ISSN: 2578-482X
Authors: Boehm B, Mezhiritsky V, Sesterhenn IA, Chesnut G and Kern SQ*
Objectives: Immune-mediated fibroinflammatory conditions are known to affect multiple organ systems, as seen in the IgG-4 related disease. While tubulointerstitial nephritis and membranous glomerulonephritis are the more common intrinsic renal diseases, IgG-4 mediated disease can mimick urologic malignancies and can pose a significant management dilemma. Our goal is to understand how to better diagnose, exclude malignancy, and preserve renal function in a rare Urologic disease. Materials and Methods: A review of current literature was obtained to provide additional insight and understanding on this rarely observed Urologic disease. In discussing the case presented, a pertinent clinical history, physical exam, and review of cross-sectional imaging is presented. Immunohistochemical analysis of the specimen was thoroughly performed with expert analysis by our consulting pathologist. Results: Very rare reports of renal, ureteral, pelvic, or scrotal IgG4 mediated pseudotumors have been published. In the absence of pathologic tissue analysis of biopsy samples, the diagnosis of this rare disease can be significantly challenging. In our presentation, a male patient was noted to have a 7cm mass replacing the left renal sinus and causing moderate left hydronephrosis. Initial concern was for upper tract urothelial cell carcinoma. However, left ureteropyeloscopy demonstrated normal left renal and ureteral architecture and excisional biopsy of a PET avid left cervical lymph node was performed however pathologic diagnosis was not able to be confirmed. This led to a management dilemma due to the progressing mass size, worsening renal drainage, and symptoms. The patient opted for surgical excision. Final pathology from a left radical nephrectomy was significant for an IgG-4 related inflammatory pseudotumor. Conclusions: IgG-4 related diseases are known to represent a systemic disease that leads to lymphoplasmacystic inflammation and fibrosis within the affected tissues. At this time, there is no standardized method for the diagnosis and treatment of patients with retroperitoneal involvement. Additional research is needed to identify improved diagnostic modalities, the role of immunosuppressive therapy, and the surgical management in this disease process.
Keywords: Pseudotumor; Lymphoplasmacytic; Pyelogram; Ureteropyeloscopy
