The gamut of primary retroperitoneal masses: multimodality evaluation with pathologic correlation.

The retroperitoneum is a large space where primary and metastatic tumors grow silently before clinical signs appear. Neoplastic retroperitoneal diseases may be solid or cystic, primary or secondary and range from benign to aggressive in behavior. Retroperitoneal neoplasms are notable for their widely disparate histologies. The solid primary retroperitoneal neoplasms are extremely uncommon and can be classified based on their tissue of origin into three main categories: mesodermal tumors, neurogenic tumors, and extragonadal germ cell tumors. These tumors can grow to a large size before clinical symptoms occur or become palpable. When symptoms do occur, they are nonspecific. The majority of these masses are malignant and imaging plays a pivotal role in the detection, staging, and pre-operative planning. Benign and malignant masses should be distinguished whenever possible to avoid unnecessary surgical procedures. Macroscopic fat, calcification, necrosis, vascularity, and neural foraminal widening are common imaging features helping for tumor differentiation. Meticulous cross-sectional imaging can triage the patient to the most appropriate therapy. Tumor morphology dictates imaging character, and biologic activity is reflected by positron emission tomography (PET). Complete surgical excision with tumor free margins is essential for long-term survival. Biopsy should be performed in consultation with surgical oncology to avoid complicating curative surgery. This pictorial essay illustrates the spectrum of multidetector computed tomography (MDCT) imaging findings in common and uncommon primary retroperitoneal masses, with an emphasis on cross-sectional imaging features for an adequate tumor characterization and staging.

Acquired constricting and restricting lesions of the descending duodenum.

The descending duodenum is a structure with distinct pathologic processes and anatomic relationships that requires a systematic approach to the differential diagnosis. Because of its tubular shape and fixed position in the retroperitoneum, both intrinsic duodenal and juxtaduodenal diseases are capable of producing luminal narrowing and obstruction. Duodenal lesions may be located in the mucosa or submucosa. Extraduodenal lesions may originate in adjacent structures--such as the pancreas, liver, gallbladder, colon, and lymph nodes--or from other retroperitoneal structures. Causes of duodenal obstruction include intraluminal masses, such as bezoars; duodenal inflammation, such as as peptic ulcers and Crohn disease; hematomas; and benign or malignant mucosal and intramural tumors. Pancreatic inflammation; tumors; and extrinsic compression caused by gallbladder processes, hepatic masses, retroperitoneal fluid collections, and tumors, including lymphoma, may produce duodenal obstruction. Abdominal radiography, barium studies, multidetector computed tomography, magnetic resonance imaging, and positron emission tomography may be used to depict and characterize duodenal strictures. Integration of imaging, clinical, laboratory, and endoscopic findings plays a major role in establishing a diagnosis of obstructive duodenal strictures.

MDCT Imaging Findings of Liver Cirrhosis: Spectrum of Hepatic and Extrahepatic Abdominal Complications.

Hepatic cirrhosis is the clinical and pathologic result of a multifactorial chronic liver injury. It is well known that cirrhosis is the origin of multiple extrahepatic abdominal complications and a markedly increased risk of hepatocellular carcinoma (HCC). This tumor is the sixth most common malignancy worldwide and the third most common cause of cancer related death. With the rising incidence of HCC worldwide, awareness of the evolution of cirrhotic nodules into malignancy is critical for an early detection and treatment. Adequate imaging protocol selection with dynamic multiphase Multidetector Computed Tomography (MDCT) and reformatted images is crucial to differentiate and categorize the hepatic nodular dysplasia. Knowledge of the typical and less common extrahepatic abdominal manifestations is essential for accurately assessing patients with known or suspected hepatic disease. The objective of this paper is to illustrate the imaging spectrum of intra- and extrahepatic abdominal manifestations of hepatic cirrhosis seen on MDCT.

Grading system modification and management of blunt aortic injury.

BACKGROUND: The traditional approach to blunt aortic injury (BAI) has been emergent intervention. This study aimed to utilize a modified imaging grading system that may allow us to categorize these injuries as needing emergent, urgent, or non-operative management. METHODS: From January 2003 to December 2011, 28 patients with BAI were managed at our institution. Imaging and medical records were reviewed retrospectively. BAI was classified into 4 grades based on imaging studies. Grade Ia: intimal tear, Grade Ib: intramural hematoma; Grade II: intimal injury with periaotic hematoma; Grade IIIa: aortic transection with pseudoaneurysm, Grade IIIb: multiple aortic injuries; and Grade IV: free rupture. Progression and clinical outcomes of ABI were analyzed. RESULTS: Of the 28 patients, 22 were males and 6 were females with mean age of 38 (range, 7 - 69) years. Twenty-five (89.3%) had descending thoracic aortic injury, two (7.1%) had abdominal aortic injury and one (3.6%) presented with multiple aortic injuries. Three patients (10.7%) with Grade I, 1 (3.6%) Grade II, 22 (78.6%) Grade III, and 2 (7.1%) Grade IV injuries. Twenty-five patients underwent thoracic endovascular aortic repair and 3 were managed medically. Median time between injury and surgical intervention was (2 ± 1) days. One (3.6%) patient developed paraplegia after thoracic endovascular aortic repair (TEVAR). One Type 2 endoleak spontaneously sealed within 1 month, and another patient died from ruptured Type 1 endoleak 3 years later. Median follow-up time was 16 (range, 1 - 96) months. Perioperative 30-day mortality rate was 3.6%. CONCLUSIONS: This study based on our modified BAI grading system indicated that Grade I BAI can be managed conservatively. Grade II injury requires close observation and repeated computerized tomography angiogram (CTA) within 48 - 72 hours. If injury appears worse on follow up imaging, surgery should be performed. Delayed repair of Grade III BAI is acceptable if associated life threatening traumatic injuries need to be addressed first.

Genitourinary imaging: part 1, congenital urinary anomalies and their management.

OBJECTIVE: Congenital urinary anomalies may be symptomatic or encountered during imaging for other clinical indications. The array of abnormalities is related to the embryologic stage at the time of the developmental insult, and these abnormalities result in a spectrum of conditions ranging from insignificant to incompatible with life. CONCLUSION: Understanding the implications of common congenital urinary anomalies is the key to detecting associated anomalies, initiating therapy, and avoiding both complications and unnecessary intervention.

Acute aortic occlusion in a child secondary to lap-belt injury treated with thromboendarterectomy and primary repair.

Abdominal aortic injury as a result of blunt trauma is a rare event and has been described in few children. A 6-year-old girl presented with acute bilateral lower extremity ischemia, and a triad of acute aortic occlusion, intra-abdominal visceral injury, and a lumbar chance fracture after sustaining a seat belt injury from a motor vehicle collision. An emergency aortic thromboendarterectomy and primary repair were performed. This represents one of the few reports of acute traumatic aortic thrombosis in a child and highlights the surgical treatment of acute abdominal aortic injury in a pediatric patient.

Primary retroperitoneal paraganglioma simulating a pancreatic mass: a case report and review of the literature.

Paragangliomas are extra-adrenal tumors of the autonomic nervous system and may be found within the skull base, neck, chest, and abdomen. When presenting within the abdominal cavity, they may arise as a primary retroperitoneal neoplasm and can mimic vascular malformations or other conditions related to specific retroperitoneal organs such as the pancreas, kidneys, or adrenals. Retroperitoneal paragangliomas are mostly benign with good prognosis; however, they can present with abdominal pain, palpable mass, or hypertensive episodes. Patients should be initially evaluated with catecholamine levels, followed by computed tomography or magnetic resonance imaging to locate the primary lesion. Surgical excision remains the mainstay of treatment, although advanced disease and proximity to vital organs can make excision difficult or impossible. This case report describes a patient who initially underwent work up for a suspected pancreatic head mass which was discovered to be a retroperitoneal paraganglioma by frozen section.

Multiple hereditary exostoses as a rare nonatherosclerotic etiology of chronic lower extremity ischemia.

Nonatherosclerotic etiologies of arterial insufficiency are uncommon but important causes of chronic lower extremity ischemia. We report a patient with multiple hereditary exostoses (MHE) presenting with lifestyle-limiting lower extremity claudication and popliteal artery occlusion secondary to a large osteochondroma. The presence of MHE with associated osteochondroma resulting in arterial occlusion is a rare condition. Management strategies for treating large osteochondromas adjacent to or with vessel involvement in asymptomatic patients remain undefined.

Accurate grading of 3 synchronous liposarcomas assessed by PET-CT in a single patient.

Positron emission tomography (PET) with 2-fluoro-deoxyglucose (FDG) has become an established imaging modality that can accurately and noninvasively differentiate malignant neoplasms from benign masses. It is increasingly being used to grade malignant neoplasms as well and has almost replaced other studies like gallium 67-citrate scans for metabolic imaging. We describe an interesting case of 3 synchronous liposarcomas with different radio-opacifications on computed tomography (CT). The more aggressive lesion with more opacity on CT showed intense FDG activity and was found to be a high-grade liposarcoma on pathology. The well-differentiated lesion with more fat content appearing less radio-opaque on CT showed almost no FDG activity and an intermediate grade lesion with intermediate radio-opacity on CT showed mildly increased FDG activity. Dual modality imaging with integrated PET/CT systems have strengthened the confidence of classifying these lesions even before knowing the pathology as depicted in this case.

Blunt traumatic injuries of the lung parenchyma, pleura, thoracic wall, and intrathoracic airways: multidetector computer tomography imaging findings.

This pictorial review discusses multi-detector computed tomography (MDCT) cases of non-vascular traumatic chest injuries, with a brief clinical and epidemiological background of each of the pathology. The purpose of this review is to familiarize the reader with common and rare imaging patterns of chest trauma and substantiate the advantages of MDCT as a screening and comprehensive technique for the evaluation of these patients. Images from a level 1 trauma center were reviewed to illustrate these pathologies. Pulmonary laceration, pulmonary hernia, and their different degrees of severity are illustrated as examples of parenchymal traumatic lesions. Pleural space abnormalities (pneumothorax and hemothorax) and associated complications are shown. Diaphragmatic rupture, fracture of the sternum, sternoclavicular dislocation, fracture of the scapula, rib fracture, and flail chest are shown as manifestations of blunt trauma to the chest wall. Finally, direct and indirect imaging findings of intrathoracic airway rupture and post-traumatic foreign bodies are depicted. The advantage of high quality reconstructions, volume rendered images, and maximal intensity projection for the detection of severe complex traumatic injuries is stressed. The limitations of the initial chest radiography and the benefits of MDCT authenticate this imaging technique as the best modality in the diagnosis of chest trauma.

Pulmonary hypertension in a patient with adult-onset Stills disease.

Pulmonary manifestations of adult-onset Still's disease (AOSD) include aseptic pneumonitis, pleural effusions, rarely acute respiratory distress syndrome, and restrictive lung disease. Pulmonary arterial hypertension (PAH) occurs with several rheumatologic diseases, however, has only been reported once in AOSD. We describe a 29-year-old woman with a 9-year history of AOSD, who developed PAH without any other obvious cause. Therefore, we conclude that this is likely a result of pulmonary vascular changes related to AOSD.

Wegener granulomatosis: a case report and update.

Wegener granulomatosis (WG) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a triad involving the upper airways, lungs and kidneys. Ninety percent of patients present with symptoms involving the upper and/or lower airways, and 80% will eventually develop renal disease. WG should be suspected in any patient with progressive or unresponsive sinus disease, glomerulonephritis, pulmonary hemorrhage, mononeuritis multiplex or unexplained multisystem disease. Before the routine use of glucocorticoids and cyclophosphamide, the one year mortality was 82%. However in 1973, Fauci and Wolf discovered that daily prednisone and cyclophosphamide induced complete remission in 75% of patients. The continued use of prednisone and cyclophosphamide for 1 year past remission leads to marked improvement in more than 90% of patients; however, is also associated with serious toxicities. Depending on the disease severity, current treatments employ induction with short-term cyclophosphamide followed by less toxic agents such as methotrexate to maintain disease remission. Although it is a rare disorder, it is pertinent to internists because it is a multisystem disease that presents in a variety of ways. We describe a 63-year-old white male with WG who presented with progressively worsening headaches, bilateral eye redness, epistaxis, hemoptysis and an unintentional 20 pound weight loss, and review the current treatment recommendations.