Abstract
Wegener's granulomatosis (WG) is a systemic disorder characterized by necrotizing vasculitis involving the respiratory tract, and in most cases, the kidneys. The most common manifestation of WG in the kidneys is segmental necrotizing glomerulonephritis. The presence of a renal mass as a manifestation of WG is rare. We report a patient with WG in whom a CT scan revealed an infiltrating mass in the lower portion of the left kidney. After surgical exploration, we performed an open radical nephrectomy. Histopathology showed clear cell type renal cell carcinoma (RCC). RCC associated with WG has been reported in only a few cases, and in most of them, the diseases started simultaneously, suggesting common pathogenetic pathways. Long-term immunosuppressive treatment is a known risk factor in the development of malignancies, so occurrence of RCC in WG has been proposed as a side effect of cyclophosphamide treatment. Furthermore, it is important to make a differential diagnosis between RCC and pseudotumors in WG as they cannot be distinguished solely on basis of imaging findings. Due to the higher risk of urologic malignancies, more frequent checkups and screening of WG patients should be considered.
Highlights
Wegener’s granulomatosis (WG) is a disease of unknown etiology characterized by systemic necrotizing granulomatous vasculitis, which primarily involves the upper respiratory tract and the lungs
Renal involvement is present in only 20% of patients at time of diagnosis, it eventually occurs in approximately 80% of all patients suffering from WG
We report a case in which renal cancer developed thirteen years after WG was diagnosed
Summary
Wegener’s granulomatosis (WG) is a disease of unknown etiology characterized by systemic necrotizing granulomatous vasculitis, which primarily involves the upper respiratory tract and the lungs. A chest x-ray showed multiple nodular and patchy shadows throughout both lungs and a prominent left hilum He was diagnosed with WG and started with pulse doses of glucocorticosteroids (IV) after which cyclophosphamide (IV) was instituted, with tapering of corticosteroid dose. The patient was admitted to our hospital due to pain in the left lumbar region, which began two months earlier. He is a nonsmoker; his body mass index was 26; with no exposure to occupational carcinogens, and a negative familial history for renal malignancies. In the non-neoplastic kidney parenchyma there were signs of glomerular sclerosis and vessels showing fibromuscular hyperplasia (Figure 3) He is being followed up on regularly and is in remission of WG and RCC
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