Hajdu‐Cheney syndrome: Report of a family and a short literature review

We report three members of an Armenian family with Hajdu‐Cheney syndrome. The history suggested that five other members of the family were also probably affected. This disorder is important for the radiologist because distinctive radiographic findings make the diagnosis possible before clinical signs and symptoms are fully developed. Additionally, radiographic examination is essential in all patients suspected of Hajdu‐Cheney syndrome for confirmation of the clinical diagnosis. Radiographic examination also detects complications of the syndrome not evident on clinical examination.     

Technologist Assessment of Abnormalities in Mammography

Clinical images must meet the needs of the radiologist in order to serve the patient well. Learning to fully appreciate and assess the range of potentially pathological concerns is an important part of the role of the mammographer within the breast imaging team: being sure of a complete study that serves the purpose for the radiologist goes beyond pure technical appreciation. It now requires some clinical knowledge in order to ensure the area of concern is appropriately imaged, clearly seen and readily characterized by the radiologist.The technologist's role in ensuring appropriate imaging to clarify the extent of potential disease becomes an integral part of the criteria for excellence. Application of our basic analytical skills to our patient history and clinical images should now be an expectation of the mammographic technologist.Assessing and demonstrating clearly an area of concern on our images is a long way from diagnosing disease. What is missed or obscured on our clinical images is not analyzed by the radiologist. Therefore it is essential that we recognize a possible concern and know how to demonstrate it clearly.To recognize what is potentially a concern, it helps to know a little about where and how pathology within the breast develops. There are three basic areas of concern when checking our images, clinical assessment, calcifications and masses.The mammographer within today's modern team oriented department must have the advanced positioning and clinical skills to ensure the area of concern is appropriately imaged, clearly seen and able to be characterized by the radiologist.To properly assess and demonstrate potential problems for our medical partner, the radiologist, we must understand well the three areas of clinical and pathological change that exposes early breast cancer.     

Technologist Assessment of Abnormalities in Mammography

Clinical images must meet the needs of the radiologist in order to serve the patient well. Learning to fully appreciate and assess the range of potentially pathological concerns is an important part of the role of the mammographer within the breast imaging team: being sure of a complete study that serves the purpose for the radiologist goes beyond pure technical appreciation. It now requires some clinical knowledge in order to ensure the area of concern is appropriately imaged, clearly seen and readily characterized by the radiologist.The technologist's role in ensuring appropriate imaging to clarify the extent of potential disease becomes an integral part of the criteria for excellence. Application of our basic analytical skills to our patient history and clinical images should now be an expectation of the mammographic technologist.Assessing and demonstrating clearly an area of concern on our images is a long way from diagnosing disease. What is missed or obscured on our clinical images is not analyzed by the radiologist. Therefore it is essential that we recognize a possible concern and know how to demonstrate it clearly.To recognize what is potentially a concern, it helps to know a little about where and how pathology within the breast develops. There are three basic areas of concern when checking our images, clinical assessment, calcifications and masses.The mammographer within today's modern team oriented department must have the advanced positioning and clinical skills to ensure the area of concern is appropriately imaged, clearly seen and able to be characterized by the radiologist.To properly assess and demonstrate potential problems for our medical partner, the radiologist, we must understand well the three areas of clinical and pathological change that exposes early breast cancer.     

Behçet disease in Emergency Department: a rare case presenting with haemoptysis and massive pulmonary arterial aneurysms

Behçet disease (BD) is a systemic disorder caused by underlying vasculitis of unknown origin. In this paper we present a case of a 26-year-old male patient who was admitted at our Emergency Department with massive haemoptysis due to pulmonary arterial involvement in BD. The discussion of this case helps to remember that BD is the main cause of aneurysm of the pulmonary arteries and a cause of haemoptysis in young patients. Therefore, the radiologist plays a key role in the identification of intrathoracic alterations with chest computed tomography. The knowledge of clinical manifestations and distinctive elements of BD allow an accurate diagnosis and let the patient to be directed towards an appropriate treatment, in order to avoid the onset of life-threatening complications.     

Role of magnetic resonance imaging (MRI) in early cervical cancer

The aim of this study was to prospectively determine if invasive carcinoma of the cervix can be ruled out by negative magnetic resonance image (MRI) findings. Eligible patients were those who were scheduled to undergo either hysterectomy or conization because of invasive or non-invasive lesions of the cervix. T2-weighted and T1-dynamic enhancement images were reviewed by two radiologists who had no information on these patients except for the preoperative diagnosis. Clinical information regarding location of the disease was not given. At least 12 longitudinal sections prepared from the surgically removed cervix were reviewed by a pathologist who was not provided with MRI information. The correlation between MRI and histological findings was analyzed in terms of depth of invasion. Seventy cases were entered and 66 were evaluable. Sensitivities of T2 and dynamic MRI for invasive and non-invasive disease by radiologist 1 were 0.8824 and 0.8235, respectively, and those by radiologist 2 were 0.6296 and 0.7647, respectively. Specificities were 0.8776 and 0.8571, respectively, for radiologist 1 and 0.8974 and 0.8367, respectively, for radiologist 2. When sensitivities and specificities were calculated for prediction of non-invasive plus microinvasive lesions < or = 5 mm vs. invasive lesions > 5 mm, specificities of T2 and dynamic MRI became 1.0000 for both radiologists. Negative findings on T2 and/or dynamic MRI indicated non-invasive or early invasive cervical diseases < or = 5 mm. The dynamic technique provided only limited additional value in the detection of early invasive cervical carcinoma.     

Interventional radiology and image-guided medicine: interventional oncology

As innovative image-guided procedures have been developed, the interventional radiologist has become an integral member of a multidisciplinary approach to treating cancer. Radiologists can now be involved in all facets of care, including diagnosis, treatment, and imaging follow-up. Initial advances in interventional oncology involved transarterial chemoembolization of hepatocellular carcinoma. With early successes, treatment expanded to additional tumor histologies and new treatment modalities. The interventional radiologist now has an arsenal that includes transarterial therapies and percutaneous ablation, as well as adjuvant treatments such as portal vein embolization. The purpose of this review is to detail some of the more widespread and promising treatments the interventional radiologist can offer in the care of cancer patients. We also will address the clinical evidence for the procedures and review important technical considerations.     

The multidisciplinary approach to bone metastases

With recent advances in the management of cancer, the clinical course of patients with metastatic bone disease is more likely to be prolonged and accompanied by morbidity, including severe pain, hypercalcemia, pathologic fracture, and spinal cord and/or nerve root compression. The early identification of patients at higher risk for developing bone metastases enables practitioners to be proactive in their diagnosis and treatment. A multidisciplinary approach that integrates the diagnosis and treatment of the cancer, symptom management, and rehabilitation ensures optimal care. Bisphosphonates can reduce the number of skeletal-related complications, delay the onset of progressive disease in bone, and relieve metastatic bone pain caused by a variety of solid tumors with a resulting enhanced quality of life. The complexity of the clinical problem and the need to involve an array of health-care providers present a logistical and clinical challenge. A strong argument is made for a thematically integrated bone metastases program as part of the primary care of patients with cancer.     

Outcomes following treatment for patients with cranial nerve involvement from nasopharyngeal cancer

Introduction: Patients with locally advanced nasopharyngeal carcinoma (NPC) commonly present with cranial nerve (CN) involvement, which can cause significant morbidity. We aimed to characterise the pattern of involvement and outcomes of these patients, as well as determine if these differed according to the mode of diagnosis. Methods: Patients were included if they had non‐distant metastatic NPC, presented with CN involvement and completed radiotherapy treatment between 2002 and 2008. The clinical response was categorised as complete response, partial response, stable or progressive disease. The radiological response was assessed using the Response Evaluation Criteria in Solid Tumors criteria. The loco‐regional control and disease‐free survival rates were estimated with the Kaplan–Meier method. Results: Forty‐seven patients fulfilled the inclusion criteria. CN lesions were diagnosed on clinical examination in 15% of patients, radiologically in 40% and both clinically and radiologically in 45% of patients. A complete or partial response of the CN lesions was seen clinically in 82% and radiologically in 95% of patients. The 3‐year local relapse free survival was 64.3%, distant metastasis‐free survival was 46.1% and overall survival was 82.8%. There were no differences in outcomes between patients with clinically versus radiologically detected CN lesions. Conclusion: Most of these patients are likely to undergo clinical and/or radiological resolution of the nerve lesions following chemoradiotherapy, but the outcome was not determined by the mode of diagnosis (radiological or clinical).     

Imaging and intervention of retroperitoneal fibrosis

Retroperitoneal fibrosis is a rare condition characterized by the development of fibrous plaques in the retroperitoneal space. The fibrous plaques characteristically arise distal to the bifurcation of the abdominal aorta and progress to encase the iliac vessels distally and are defined by the associated encasement of one or both ureters. Imaging plays an important role in not only establishing the diagnosis, but also in monitoring disease progression. Historically, the radiological diagnosis was made predominantly by intravenous urography and retrograde pyelography. More recently, advances in cross‐sectional imaging with ultrasound and contrast‐enhanced CT have allowed for a more precise diagnosis as well as helping to accurately define the extent of the disease. At our institution, we have found ultra‐fast MRI to also play a useful role in establishing the diagnosis. In particular, magnetic resonance urography using HASTE (half Fourier‐acquired single shot turbo spin‐echo) sequences allow a safe alternative to intravenous urography, particularly in patients with poor renal function. The purpose of this article is to describe the role of the various imaging methods available to the radiologist and to emphasize the important role that the interventional radiologist now plays, not only in obtaining tissue for diagnosis, but also in providing treatment of the disease by percutaneous nephrostomy drainage and subsequent stent placement in select cases.     

Posttransplant lymphoproliferative disorder: A pictorial review

Posttransplant lymphoproliferative disorder (PTLD) is a serious and potentially fatal complication after solid organ and haemopoietic stem cell transplantation. The frequency of PTLD varies with the type of organ transplant but overall it affects 2–10% of all solid organ transplant recipients. Most cases develop within 1 year after the transplant, although occasional cases present 5–10 years later. Posttransplant lymphoproliferative disorder is clinically and pathologically heterogeneous – the majority are of the non–Hodgkin’s lymphoma type, whereas Hodgkin’s lymphoma arising after transplantation is rare. We have retrospectively reviewed patients with a histological diagnosis of PTLD after a solid organ transplant. We present the imaging features and a clinical review of this condition. Early diagnosis of PTLD may alter the management and outcome of the disease. The radiologist can play a vital role in establishing the diagnosis by imaging features supplemented with percutaneous biopsy and also in monitoring the disease response to treatment.     

Selecting patients for cytoreductive nephrectomy in advanced renal cell carcinoma: who and when

Renal cell carcinoma presents with metastatic disease in approximately 30% of patients at the time of diagnosis. Cytoreductive nephrectomy (CN) of the primary tumor in the face of metastatic disease is part of a multimodality approach including systemic therapy that is based on evidence from randomized trials in the cytokine era. Data from the pretargeted therapy era showed that CN had a clear role in metastatic renal cell carcinoma, increasing life expectancy by approximately 6 months. The substantial improvement in outcomes reported for targeted therapy has challenged the previous role of CN. However, despite the absence of data from Phase III trials, available evidence suggests that some patients may benefit substantially from CN in the era of targeted therapy. This review summarizes current arguments for CN and how to best select patients for surgery. Ongoing trials are key in generating evidence towards a personalized approach to debulking nephrectomy.     

Selecting patients for cytoreductive nephrectomy in advanced renal cell carcinoma: who and when

Renal cell carcinoma presents with metastatic disease in approximately 30% of patients at the time of diagnosis. Cytoreductive nephrectomy (CN) of the primary tumor in the face of metastatic disease is part of a multimodality approach including systemic therapy that is based on evidence from randomized trials in the cytokine era. Data from the pretargeted therapy era showed that CN had a clear role in metastatic renal cell carcinoma, increasing life expectancy by approximately 6 months. The substantial improvement in outcomes reported for targeted therapy has challenged the previous role of CN. However, despite the absence of data from Phase III trials, available evidence suggests that some patients may benefit substantially from CN in the era of targeted therapy. This review summarizes current arguments for CN and how to best select patients for surgery. Ongoing trials are key in generating evidence towards a personalized approach to debulking nephrectomy.     

Importance of pulmonary imaging diagnostics in the management of febrile neutropenic patients

Early detection and characterisation of a pulmonary focus is a major goal in febrile neutropenic patients. Thus, an intensive interdisciplinary co-operation between radiologists and haemato-oncologists on a patient basis, as well as on a department basis is essential to develop a differential diagnosis. The radiologist can contribute much to a differential diagnosis if information about the patient's disease, status and medication is made available. On the other hand, the haemato-oncologist needs to understand the opportunities and limitations of imaging techniques to evaluate better the images and results. This article focuses on pneumonia as the most common focus. First, imaging techniques are summarised shortly. Then, the perspectives for imaging techniques beyond early detection of pulmonary foci--exclusion of pneumonia, monitoring, characterisation of infiltrates and guidance for intervention--are reviewed.     

Clinicopathological study of otogenic brain abscess

Chronic suppurative otitis media (CSOM) is one of the commonest disease entities encountered in otolaryngology practice. Due to poor economic conditions poor hygiene lack of education and death of knowledge about the disease and its complications, we frequently encounter patients of CSOM with extracranial and intracranial complications. Among the intracranial complications otogenic brain abscess is one of the dreadest. With the improvement of healthcare and accessibility of the poor people to healthcare system and development of CT scan, MRI as diagnostic tools incidence of otogenic brain abscess is becoming less due to early diagnosis and interventions. The objectives of this study are to assess the incidence of brain abscesses to explore age, sex, variation to study clinical presentations and common pathogens responsible.     

Imaging of acute pancreatitis

Pancreatitis is one of the most complex and clinically challenging of all abdominal disorders. It is classified according to clinical, morphologic and histologic criteria. The primary role of radiologic imaging in patients with suspected pancreatitis is to confirm or exclude the clinical diagnosis of pancreatitis. Second, if possible, the cause of the disease is established with the assessment of disease severity and detection of complications. Imaging can also provide guidance for percutaneous therapy. Sonography in acute pancreatitis is a good screening test in patients with suspected biliary pancreatitis and a mild clinical course. Contrast-enhanced CT is preferred for patients with acute pancreatitis because it can accurately diagnose and stage the disease and the necessary information for percutaneous management is provided. The diagnosis of acute pancreatitis on MRI relies on the presence of morphologic and peripancreatic changes. Pancreatic necrosis and complications of acute pancreatitis such as hemorrhage, pseudocysts or abscesses are well-examined by MRI.     

儿童颅内皮样囊肿合并感染1例并文献复习

目的:探讨颅内皮样囊肿的临床表现、诊断、治疗及并发症。方法:回顾性分析我院1例颅内皮样囊肿合并感染患儿的临床资料,并结合文献复习,总结其临床表现、诊断及治疗。结果:本例患儿出现了囊内感染,经手术切除后患儿预后良好。结论:儿童颅内皮样囊肿发病率低,一般预后良好,可出现囊内感染、无菌性脑膜炎、脑积水等并发症,核磁共振在诊断中起重要作用,手术是其主要治疗方式。     

Recent progress in the diagnosis and therapy for veno-occlusive disease of the liver

Veno-occlusive disease (VOD) is one of the severe complications of the liver, which may occur after hematopoietic stem cell transplantation (HSCT). Although an early diagnosis is important to initiate antithrombotic therapy before serious organ failure, the widely used clinical criteria only become clinically fulfilled at an advanced stage of disease. Liver biopsy provides useful findings for the diagnosis of VOD, however, in the later or less severe stages of VOD liver biopsy may provide false-negative sampling error because the biopsy sample may be too small to evaluate the whole liver. In addition it may be difficult to follow the clinical course with repeat biopsy in individual cases. Imaging diagnosis of VOD including gray-scale US, Doppler US, and MRI have been reported as convenient and useful. Color-Doppler US is superior because of its specificity and sensitivity. Blood sampling tests including factor VII, protein C, N-terminal propeptide for type III procollagen (P-III-P) and hyarulonic acid have predictive value, and their measurement may simply be another way to evaluate early hepatic impairment. Since no optimal treatment for VOD has been established as yet, the prophylaxis of VOD or early initiation of treatment is important. These new diagnostic approaches for VOD may provide a direction to resolve the clinical problems of VOD such as the time of initiation of therapy, the therapeutic regimen of choice, and the cessation of therapy.     

Vascular events associated with alpha interferon therapy

Alpha Interferon (IFN) is a biological agent used for the therapy of an increasing number of diseases, either as an established effective therapeutic tool or in the context of clinical trials. The use of IFN may be complicated by serious adverse reactions. We describe here the clinical course of a variety of vasculopathic complications in association with IFN-therapy in 12 patients with the diagnosis of chronic myeloid leukemia and 1 patient with malignant melanoma treated at our institute. Vascular manifestations in these patients include Raynaud's phenomena, digital ulcerations and gangrene, pulmonary vasculitis, pulmonary hypertension and thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). These reactions occurred after 3 months to 3 years of 3-10 million units (MU) daily IFN therapy. Concomitant administration of hydroxyurea (HU) was noted in 5 patients. Discontinuation of IFN and initiation of immunosuppressive therapy brought about a complete resolution or arrested progression of these reactions. IFN-therapy may be complicated by severe vasculopathic/vasospastic complications that usually improve after its discontinuation. Possible underlying mechanisms for these complications are discussed. The early diagnosis of these complications may be vital and IFN should be immediately discontinued when early signs of these complications become evident.     

Imaging of small bowel tumors

Small bowel tumors represent less than 25% of all gastrointestinal tumors and 1-2% of malignant tumors in general. However for their nonspecific clinical presentation, diagnosis is often late, because of the patient delay to contact the doctor and especially the doctor difficulty to advance the clinical suspicion and request the suitable clinicoinstrumental diagnostic tests. The radiologist plays a major role in early diagnosis, treatment and prognosis. In the last decade diagnostic imaging (US, CT, MRI) has supported conventional barium studies: the diagnostic accuracy has been enhanced in terms of identification, characterization and evaluation of the degree of severity of these diseases. The choice of the most suitable technique should be based on the diagnostic skills acquired in the field and on the awareness of the limits and indications of each method. In this article, the contribution of imaging to the diagnosis of small bowel tumors, is analyzed.     

超声造影灌注时相分析在监控肝硬化结节演变和恶变中的临床价值

背景与目的：原发性肝癌多在慢性肝炎、肝硬化的基础上发生,一般认为,肝硬化结节（cirrhotic nodule,CN）、异型增生结节（dysplastic nodule,DN）等一系列过程,最终演变为肝细胞癌（hepatocellular carcinoma,HCC）。应用实时超声造影对肝硬化患者进行定期跟踪监测,探讨超声造影在CN演变和HCC早期诊断中的价值。方法：在2007年3月—2017年12月于复旦大学附属中山医院就诊的9 550例慢性乙肝患者中,选取定期超声随访的392例患者的551枚结节作为研究对象,以病理学检查及影像学诊断为标准,分析不同性质结节在超声造影灌注时相的增强表现特点。结果：肝内551枚结节中确诊微小HCC（≤2 cm）55枚、DN 19枚和CN 477枚。常规超声显示,肝内CN、DN和HCC均以低回声多见,分别占61.6%（294/477）、78.9%（15/19）和67.3%（37/55）。超声造影显示,CN的造影特征以等-等-等的增强方式为主,占89.5%（427/477）;DN的造影特征以低-等-等的增强方式最多见,占52.6%（10/19）;微小HCC的造影特征则以高-等-等和高-等-低的增强方式最多见,占85.5%（47/55）。超声造影诊断CN、DN和HCC的准确率分别为93.3%、78.9%和85.5%。结论：超声造影灌注时相分析可客观地反映肝内CN、DN及微小HCC的不同灌注模式,应用超声造影定期监测肝硬化的结节,可及时预警和早期诊断微小HCC,改善患者预后。