Multiple Endocrine Neoplasia: Spectrum of Abdominal Manifestations.

OBJECTIVE. Multiple endocrine neoplasia (MEN) syndromes are autosomal-dominant genetic disorders that predispose two or more organs of the endocrine system to tumor development. Although the diagnosis relies on clinical and serologic findings, imaging provides critical information for surgical management with the ultimate goal of complete tumor resection. CONCLUSION. This article reviews abdominal neoplasms associated with the various subtypes of MEN syndromes, with a focus on clinical presentation and characteristic imaging features.

Segmental Arterial Mediolysis: Abdominal Imaging of and Disease Course in 111 Patients.

OBJECTIVE: The purpose of this study is to identify the imaging characteristics of segmental arterial mediolysis (SAM) at presentation and establish the longitudinal course of disease. MATERIALS AND METHODS: We retrospectively identified patients with SAM at a single institution from 2000 through 2015. Diagnosis was based on published guidelines with multidisciplinary consensus. Imaging studies obtained at initial evaluation were reviewed to evaluate imaging findings and vascular territory distribution. All subsequent follow-up imaging studies were reviewed to assess for progression, stability, or regression. RESULTS: We identified 111 patients (79 men and 32 women; median age, 51 years) who met the diagnostic criteria for SAM. Abdominal pain was the most common presentation (74%), followed by flank pain (21%). SAM most commonly affected the renal arteries (47%), superior mesenteric artery (46%), celiac trunk (46%), hepatic artery (23%), iliac arteries (18%), and splenic artery (14%). The most common imaging findings were dissection (86%), aneurysm (57%), beading or webs (28%), occlusion (19%), and a rind or wall thickening (15%). The 247 available follow-up imaging studies for 97 patients (median follow-up, 12 months) showed progression in 19 patients (20%), with either stability or regression observed in the remaining patients. CONCLUSION: SAM most commonly affects the renal arteries, superior mesenteric artery, and celiac artery. Dissections and aneurysms are the most common imaging findings. Follow-up imaging studies show stability or regression in most patients.

Spectrum of Xanthogranulomatous Processes in the Abdomen and Pelvis: A Pictorial Review of Infectious, Inflammatory, and Proliferative Responses.

OBJECTIVE: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. XG infectious processes are mainly seen in cholecystitis and pyelonephritis, but several other organs can also be involved. Histiocytic processes can be divided into Langerhans and non-Langerhans cell histiocytosis. The non-Langerhans cell histiocytosis entities include Erdheim-Chester disease, Rosai-Dorfman disease, juvenile xanthogranuloma, and hemophagocytic lymphohistiocytosis. The inherited lysosomal disorders resulting in XG processes include Nieman-Pick, Gaucher, and other lysosomal storage disorders. CONCLUSION: Radiologists need to be able to recognize features of xanthogranulomatous processes to help facilitate patient management.

Imaging of Abdominal and Pelvic Manifestations of Graft-Versus-Host Disease After Hematopoietic Stem Cell Transplant.

OBJECTIVE: Graft-versus-host disease (GVHD) is a common complication of hematopoietic stem cell transplant (HSCT). GVHD predominantly affects the skin, gastrointestinal system and hepatobiliary systems. Imaging findings in the gastrointestinal tract include bowel wall thickening with mucosal enhancement, mesenteric edema, and vascular engorgement. In the hepatobiliary system, hepatosplenomegaly, periportal edema, bile duct dilatation, and gallbladder and biliary wall thickening are seen. Although the imaging findings of GVHD are nonspecific, with a known history of HSCT, GVHD should be considered. CONCLUSION: GVHD is a serious complication of HSCT, which involves multiple organ systems, with imaging manifestations most commonly seen in the gastrointestinal tract and hepatobiliary system. Knowledge of the imaging manifestations of GVHD, which alone may be relatively nonspecific, taken in conjunction with clinical history including the timing and type of HSCT, laboratory values, stool studies, and dermatologic findings can increase radiologist confidence in suggesting this diagnosis.

Pancreatic Calcifications and Calcified Pancreatic Masses: Pattern Recognition Approach on CT.

OBJECTIVE: The purpose of this article is to review a spectrum of calcified pancreatic masses and propose an algorithm for diagnostic radiologic evaluation. CONCLUSION: Pancreatic calcifications are being detected more frequently because of the widespread use of imaging, particularly CT. Pancreatic calcifications are most commonly associated with chronic pancreatitis related to alcohol abuse. Several other pathologic entities, however, can cause pancreatic calcifications. Familiarity with these entities and their CT appearance is helpful in making an accurate diagnosis.

Imaging and clinical findings in segmental arterial mediolysis (SAM).

Segmental arterial mediolysis (SAM) is an uncommon, non-atherosclerotic, non-inflammatory arteriopathy that tends to affect the medium-sized splanchnic branches of the aorta along with renal, carotid, cerebral, and coronary arteries. The clinical presentation ranges from asymptomatic to severe, life-threatening intra-abdominal hemorrhage and shock. SAM overlaps clinically and radiologically with other inflammatory vasculitides. This article describes the pathologic-radiologic correlation, imaging findings, and the management of the disease. Radiologists should be familiar with this disease entity as imaging plays a crucial role in the diagnosis.