Aletta Ann Frazier

Primary pulmonary and mediastinal synovial sarcoma: a clinicopathologic study of 60 cases and comparison with five prior series

Primary pulmonary and mediastinal synovial sarcoma is rare and poses a diagnostic challenge particularly when unusual histological features are present. We present 60 cases of primary pulmonary and mediastinal synovial sarcoma (29 male and 27 female subjects; mean age, 42 years) and compare our results with five prior series to better define unusual histological features. Clinically, patients with mediastinal synovial sarcoma were younger with a male gender bias. Radiologically, tumors were well delineated with distinctive magnetic resonance imaging features and little vascular enhancement. In all, 21/46 patients died of disease within 5 years. Histologically, all tumors had dense cellularity, interlacing fascicles, hyalinized stroma, and mast cell influx. Hemangiopericytoma-like vasculature (48/60), focal myxoid change (30/60), and entrapped pneumocytes (23/60) were seen. Calcification was not prevalent (10/60). Unusual histological features included Verocay body-like formations (7/60), vague rosettes (6/60), well-formed papillary structures (3/60), adenomatoid change (3/60), and rhabdoid morphology (2/60). Immunohistochemistry demonstrated expression of pancytokeratin (39/58), epithelial membrane antigen (29/53), cytokeratin 7 (26/40), cytokeratin 5/6 (5/7), calretinin (15/23), CD99 (19/23), bcl-2 (24/24), CD56 (11/11), S-100 (9/51), and smooth muscle actin (8/32). In total, 92% (36/39) of primary pulmonary and mediastinal synovial sarcomas studied were positive for t(x;18). In conclusion, our study confirms the clinical, histological, immunohistochemical, and molecular data from previous large series of primary pulmonary and mediastinal synovial sarcoma. Compared with soft tissue synovial sarcoma, primary pulmonary and mediastinal synovial sarcoma has less calcification, less obvious mast cell influx, and less radiologic vascularity, but similar magnetic resonance imaging features, percentage of poorly differentiated tumors, and number of t(x;18)-positive tumors. Awareness of focal unusual histology can prevent misdiagnosis particularly in t(x;18)-negative tumors.

The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: Proposal for an Evidence-Based Stage Classification System for the Forthcoming (8th) Edition of the TNM Classification of Malignant Tumors

A universal and consistent stage classification system, which describes the anatomic extent of a cancer, provides a foundation for communication and collaboration. Thymic epithelial malignancies have seen little progress, in part because of the lack of an official system. The International Association for the Study of Lung Cancer and the International Thymic Malignancies Interest Group assembled a large retrospective database, a multispecialty international committee and carried out extensive analysis to develop proposals for the 8th edition of the stage classification manuals. This tumor, node, metastasis (TNM)-based system is applicable to all types of thymic epithelial malignancies. This article summarizes the proposed definitions of the T, N, and M components and describes how these are combined into stage groups. This represents a major step forward for thymic malignancies.

CT Angiography of the Cardiac Valves: Normal, Diseased, and Postoperative Appearances

Although echocardiography remains the principal imaging technique for assessment of the cardiac valves, contrast material-enhanced electrocardiographically gated computed tomographic (CT) angiography is proving to be an increasingly valuable complementary modality in this setting. CT angiography allows excellent visualization of the morphologic features and function of the normal valves, as well as of a wide range of valve diseases, including congenital and acquired diseases, infectious endocarditis, and complications of valve replacement. The number, thickness, and opening and closing of the valve leaflets, as well as the presence of valve calcification, can be directly observed. CT angiography also permits simultaneous assessment of the valves and coronary arteries, which may prove valuable in presurgical planning. Unlike echocardiography and magnetic resonance imaging, however, CT angiography requires ionizing radiation and does not provide a direct measure of the valvular pressure gradient. Nevertheless, with further development of related imaging techniques, CT angiography can be expected to play an increasingly important role in the evaluation of the cardiac valves. Supplemental material available at http://radiographics.rsna.org/cgi/content/full/29/5/1393/DC1.

Collaborative Radiologic and Histopathologic Assessment of Fibrotic Lung Disease

The idiopathic interstitial pneumonias (IIPs) are a seemingly disconnected collection of diseases usually associated with the presence of pulmonary fibrosis. Categorization of the IIPs continues to be problematic despite recent attempts to refine the diagnostic criteria and suggests that rather than separate diseases, these pneumonias represent a spectrum of injury and abnormal repair of the alveolar wall. Although the initiating injury or injuries are unknown, the IIPs share a restricted number of final common abnormal pathways that lead to volume loss and lung distortion. The pathways include (a) alveolar collapse, (b) incorporation of fibroblastic material into alveolar walls, and (c) cigarette smoke-related inflammation and fibrosis. A collaborative diagnostic process in which data from radiologic and histologic assessments are combined allows a more reliable identification of the predominant pathways leading to pulmonary fibrosis. This approach has implications for therapy and the future direction of research.

From the Radiologic Pathology Archives: Cardiac Lymphoma: Radiologic-Pathologic Correlation

Lymphoma of the heart and pericardium is usually present as one aspect of disseminated disease and rarely occurs as a primary malignancy. It accounts for 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. Cardiac lymphomas are most commonly diffuse large cell lymphomas and frequently manifest as an ill-defined, infiltrative mass. Atrial location is typical; the right atrium is most often affected. Pericardial thickening or effusion is often a common early feature of disease. Infiltration of atrial or ventricular walls with extension along epicardial surfaces is also a notable feature. At computed tomography, the attenuation of cardiac lymphoma may be similar to or lower than that of normal myocardium. At magnetic resonance imaging, it has variable signal intensity and contrast enhancement. Clinical manifestations may include pericardial effusion, cardiac arrhythmias, and a variety of nonspecific electrocardiographic abnormalities, notably first- to third-degree atrioventricular block. Treatment most commonly includes anthracycline-based chemotherapy and anti-CD20 treatment. Chemotherapy has been used alone or combined with radiation therapy. Palliative surgery has been performed, mainly for tumor debulking. The prognosis for patients with either primary or secondary lymphomatous heart involvement is usually poor; late diagnosis is one of the major factors affecting outcome.

Imaging of Whole-Organ Pancreas Transplants

Whole pancreas transplantation is an established treatment for selected patients with diabetic nephropathy or poorly controlled diabetes. Surgical techniques vary and have evolved over the past 4 decades. Imaging evaluation of the whole-pancreas transplant should begin with an understanding of the most commonly used surgical techniques and the spectrum of postoperative complications. Ultrasonography (US) should be the first-line modality in evaluating the pancreas allograft and vasculature. Computed tomography (CT) is useful in the assessment of extra-allograft processes, particularly in ruling out abscess formation or evaluating suspected bowel complications. Magnetic resonance (MR) imaging is reserved for cases in which complete evaluation with US or CT is not possible. MR angiography can help provide an accurate assessment of vascular abnormalities. The radiologist must be familiar with the spectrum of surgical techniques and the normal postoperative imaging appearances of the whole-pancreas transplant so as to be able to recognize abnormal postoperative findings. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.322115144/-/DC1.

Aortic Arch Dissection: A Controversy of Classification

Aortic dissections originating in the ascending aorta and descending aorta have been classified as type A and type B dissections, respectively. However, dissections with intimal flap extension into the aortic arch between the innominate and left subclavian arteries are not accounted for adequately in the widely used Stanford classification. This gap has been the subject of controversy in the medical and surgical literature, and there is a tendency among many radiologists to categorize such arch dissections as type A lesions, thus making them an indication for surgery. However, the radiologic perspective is not supported by either standard dissection classification or current clinical management. In this special report, the origin of dissection classification and its evolution into current radiologic interpretation and surgical practice are reviewed. The cause for the widespread misconception about classification and treatment algorithms is identified. Institutional review board approval and waiver of informed consent were obtained as part of this HIPAA-compliant retrospective study to assess all aortic dissection studies performed at the University of Maryland Medical Center, Baltimore between 2010 and 2012 to determine the prevalence of arch dissections. Finally, a unified classification system that reconciles imaging interpretation and management implementation is proposed.

The Imaging of Pulmonary Hypertension

Pulmonary hypertension (PH) is the remarkable hemodynamic consequence of widespread structural and functional changes within the pulmonary circulation. Elevated pulmonary vascular resistance leads to increased mean pulmonary arterial pressure and, ultimately, right ventricular dysfunction. PH carries a poor prognosis and warrants timely and accurate diagnosis for appropriate intervention. The 2008 Dana Point classification system provides the categorical framework currently guiding therapy and surveillance. Radiologic imaging is an essential tool in the detection and diagnostic evaluation of patients with PH. Echocardiography, ventilation-perfusion scintigraphy, multidetector computed tomography, and cardiac magnetic resonance imaging provide insights into vascular morphology, pulmonary parenchymal status, cardiac function, and underlying etiology of the disorder. Emerging techniques of functional pulmonary and cardiac imaging hold great promise for the assessment and monitoring of these patients in the future.

Cardiac sarcoidosis: the challenge of radiologic-pathologic correlation: from the radiologic pathology archives.

Cardiac sarcoidosis is a rare but potentially fatal disorder with a nonspecific spectrum of clinical manifestations, including conduction disorders, congestive heart failure, ventricular arrhythmias, and sudden cardiac death. Although early treatment to improve morbidity and mortality is desirable, sensitive and accurate detection of cardiac sarcoidosis remains a challenge. Except for the histopathologic finding of noncaseating granulomas in an endomyocardial biopsy specimen, most diagnostic tests are limited and nonspecific at best. Therefore, the decision to initiate treatment is based largely on the patients clinical symptoms and the course of the disease, rather than histologic confirmation. Successful recognition of cardiac sarcoidosis ultimately requires rigorous collaboration among a clinician, radiologist, and pathologist. Advanced imaging modalities, such as cardiac magnetic resonance imaging and positron emission tomography with fluorodeoxyglucose, have become increasingly useful in facilitating diagnosis and therapeutic monitoring, although limited prospective studies exist. This article describes the clinical parameters and pathologic findings of cardiac sarcoidosis and the advanced imaging features and differential diagnostic challenges that must be considered for a successful diagnostic approach. In addition, to improve the understanding of abnormalities detected with different imaging modalities, we suggest a unified terminology in describing radiologic findings related to cardiac sarcoidosis.

CT of acute perianal abscesses and infected fistulae: a pictorial essay

Computed tomography (CT) is an effective, readily available diagnostic imaging tool for evaluation of the emergency room (ER) patients with the clinical suspicion of perianal abscess and/or infected fistulous tract (anorectal sepsis). These patients usually present with perineal pain, fever, and leukocytosis. The diagnosis can be easy if the fistulous tract or abscess is visible on inspection of the perianal skin. If the tract or abscess is deep, then the clinical diagnosis can be difficult. Also, the presence of complex tracts or supralevator extension of the infection cannot be judged by external examination alone. Magnetic resonance imaging (MRI) is the best imaging test to accurately detect fistulous tracts, especially when they are complex (Omally et al. in AJR 199:W43–W53, 2012). However, in the acute setting in the ER, this imaging modality is not always immediately available. Endorectal ultrasound has also been used to identify perianal abscesses, but this modality requires hands-on expertise and can have difficulty localizing the offending fistulous tract. It may also require the use of a rectal probe, which the patient may not be able to tolerate. Contrast-enhanced CT is a very useful tool to diagnose anorectal sepsis; however, this has not received much attention in the recent literature (Yousem et al. in Radiology 167(2):331–334, 1988) aside from a paper describing CT imaging following fistulography (Liang et al. in Clin Imaging 37(6):1069–1076, 2013). An infected fistula is indicated by a fluid-/air-filled soft tissue tract surrounded by inflammation. A well-defined round to oval-shaped fluid/air collection is indicative of an abscess. The purpose of this article is to demonstrate the usefulness of contrast-enhanced CT in the diagnosis of acute anorectal sepsis in the ER setting. We will discuss the CT appearance of infected fistulous tracts and abscesses and how CT imaging can guide the ER physician in the clinical management of these patients.