Abstract
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. A 77-year-old woman with a history of surgery for ectopic pregnancy and wound dehiscence presented with intestinal obstruction. Abdominal enhanced computed tomography (CT) revealed an enhanced, radially shaped, oval mass, 3 cm in diameter, with an unclear rim in the mesentery of the distal ileum, which may have involved the distal ileum. To remove the cause of bowel obstruction, the SM was resected completely and the ileum was resected partially. Histologic examination showed that the mass was composed of spindle cells arranged in a fascicular or storiform pattern; moreover, fibrous stroma was observed, with dense lymphoplasmacytic infiltration and lymphoid follicles. Immunohistochemically, numerous IgG4-positive plasma cells were observed, at a density of 253 per high-powered field, and the IgG4/IgG ratio was about 50 %. Elastica van Gieson (EVG) staining also showed obstructive phlebitis. These findings indicated IgG4-related SM. Although the accurate diagnosis of SM remains difficult without histological analysis, IgG4-RD should be included in the differential diagnosis of unknown mesenteric tumors. Identification of IgG4-RD may prevent unnecessary surgery because corticosteroids may be effective in these patients.
Highlights
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease of unknown etiology that primarily involves the small-bowel mesentery, most frequently observed in middle-aged and older men [1,2,3,4,5,6]
SM was recently reported to be closely related to IgG4-related disease (IgG4-RD) [2, 10–13], a systemic syndrome characterized by masses in various organs infiltrated by IgG4-positive plasma cells and high serum IgG4 concentrations [14, 15]
The spindle cells were negative for anaplastic lymphoma kinase (ALK), desmin, CDK4, MDM2, CD21, CD35, and nuclear β-catenin, whereas the lymphocytes in inter-lymphoid follicles were positive for CD3 and the lymphocytes in germinal centers were positive for CD20
Summary
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease of unknown etiology that primarily involves the small-bowel mesentery, most frequently observed in middle-aged and older men [1,2,3,4,5,6]. Laboratory tests, including those for tumor markers such as CEA and CA19-9, showed no abnormalities Her serum IgG4 concentration was not measured. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) revealed a slight uptake of fluorodeoxyglucose (standardized uptake value 4.4) by the mass These findings could not yield an accurate diagnosis, including whether the mass was malignant or inflammatory. The spindle cells were negative for anaplastic lymphoma kinase (ALK), desmin, CDK4, MDM2, CD21, CD35, and nuclear β-catenin, whereas the lymphocytes in inter-lymphoid follicles were positive for CD3 and the lymphocytes in germinal centers were positive for CD20 These findings indicated a diagnosis of IgG4-related SM, while excluding diagnoses of leiomyosarcoma, inflammatory myofibroblastic tumor, desmoids tumor, liposarcoma, and follicular dendritic tumor. After follow-up for 4 years, there has been no evidence of SM recurrence or symptom relapse
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