좌측 신장을 침범한 특발성후복막강섬유증의 1예

Abstract

Idiopathic retroperitoneal librosis is a rare disease which is characterized by the formation of a dense fibrous plaque in the rctroperitoneum The basic pathologic picture is one of a proliferative fibrotic reaction involving the retroperitoneal tissues associated with a chronic inflammatory response. We experienced a case of idiopathic retroperitoneal librosis which involved the left kidney in a 42-year-old man who had a history of gastrectomy for peptic ulcer perforation 10 years ago. The abdominal mass was explored and a large retroperitoneal mass was removed under an impression of malignant tumor. The mass was located in left perirenal tissue, displacing the kiney upward and medially. The tumor mass was lobulated , well circumscribed. It measured 25 x 17x4cm. in maximum dimensions and I. 7kg in weight. Microscopically , the vast majority of the tumor consisted of a dense collagen fibrosis admixed with islands of fat tissue where some fibroblastic activity was seen. Bizarre giant cells were also noted in the midst of seemingly inactive fibrotic process Perivascular inflammatory cell infiμration was another prominent feature throughout the slides , and it was more pronounced around venous channels. A small area of superficial kidney just beneath the involved capsule , showed focal but irregular invasion of fibrotic process into the parenchyma and was associated with rather heavy inflammatory response, perivascultis and glomerular hyalinization