ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005


The Latest on Twitter

Kidney Week

Abstract: PO2215

A Case of Extranodal Rosai-Dorfman Disease Confirmed by Renal Biopsy

Session Information

  • Onco-Nephrology - 2
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

  • 1500 Onco-Nephrology


  • Chavez, Octavio, USF Health Morsani College of Medicine, Tampa, Florida, United States
  • Chow, Timothy M., USF Health Morsani College of Medicine, Tampa, Florida, United States

Group or Team Name

  • University of South Florida Nephrology

Perinephric stranding is typically caused by infection or obstruction. In rare cases other etiologies has been found. We present a case of the extremely rare extranodal Rosai Dorfman Disease confirmed by renal biopsy.

Case Description

A 72 yo M with CKD III was found to have perinephric stranding during CT surveillance for ascending thoracic aneurysm. Subsequent imaging revealed similar findings worisome for lymphoma. Renal biopsy was performed and revealed xanthogranulomatous inflammation with prominent plasma cells, dense histiocytic infiltrate mixed with scattered lymphoid aggregated plasma cells, histiocytoid cells with large nuclei and abundant cytoplasm. Immunostains showed histiocytoid cells to be immunoreactive to CD68 and S100 protein which is consistent with Rosai Dorfman Disease. Renal function remained stable and given unresectability of the lesion and stability of renal function, the patient was managed conservatively with serial imaging. Renal scan ruled out an obstructive process


Rosai Dorfman Disease is an extremely rare, though benign, disease characterized by proliferation of histiocytes that typically proliferates in lymph nodes in the 2nd -3rd decades of life. While its cause is unknown, consequences of this disease are secondary to mass effect of the lesion. Rarely, RDD can present extra-nodally and pose a risk to renal function.

It is important to evaluate causes of renal perinephric stranding. Rosai Dorfman Disease poses a difficult treatment dilemma given unresectability and risk of renal obstruction.