Erdhelm-Chester disease (ECD) is a rare, progressive, non-Langerhans cell histlocytosis that portends a poor prognosis. Misdiagnosis is common until adequate tissue analysis reveals the infiltrative histlocytic process. The disease process affects various organ systems, and effective treatment options are limited. Knowledge of the constellation of signs and symptoms associated with ECD is important to avoid misdiagnosis. We describe a patient who underwent laparoscopic bilateral ureterolysis and laparoscopic biopsy for presumed retroperitoneal fibrosis confirmed previously by percutaneous needle biopsy findings. The final pathologic diagnosis based on laparoscopic biopsy results was ECD. As evidenced by this case, ureterolysis offers little benefit to patients with ECD.
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