Abstract
BackgroundImmunoglobulin G4-related disease (IgG4-RD) is a novel disease concept of unknown cause that is characterized by abundant infiltration of IgG4-positive cells, mass-forming lesions, and elevated serum IgG4 levels. The infiltration of IgG4-positive plasma cells and lymphocytes causes swelling, inflammation, fibrosis, and obliterative phlebitis in multiple organs. On the other hand, IgG4-RD occurring in the ureters has rarely been reported. To our knowledge, this is the first report of laparoscopic partial ureteral resection for IgG4-related focal retroperitoneal fibrosis in a ureter with suspected colon cancer recurrence.Case presentationA 72-year-old man with a history of sigmoid colon cancer visited Shiga University of Medical Science Hospital for regular follow-up in December 2019. Enhanced abdominal computed tomography revealed a mass involving the left ureter. Furthermore, fluorine-18 fluorodeoxyglucose positron emission tomography showed significant accumulation of fluorodeoxyglucose uptake in the same region. Due to the possibility of colon cancer recurrence, a laparoscopic excisional biopsy with partial ureteral resection was performed. Histologically, IgG4-positive plasma cell infiltration exceeding 10 cells per high-power field and a high ratio of IgG4-positive/IgG-positive cells exceeding 40% were observed. The postoperative serum IgG4 level was 384 mg/dL. With the application of these findings to the diagnostic algorithm in the comprehensive diagnostic criteria for IgG4-RD, the mass-forming lesion was diagnosed as definitive IgG4-related focal retroperitoneal fibrosis.ConclusionsIgG4-RD should be considered in the differential diagnosis of retroperitoneal lesions. Moreover, laparoscopic surgery may be useful for making the diagnosis in difficult-to-biopsy cases.
Highlights
Immunoglobulin G4-related disease (IgG4-RD) is a novel disease concept of unknown cause that is characterized by abundant infiltration of IgG4-positive cells, mass-forming lesions, and elevated serum IgG4 levels
IgG4-RD should be considered in the differential diagnosis of retroperitoneal lesions
Ueki et al Surgical Case Reports (2020) 6:197 of IgG4-related focal retroperitoneal fibrosis in a ureter suggestive of colon cancer recurrence that was diagnosed by laparoscopic resection
Summary
IgG4-RD should be considered in the differential diagnosis of retroperitoneal lesions.
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