Rosai-dorfman-destombes disease with renal involvement and s
Rosai-Dorfman-Destombes disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare disorder. This disease occurs mainly in children and young adults, however, it may occur at any age.

Published in the journal, Urology Case Reports, the authors present an unusual case of RDD in a 64-year-old woman with a literature review of renal RDD.

A 64-year-old woman with hypertension and Sjogren's syndrome, was evaluated in 2014 because of chronic diffuse abdominal pain, without any other complaints. An abdominal computerized tomography (CT) showed a left renal pelvic lesion suggestive of transitional cell carcinoma.

To further characterize the lesion, an abdominal magnetic resonance (MRI) was performed, which showed a mass of 5.0× 2.4 cm in the left renal pelvis, associated with moderate hydronephrosis. It also informed multiple bilateral perirrenal adenopathies, up to 13 mm in diameter.

Main laboratory findings were serum creatinine of 1.06 mg/dL, (eGFR 52 ml/min/1,73 m2 by MDRD) and ESR of 55 mm/hr. A laparoscopic left nephroureterectomy was performed. The biopsy informed a polymorphic inflammatory infiltrate, with lymphocytes, plasmocytes, eosinophilic and histiocytic large cells with intracytoplasmic lymphocytes (emperipolesis) (, replacing the renal cortex.

The patient remained asymptomatic until April 2017, when she complained of two weeks of progressive severe hypogastric pain, with a creatinine rise to 1.89 mg/dL. Abdominal CT scan evidenced right ureteral obstruction secondary to multiple retroperitoneal adenopathies up to 18 mm diameter, with accentuated hydronephrosis.

A laparoscopic retroperitoneal lymphadenectomy was performed, and the biopsy informed a proliferative lesion composed of a dense monomorphic cellular infiltrate, again compatible with RDD.

After that, corticosteroids were started and the patient evolved favorably, without new episodes of extrinsic urinary compression nor reported complications. She remains in ambulatory medical controls with urologist and nephrologist.

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