A 67 y.o. Albanian woman with a hx of endometrial cancer presents with leg swelling.
Her CT is shown below
What do you notice on CT?
Our Albanian patient had retroperitoneal fibrosis which obstructed both kidneys and encased her aorta and iliac vessels.
the arrow demonstrates retroperitoneal fibrosis encasing the vessels
Although it had been first described in 1905 by Albarran, it was rediscovered by Ormand, an American urologist in 1948 and has been known since then as Ormand’s disease. The usual presentation is pain in the back or abdomen.
The origin of the disease is idiopathic in 2/3 of cases while the remaining 1/3 arise from malignancies, infection, drugs, and injuries. Differentiation of these two forms is essential since the treatments differ. In the idiopathic cases about half are associated with IgG4-related disease which is an immune-mediated disease with fibroinflammatory involvement of organs and lymph nodes. IgG4-related disease is treated with steroids and immune suppression.
One other interesting possibility for the differential in a woman from the Balkans with worsening renal failure is Balkan endemic nephropathy(BEN). The patients most commonly affected reside in the Balkans.
Where are the Balkans?
They comprise a peninsula of land in Eastern Europe including Bosnia, Croatia and Albania as pictured above..
The disease is thought to be related to aristolochic acid , which is an environmmental nephrotoxin which can contaminate flour. It is found in a plant native in the Balkans, Aristolochia clematis commonly called birthwort. It has been used to treat kidney problems since Roman times .It has been implicated in poisoning diagnosed at a clinic in Brussels, Belgium when women taking a Chinese weight loss drug developed renal failure.
Our pt had been treated with pelvic radiation and vaginal brachytherapy after hysterectomy. Her last visit was 7/19 and she was felt to be free of disease. When she returned with a retroperitoneal mass and bilateral hydronephrosis bilateral ureteral stents were placed. A biopsy could not be performed because IR felt there was no safe window. Dr. Thaker ordered a PET-CT to determine if there is a safe window for biopsy. Her CA-125 was elevated and she was not given immunosuppression.
Runowska M, Majewski D, Puszczewicz M. retroperitoneal fibrosis—a report of five cases. 2017. Reumatologia 55(3):140-144.
Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006;367:241-251.
Yachoui R, Sehgal R, Carmichael B. Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis. Clin Rheumatol 2016;35:401-407.
De Broe M. Chinese herbs nephropathy and Balkan endemic nephropathy; toward asingle entity, aritolochic acid nephropathy. Kidney Int 2012;81(60:513.