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CT of the abdomen W/contrast showing decrease in soft tissue mass just proximal to the iliac bifurcation along the right common iliac artery.
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A 77-year-old African American female was referred to oncology for evaluation of an adrenal fossa mass detected on computed tomography scan of the abdomen and pelvis (CT-scan A/P) that was ordered as a work-up for painless hematuria. Further evaluation by positron emission tomography (PET) scan showed hypermetabolic masses in the left suprarenal an...
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Citations
... In such cases, IgG4-RD has been shown to mimic gynecologic malignancy in ovarian (Akyol et al., 2020;Maruyama et al., 2016;Michaud Maturana et al., 2019), uterine (Ohkubo et al., 2015;Senda et al., 2021), and cervical tissues (Mizuno et al., 2016). It is crucial to recognize and distinguish IgG4-RD from gynecologic malignancy because the former is treated with glucocorticoids and/or rituximab with good response (Maritati et al., 2020;Samji et al., 2020). We present two cases in which IgG4-RD mimicked primary metastatic uterine and recurrent cervical carcinomas. ...
Immunoglobulin G4 (IgG4) related disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4 positive plasma cells. It can manifest in many organ systems; however, there are few cases that report gynecologic organ involvement. It is crucial to correctly diagnose IgG4-related disease versus malignancy because the former is treated with glucocorticoids or rituximab. In this case report, we describe two patients in which IgG4-related disease mimics gynecologic cancer. In the first case, an 85 year old woman presented with diffuse lymphadenopathy and a uterine mass concerning for malignancy. Biopsies were negative for carcinoma. Inguinal lymph node biopsy demonstrated IgG4 positive plasma cells and the patient was treated with rituximab therapy given concurrent severe rheumatoid arthritis. In the second case, a 35 year old woman under surveillance for Stage IB2 squamous cell carcinoma of the cervix (status post definitive chemoradiation therapy) presented with fluorodeoxyglucose (FDG) avid paraaortic lymph nodes on positron emission tomography (PET) imaging with subsequent negative paraaortic lymph node biopsies. Serial imaging and biopsies remained inconclusive despite ongoing diffuse lymphadenopathy and clinical concern for recurrence. Supraclavicular lymph node excision was performed which demonstrated lymphoid hyperplasia with increased IgG4 plasma cells and no evidence of carcinoma, supporting the diagnosis of IgG4-related disease. The patient was treated with high dose steroids with clinical improvement and resolution of abnormal imaging findings. We demonstrate that IgG4-related disease can present with FDG-avid lesions on PET imaging and lymphadenopathy that mimics primary or recurrent gynecologic malignancy. While rare, we conclude the IgG4-related disease is an important differential diagnosis to consider in the workup of primary or recurrent gynecologic malignancy and highlights the value of PET imaging to identify unusual patterns of lymphadenopathy and guide histologic confirmation of disease.
... The patient started prednisone 20mg daily. One month after initiating steroid therapy, interval CT demonstrated decrease in the soft tissue masses [7]. This is a significant biopsy finding as it confirmed diagnosis while preventing the patient an invasive surgical procedure to rule out malignancy. ...
... If two of the three key histologic findings are present, the possibility of IgG4-related disease is strongly suggested pathologically. The most essential criterion to diagnose the disease is the detection of IgG4-positive plasma cells in the lesion [7]. ...
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.
