Case Report Wegener’s Granulomatosis Presenting as a Renal Mass Maria Roussou, Stavros K. Dimopoulos, Meletios A. Dimopoulos, and Maria I. Anastasiou-Nana Wegener’s granulomatosis is a systemic necrotizing vasculitis that usually involves the kidneys, typically causing segmental necrotizing glomerulonephritis. An association between Wegener’s granulomatosis and renal cell carcinoma was recently reported. We describe a case of Wegener’s granulomatosis presenting as a renal mass in a 72-year-old woman. Histologic examination of the mass revealed granulomatous inflammation, an extremely rare manifestation of this disease. We also review the incidence of renal mass in Wegener’s granulomatosis and highlight the importance of excluding the coexistence of renal cell carcinoma. UROLOGY 71: 547.e1–547.e2, 2008. © 2008 Elsevier Inc.
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egener’s granulomatosis is a systemic necrotizing vasculitis, which primarily involves the upper respiratory tract, the lungs and the kidneys. Its most common renal manifestation is segmental necrotizing glomerulonephritis.1 An association between Wegener’s granulomatosis and renal mass has recently been reported.2 However, the presence of a renal mass in absence of renal cell carcinoma is rare.3–5 We report a patient who presented with upper respiratory symptoms and a renal mass, and was subsequently diagnosed as having Wegener’s granulomatosis.
CASE REPORT A 72-year-old woman was admitted to our hospital with a 3-month history of low-grade fever, pain in the right ear and headache. Otalgia persisted despite treatment with multiple antibiotics and the patient reported progressive hearing loss. One week before admission to our center, the fever rose to 39°C. Her medical history was unremarkable and physical examination was normal. Laboratory tests showed a normochromic, normocytic anemia (hematocrit 29.1%), an erythrocyte sedimentation rate at 90 mm/h, a C-reactive protein concentration at 171 mg/L, and a positive serum test for antineutrophil cytoplasmic antibodies by immunofluorescence in a perinuclear pattern (titer 1:20). The serum creatinine concentration was 0.5 mg/dL, and the urinalysis revealed no proteinuria, hematuria, or casts. Computed tomography (CT) of the chest was normal, CT of the temporal bones showed inflammation of the paranasal sinuses, and abdominal CT revealed two infiltrating processes in the left kidney with increased enhancement on the nephrogram (Fig. 1). There
From the Department of Clinical Therapeutics, “Alexandra” Hospital, University of Athens School of Medicine, Athens, Greece Reprint requests: Maria I. Anastasiou-Nana, M.D., 24 Makedonias, 104 33, Athens, Greece. E-mail:
[email protected] Submitted: August 6, 2007, accepted (with revisions): November 9, 2007
© 2008 Elsevier Inc. All Rights Reserved
Figure 1. Abdominal contrast-enhanced CT scan revealing two infiltrating processes in the left kidney with increased enhancement on the nephrogram. There was a dominant infiltrating mass with a maximum diameter of 4 cm in the lower portion of the left kidney and a second smaller mass (1.3 cm) in the mean portion of the same kidney.
was a main infiltrating mass, 4-cm at its widest diameter, in the lower portion of the left kidney, and a 1.3-cm mass in the midportion of the same kidney. Because we suspected a renal carcinoma, the patient underwent open total left nephrectomy. At the time of operation the surgery revealed two white compact regions in the middle and lower portion of the left kidney, respectively. Histologic examination of the surgical specimen revealed granulomatous inflammation with multinucleated giant cells, lymphocytes, plasmocytes, and neutrophils and basophils necrosis. We made a diagnosis of segmental necrotizing glomerulonephritis, consistent with Wegener’s granulomatosis. The fever reappeared 10 days after the operation and the patient was given a regimen of prednisone, 40 mg/day, and cyclophosphamide, 2 mg/kg per day. At 1 year of follow-up, she remained clinically in remission. 0090-4295/08/$34.00 547.e1 doi:10.1016/j.urology.2007.11.046
DISCUSSION Renal involvement occurs in 80% of patients with Wegener’s granulomatosis. However, renal disease is present in only 20% of patients at the time of diagnosis. Rapidly progressive segmental glomerulonephritis with proteinuria seems to be the most common renal manifestation of the disease.1 Moreover, a recent case-control study showed a strong association between Wegener’s granulomatosis and renal cell carcinoma.2 However, the presence of a renal mass resembling carcinoma in patients suffering from Wegener’s granulomatosis is extremely rare. To our knowledge 10 cases have been reported in the literature.5 We did not proceed with a fine-needle biopsy in this patient because of the location of the lesions (interior portion of the kidney, near to renal hilus), the strong suspicion of renal cell carcinoma raised by the abdominal CT, and the prior knowledge of an association between Wegener’s granulomatosis and renal cell carcinoma.
547.e2
In conclusion, this case highlights the importance of excluding the diagnosis of renal carcinoma in a patient with Wegener’s granulomatosis, presenting with a renal mass.
References 1. Hoffman GS, Kerr GS, Leavitt RY, et al: Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116: 488 – 498, 1992. 2. Tatsis E, Reinhold-Keller E, Steindorf K, et al: Wegener’s granulomatosis associated with renal cell carcinoma. Arthritis Rheum 42: 751–756, 1999. 3. Fairbanks K, Hellmann D, Fishman E, et al: Wegener’s granulomatosis presenting as a renal mass. Am J Roentgenol 174: 1597–1598, 2000. 4. Schapira HE, Kapner J, and Szporn AH: Wegener granulomatosis presenting as renal mass. Urology 28: 307–309, 1986. 5. Krambeck A, Miller D, and Blute M: Wegener’s granulomatosis presenting as renal mass: a case for nephron-sparing surgery. Urology 65: 798, 2005.
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