IgG4-related renal pseudotumor in a child with history of Wi
IgG4-related disease (IgG4-RD) is a novel immune-mediated systemic disease characterized by lymphoplasmacytic infiltration and fibrosis of tissues. Published in the Urology Case Reports, the authors describe the first case of an isolated renal IgG4 pseudotumor in an 11-year-old boy with a history of previously treated Wilms tumor (WT).

An 11-year-old asymptomatic male presented to clinic with a right upper pole renal mass, found on surveillance imaging in follow up for WT. At age 4 he was treated for stage III WT of the left kidney with nephrectomy, chemotherapy (actinomycin, vincristine, doxorubicin), and flank radiation.

He had no concerning findings on surveillance imaging between initial treatment and age 11 years. Surveillance ultrasound followed by computed tomography scan showed a right upper pole 2.3 cm mass concerning for malignancy.

Given his history of solitary kidney and potential local upstaging with biopsy given concern for metachronous WT, the patient underwent right partial nephrectomy with regional lymph node dissection.

Learning Points:-
- IgG4-RD is a recently defined condition that may present with a pseudotumor mimicking malignancy and is commonly misdiagnosed.

- History of malignancy may portend future IgG4-RD development.

Read about the case in detail here: https://pxmd.co/sNPIN