Chronic Fibrosing Conditions in Abdominal Imaging

Abstract

Chronic fibrosing conditions of the abdomen are relatively poorly understood and involve varied and often multiple organ systems. At histopathologic analysis, they share the unifying features of proliferative fibrosis and chronic inflammation. Different conditions in this group are often found in association with each other and with other fibrosing conditions outside the abdomen. Some of the confusion about these conditions stems from their complex nomenclature, which includes a gamut of alternate terms and eponyms. Many of them can be categorized within two large subgroups: the fibromatoses and immunoglobulin G4 (IgG4)-related disorders. While many of these entities are of uncertain etiology, some, especially the IgG4-associated conditions, appear to have an immune-mediated pathogenesis. Nephrogenic systemic fibrosis, sclerosing peritonitis, and retroperitoneal fibrosis have iatrogenic associations, while some of the fibromatoses are genetically inherited. Imaging differentiation of these conditions is difficult due to considerable overlap in their radiologic findings. However, certain conditions such as penile fibromatosis and sclerosing peritonitis may have unique imaging features that can help the radiologist make the diagnosis. Others such as deep fibromatoses and inflammatory pseudotumor demonstrate fibroproliferative mass formation and cannot be differentiated from neoplastic conditions at imaging. Thus, histopathologic correlation is often required to confirm their diagnosis.

Renographic Demonstration of Desmoid Tumor-Ureteral Fistula

“…[1][2][3][4] They are rare, constituting only 0.03% of all neoplasms, with an incidence of 2 to 4 cases per million per year. 1,5 The etiology is suspected to be multifactorial with hormonal factors, trauma, and genetic factors implicated.…”

“…1,5 The etiology is suspected to be multifactorial with hormonal factors, trauma, and genetic factors implicated. 1,2,5 One important genetic factor is Gardner syndrome, a subtype of FAP, characterized by extracolonic lesions, including desmoid tumors. 2 Desmoid tumors occur in approximately 10% to 15% of patients with FAP, and approximately 5% of all desmoid tumors are associated with FAP.…”

“…1,2,5 One important genetic factor is Gardner syndrome, a subtype of FAP, characterized by extracolonic lesions, including desmoid tumors. 2 Desmoid tumors occur in approximately 10% to 15% of patients with FAP, and approximately 5% of all desmoid tumors are associated with FAP. 3 Desmoid tumors associated with Gardner syndrome tend to be multiple and involve the mesentery and abdominal wall.…”

A rare cause of acute post renal failure: Retroperitoneal fibrosis

“…Non-contrast abdominal CT and magnetic resonance imaging (MRI) provide limited information in the diagnosis of the disease (14). In non-contrast CT, RF usually appears as an increase in soft tissue density, surrounding the abdominal aorta and iliac vessels, and usually surrounding the ureter and inferior vena cava (15). Positron emission tomography (PET-CT) provides more comprehensive clinical information as it includes whole-body metabolic studies.…”

The Development of Rapidly Progressive Glomerulonephritis in Patient Diagnosed with Idiopathic Chronic Periaortitis

“…5 Kronik periaortit tanısı görüntüleme yöntemlerine dayanılarak konulur. 6 Distal abdominal aortada geniş-leme oluşturarak veya oluşturmadan kronik adventisyal inflamasyon ile beraber ateroskleroz ve aort duvarı mediya tabakasında kalınlaşmaya neden olan ve komşu dokulara da yayılabilen inflamatuar bir süreçtir. 7 Kronik periaortit, ilk kez 1905 yılında Fransız ürolog Albarran tarafından tanımlanmıştır.…”