and microscopic findings. In the second case there was focal involvement of the kidney. Computed tomography greatly assisted in making a correct preoperative diagnosis and in assessing the degree of extrarenal involvement.     

Chronic granulomatous disease of childhood. Report of two cases with unusual involvement of the gastric antrum and spleen

Chronic granulomatous disease (CGD) of childhood is a rare entity. The disease is characterized by recurrent infections with granuloma and abscess formation caused by an inherited defective neutrophil leukocyte function. The most common sites of involvement are the lungs, lymph nodes, skin, liver, spleen and bones. Rarely are other organs affected. Two children with CGD are presented. The children were cousins, the older with bone, lung and splenic involvement. The younger had circumferential thickening of the gastric antrum. Some of the lesions were well delineated with ultrasonography. The unusual gastric antrum wall and focal splenic involvement in this disease are emphasized.     

Ultrasound of extranodal abdominal lymphoma--a review

Ultrasound is a valuable method for the detection of abdominal lymph node involvement in malignant lymphoma. In this review the characteristic patterns of organ involvement with lymphoma are described, the features compared with other pathologies and the appearances correlated with histological findings.     

AIDS合并播散性结核的影像学表现

目的探讨获得性免疫缺陷综合征（AIDS）合并播散性结核的影像学表现，提高对该病的认识。资料与方法回顾性分析19例AIDS合并播散性结核患者的影像学资料。结果19例中，8例累及2个不相邻部位，4例累及2个以上不相邻部位；肺部受累17例，胸膜受累4例，纵隔淋巴结受累3例，颈部淋巴结受累2例，腹部淋巴结受累3例，胰腺受累1例，腰椎受累1例。结论AIDS合并播散性结核可累及多个器官、系统，以肺部最常见，其次为淋巴结、胸膜等部位，其影像学表现较无免疫功能损害者所患结核复杂且不典型。     

Primary mediastinal echinococcosis

Computed tomography findings with surgical details are presented in a case of hydatid cyst of posterior mediastinum, a rare site of involvement in echinococcosis infestation. The computed tomography appearance of posterior mediastinal echinococcosis is quite characteristic and can be readily distinguished from other cystic lesions of the mediastinum. Computed tomography provides additional information regarding the involvement of neighboring structures. In this case, computed tomography detected involvement of the descending aorta.     

The xeroradiographic appearances of some uncommon malignant mammary neoplasms

The clinical and xeroradiographic appearances are described of four patients with malignant lymphomatous involvement of the breast, two with breast involvement by acute leukaemia, and four with primary fibrosarcoma of the breast. In one patient with malignant lymhoma and multiple palpable masses, the radiological appearance resembled benign mammary dysplasia, but in the three other patients, one with a discrete clinical mass, and two with diffuse clinical involvement, radiological examination of the breast showed skin thickening with diffuse abnormalities of the trabecular pattern, most marked in the subdermal area. One of the patients with leukaemia had a discrete palpable mass and the other had diffuse clinical involvement, but the radiological appearances in both patients were similar and resembled those of a carcinoma. The clinical and radiological appearance of the patients with fibrosarcoma were those of a benign cyst or a fibroadenoma.     

Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients

Langerhans cell histiocytosis (LCH) is an uncommon disorder of unknown etiology with a wide spectrum of biological behavior. The most common sites of involvement are bone, skin, and lung. While osseous LCH can result in secondary soft tissue involvement, primary soft tissue LCH in extremities with secondary bone involvement is rare, and little is known about its natural course. We report two adult patients with multi-organ system LCH who had primary soft tissue lesions with secondary involvement of adjoining bones in lower extremities, and illustrate the important role played by various imaging modalities in detection and monitoring of treatment response. We also document spontaneous clinical resolution of LCH lesions without therapy, and positive response of these lesions to chemotherapy.     

Hepatic Kaposi's sarcoma and AIDS. Ultrasonographic and x-ray computed tomographic aspects

AIDS-related Kaposi sarcoma is most often multicentric and extensive. Hepatic involvement is unusual and asymptomatic. An anicteric cholestasis may exist. Ultrasonography shows a pedicular echogenic infiltration and a heterogeneous parenchyma with small hyperechoic nodules. On CT, these hypodense lesions are related to the involvement of the hepatic pedicle. This is linked to angiosarcomatous tumorous tissue infiltration of the liver evolving along portal branches. In a patient suffering from cutaneous or digestive Kaposi sarcoma lesions, these radiological aspects are suggestive of hepatic involvement.     

Cytomegalovirus esophagitis and gastritis in AIDS

The radiographic features of cytomegalovirus involvement of the upper gastrointestinal tract in four homosexual men with acquired immunodeficiency syndrome (AIDS) are described. Esophageal involvement (three cases) was demonstrated as a localized ulceration or as a more diffuse esophagitis affecting predominantly the distal esophagus. Gastric involvement (two cases) presented as large nodular rugal folds in the fundus or as a circumferentially narrowed antrum deformed by numerous large nodular contour defects. Endoscopy revealed mucosal erosions, serpiginous ulcers, and inflammatory exudate; biopsies documented the presence of typical inclusion bodies in the epithelial and endothelial cells. Although the radiographic findings are nonspecific, the diagnosis of cytomegalovirus esophagitis and gastritis should be suspected when similar abnormalities are present in immunocompromised patients, particularly homosexual men with AIDS.     

Diffuse skeletal angiomatosis

Diffuse skeletal angiomatosis is a rare disease that is usually associated with visceral angiomatosis. The bony changes are striking and the roentgen examination is the most important diagnostic measure. The lesions are almost invariably lytic and are usually well-defined. The most commonly involved bones are the femur, the pelvis, ribs, humerus, the skull, and vertebrae. The pattern of bony involvement is reviewed and the types of involvement are illustrated including a unique example with extensive changes in the bones of the hand.     

CNS involvement in neuro-Beh?et syndrome: an MR study.

BACKGROUND AND PURPOSE: Beh?et disease (BD) is a multisystem vasculitis of unknown origin in which neurologic involvement has been reported in the range of 5% to 10% in large series. Reports on clinical and radiologic aspects of neuro-Beh?et syndrome (NBS) are in general limited in number. Our purpose was to determine the MR patterns in patients with NBS who had neural parenchymal involvement and to correlate our findings with possible vascular pathophysiology. METHODS: The MR images of 65 patients with NBS and neural parenchymal involvement were reviewed. In a subgroup of patients who had serial MR studies, we evaluated the anatomic-radiologic location and distribution of the lesions and whether they corresponded to any vascular territory, and studied their extension, enhancement patterns, and temporal course. RESULTS: The most common imaging finding in NBS patients who had neural parenchymal involvement was a mesodiencephalic junction lesion with edema extending along certain long tracts in the brain stem and diencephalon in 46% of the patients. The next most common location of involvement was the pontobulbar region, seen in 40% of the cases. Three primary cervical spinal cord lesions and one case of isolated optic nerve involvement were observed. CONCLUSION: The parenchymal distribution of lesions in NBS appears to support the hypothesis of small-vessel vasculitis; mainly, venular involvement. The anatomic distribution of intraaxial veins of the CNS explains the predominant involvement of the brain stem structures observed in our patients. This pattern of lesion distribution might help to differentiate NBS from other vasculitides as well as from the inflammatory-demyelinating diseases of the CNS, such as multiple sclerosis.     

儿童线粒体脑肌病的MRI表现

目的回顾性研究20例线粒体脑肌病患儿(年龄10个月到14岁)的MRI表现.方法20例由肌肉活检及实验室检查证实为线粒体脑肌病的患儿,脑内均有MRI阳性表现,研究其MRI表现的类型.结果20例患儿脑内病灶均表现为T1WI低、T2WI高信号,8例有不同程度的脑萎缩.18例患儿主要为灰质受累,其中10例为只有深部灰质病变;4例为深部灰质和大脑皮质同时受累.4例为深部灰质病变合并脑梗塞而同时累及灰质和白质.2例主要为白质受累,表现侧脑室后角旁白质异常信号.结论儿童线粒体脑肌病的MRI表现是多样性的.当儿童脑MRI表现为灰质(尤其是深部灰质)异常信号、灰质萎缩、不典型梗塞、不典型白质病变且合并临床难以解释的神经、肌肉等多系统的症状时,应考虑到线粒体肌病的可能.     

Alimentary tract lesions in Cowden's disease

Cowden's disease was first recognized by its striking dermatological manifestations and subsequently by the increased incidence of neoplastic involvement of the thyroid and breast. Two cases of Cowden's disease with gastrointestinal polyps are presented to illustrate the alimentary tract involvement in this syndrome, gastrointestinal tract involvement with polyposis being seen in at least 50% of cases. As opposed to the more serious potential of neoplasia in the thyroid and breast, involvement of the alimentary tract has negligible clinical impact to the patient.     

Pulmonary manifestations in rheumatic systemic diseases

Collagen based vascular diseases with pulmonary involvement comprise rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis and polymyositis, ankylosing spondylitis, Sj?gren's syndrome, and mixed connective tissue diseases. The different characteristics of pulmonary involvement are described. In such circumstances, early recognition of lung involvement is of considerable significance and the relationship to the corresponding disease has to be made. Frequently unrecognised pulmonary involvement ends up as fibrosis with irreversible deficits in respiratory function.     

Globoid cell leukodystrophy: distinguishing early-onset from late-onset disease using a brain MR imaging scoring method

BACKGROUND AND PURPOSE: Our purpose was to determine the characteristic MR features of early-onset (before age 2 years) versus late-onset (after age 2 years) globoid cell leukodystrophy (GLD). METHODS: Thirty-four brain MR images in 22 patients with GLD were reviewed. A severity score (0 to 32), based on a point system derived from the location and extent of disease and the presence of focal and/or global atrophy, was calculated for each examination. RESULTS: Of the 22 patients, three were asymptomatic and 19 were symptomatic. Ten patients had early-onset disease, whereas nine had late-onset disease. MR images of all patients showed abnormalities. In the early-onset group (n = 10; mean maximum MR score, 8.1; range, 3-18), 90% had pyramidal tract involvement, 80% had cerebellar white matter involvement, 70% had deep gray matter involvement, 60% had posterior corpus callosal involvement, 50% had parietooccipital white matter involvement, and 40% had cerebral atrophy. Serial MR imaging in four of these patients revealed progressive disease. In the late-onset group (n = 9; mean maximum MR score, 5.6; range, 4-10), 100% had pyramidal tract involvement, 100% had parietooccipital white matter involvement, 89% had posterior corpus callosal involvement, and none had cerebellar white matter involvement, deep gray matter involvement, or cerebral atrophy. Serial MR imaging in one patient with late-onset GLD did not reveal any change. A spectrum of findings was observed in the three patients who were asymptomatic. CONCLUSION: Cerebellar white matter and deep gray matter involvement are present only in early-onset GLD. Pyramidal tract involvement is a characteristic finding in both early- and late-onset GLD. This scoring method for brain MR observations will assist in the objective assessment of the impact of hematopoietic stem cell transplantation in patients with GLD.     

Imaging of abdominal lymphoma

This article discusses the radiologic appearances of solid organ involvement with Hodgkin and non-Hodgkin lymphoma in the abdominopelvic region. The most common radiologic patterns of involvement are illustrated. The imaging characteristics of lymphomatous involvement of abdominal organs overlap with several other disorders and the specific features pertaining to lymphoma are highlighted. In patients who have known lymphomas, other important management considerations, such as staging, response to therapy, malignant transformation, and identification of recurrent disease, are also discussed. The emerging role of fluorodeoxyglucose positron emission tomography is briefly outlined.     

Adenocarcinoma of the lung: a tumor with a changing pleomorphic character

Adenocarcinoma is widely accepted to occur as a solitary, peripheral, subpleural, pulmonary mass with infrequent hilar and rare mediastinal involvement. The older literature indicates that hilar involvement occurs in 18% of cases and that mediastinal involvement occurs in only 2% of cases. A retrospective study of 100 randomly selected cases of primary adenocarcinoma of the lung diagnosed between 1976 and 1982 indicates that hilar masses are present in 40% of cases and that mediastinal masses are present in 27% of cases. In all, hilar and mediastinal masses occurred alone or in combination so that 51% of the patients in this series had plain radiographic evidence of such involvement. Since the patients in this series were studied by conventional radiographic techniques in a manner similar to patients in previous reports, it appears that adenocarcinoma of the lung may present with a much more pleomorphic radiographic appearance than was previously recognized. The reasons for this change in the radiographic appearance of adenocarcinoma are not completely understood but include a change in the histopathologic criteria for the diagnosis of adenocarcinoma and an increase in the incidence of adenocarcinoma, particularly of the poorly differentiated form.     

Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation

Wegener granulomatosis is an uncommon necrotizing vasculitis that classically manifests as a clinical triad consisting of upper and lower airway involvement and glomerulonephritis. Other less frequently involved organ systems include the central and peripheral nervous system and large joints. The diagnosis is based on a combination of clinical and laboratory findings. Because thoracic involvement often predominates, chest radiographic findings are often the first to suggest the diagnosis. However, chest computed tomography (CT) has superior sensitivity and specificity for evaluation of the airways, lung parenchyma, and mediastinum, particularly with the use of multiplanar reformatted and three-dimensional images. Common pulmonary radiologic findings include waxing and waning nodules, masses, ground-glass opacities, and consolidation. Airway involvement is usually characterized by circumferential tracheobronchial thickening, which can be smooth or nodular. Pleural effusions are the most common manifestation of pleural disease and can result from primary involvement or be secondary to renal failure. Mediastinal lymphadenopathy is a nonspecific finding and is usually reactive. Uncommon thoracic radiologic manifestations include involvement of the heart and great vessels. CT is the imaging modality of choice for diagnosis, surveillance, and follow-up in patients with Wegener granulomatosis.     

A spectrum of renal tubular ectasia and hepatic fibrosis.

Renal tubular cystic disease and hepatic fibrosis exhibit a specific genetic pattern and pathological findings. The renal collecting tubules are dilated and the liver shows fibrosis with proliferation and dilatation of the bile ducts. The findings fall into a spectrum with marked renal disease and mild liver involvement at one end and mild renal involvement with severe liver disease at the other. Between these extremes lies an intermediate form which is genetically and pathologically similar, but exhibits a wide range of clinical and radiological findings due to the variability of renal and liver involvement.     

Eosinophilic disorders of the gastrointestinal tract: imaging features

Eosinophilic disorders of the gastrointestinal tract are increasingly rare but remain an important cause of long-standing gastrointestinal symptoms. Diagnosis is usually delayed because the disease mimics other inflammatory disorders and is often not suspected initially. We report a series of four cases to highlight the various imaging appearances of this condition. Two patients presented with upper gastrointestinal involvement, one patient presented with small and large bowel involvement, and one patient presented with diffuse involvement of the entire gastrointestinal tract.