Histopathological studies have significantly contributed to clarifing the pathogenetic mechanisms of idiopathic retroperitoneal fibrosis (Ormond's disease), confirming in most cases its inflammatory nature. In this review we describe the principal pathologic features of the lesion, describing the different inflammatory and fibrogenic cell components. In addition, the differential diagnosis of Ormond's disease and the clinical role of histopathology and immunophenotypical analysis are briefly discussed.
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