[Intraabdominal sclerosing panniculitis--myofibroblast proliferation that can mimic malignancy]. / Intraabdominalis sclerotisáló panniculitis--malignitást utánozni képes myofibroblast-proliferáció.

ABSTRACT

Intraabdominal sclerosing panniculitis is a fibroinflammatory lesion of the intraabdominal fat tissue of unknown origin. The authors report 4 secondary cases, that were associated with other kind of intestinal pathology. The cases had different clinical manifestation (mesenterial sclerosis leading to bowel obstruction, lesion simulating transmural spread or peritoneal metastasis of colorectal carcinoma, and chance finding associated with ulcerative colitis). They review the literature and summarize the features of the reactive process characterized by a spindle cell proliferation, fibrosis (sclerosis), chronic inflammatory infiltrate and fat necrosis. The immunohistochemical staining pattern of spindle cells favors a myofibroblastic origin. These cells, like cells of many other, but not all myofibroblastic lesions are CD-34 negative. The significance of recognizing the lesion as such is highlighted by the fact that the correct diagnosis has been seldom made without excision of the involved bowel segment. Theoretically surgical excision should be reserved for cases with bowel obstruction, or underlying pathology requiring this intervention. They believe that with awareness of the lesion secondary cases are not as rare as previously thought, although primary cases (those not associated with other intestinal pathology or specific etiologic agents) are only rarely encountered in everyday practice. They share the view that both primary and secondary cases are reactions to noxious agents, but this agent is unknown in primary cases.