Compression of kidneys in Erdheim-Chester disease of retroperitoneum: Open surgical approach.

Erdheim-Chester disease is a rare systemic non-Langerhans cell histiocytosis. We report the first case of surgical treatment of severe compression of renal parenchyma by retroperitoneal masses in a 61-year-old male patient with progressing renal failure. After 3 years of follow-up, we have concluded that the open surgical approach is an option in the management of renal complications in Erdheim-Chester disease.