Extranodal Rosai-Dorfman-Destombes disease mimicking renal cell carcinoma: a case report and review of literature


  • Anil Kumar Nallabothula Department of Urology, SVIMS, Tirupati, Andhra Pradesh, India
  • Konda Reddy Chilekampalli Department of Urology, SVIMS, Tirupati, Andhra Pradesh, India
  • Anirudh Suseel Nalumaru Department of Urology, SVIMS, Tirupati, Andhra Pradesh, India
  • Harshavardhan Varma M Department of Urology, SVIMS, Tirupati, Andhra Pradesh, India
  • Anupam Bhargava Department of Urology, SVIMS, Tirupati, Andhra Pradesh, India
  • Ashish Kumar Jayanth Department of Urology, SVIMS, Tirupati, Andhra Pradesh, India




Rosai-Dorfman, Histiocytic disorders, Non-Langerhans cell histiocytoses, Lymphadenopathy, Extra nodal, Renal mass, Rare


Rosai-Dorfman-Destombes disease (RDD) is an uncommon histiocytic disorder of unknown aetiology. Extranodal involvement occurs in a significant proportion of patients but involvement of the kidney is rare. Here we present a case report of extranodal RDD of the kidney. Imaging revealed a heterogeneously enhancing solid mass lesion in left renal hilum infiltrating renal medulla and deeper layers of renal cortex. With the clinical diagnosis of suspected left renal cell cancer, laparoscopic left radical nephrectomy was performed. Macroscopic examination showed specimen covered with Gerota’s fascia and perinephric pad of fat. Renal capsule could be easily split off. On cut section, ill circumscribed solid lesion having variegated appearance with areas of necrosis and haemorrhage, involving middle and lower pole, was noted. Renal sinus appeared to be involved. Microscopic examination showed sheets of histiocytes with abundant eosinophilic to vacuolated cytoplasm. There was emperipolesis of intact lymphocytes. Also, seen were sheets of plasma cells, lymphocytes with intervening dense hyalinised stroma. Retro peritoneal lymph nodes and para-aortic lymph nodes also showed similar histological picture. Immunohistochemistry showed that the histiocytes were positive for S100 and CD68. IgG4-positive plasma cells were not seen. These findings showed that the lesion represented and extranodal RDD of the kidney. Although rare, extranodal RDD should also be included in the differential diagnosis of a renal mass and frozen section be advised whenever feasible.


Weitzman S, Jaffe R. Uncommon histiocytic disorders: the non-langerhans cell histiocytoses. Pediatr Blood Cancer. 2005;45(3):256-64.

Destombes P. Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali (4 cases). Bull Soc Pathol Exot. 1965;58(6):1169-75.

Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63-70.

Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol. 1990;7(1):19-73.

Gaitonde S. Multifocal, extranodals sinus histiocytosis with massive lymphadenopathy: an overview. Arch Pathol Lab Med. 2007;131:1117-21.

Mahzoni P, Zavareh MH, Bagheri M, Hani N, Moqtader B. Intracranial ROSAI-DORFMAN disease. J Res Med Sci. 2012;17(3):304-7.

Kutlubay Z, Bairamov O, Sevim A, Demirkesen C, Mat MC. Rosai-Dorfman disease: a case report with nodal and cutaneous involvement and review of the literature. Am J Dermatopathol. 2014;36(4):353-7.

Paulli M, Bergamaschi G, Tonon L. Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Br J Haematol. 1995;91(2):415-8.

Delacretaz F, Meug e-Moraw C, Anwar D, Borisch B, Chave JP. Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient. Virchows Arch A Pathol Anat Histopathol. 1991;419(3): 251-4.

Diamond EL, Durham BH, Haroche J. Diverse and targetable kinase alterations drive histiocytic neoplasms. Cancer Discov. 2016;6(2):154-65.

Garces S, Medeiros LJ, Patel KP. Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease. Mod Pathol. 2017;30(10):1367-77.

Milne P, Bigley V, Bacon CM. Hematopoietic origin of Langerhans cell histiocytosis and Erdheim-Chester disease in adults. Blood. 2017;130(2):167-75.

Emile J-F, Abla O, Fraitag S. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016;127:2672-81.

Krishnan A, Nassar A, Nieh PT. Rosai-Dorfman disease presenting as extranodal renal mass. Urology. 2005;66(6):1319.

El Majdoub A, El Houari A, Chbani L, El Fatemi H, Khallouk A, Farih MH. Isolated localization of Rosai Dorfman disease as renal mass: a case report and review of literature. Pan Afr Med J. 2016;24:64.

Sasaki K, Pemmaraju N, Westin JR. A single case of Rosai-Dorfman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single agent cladribine. Front Oncol. 2014;4:297.

Rocken C, Wieker K, Grote HJ, Muller G, Franke A, Roessner A. Rosai-Dorfman disease and generalized AA amyloidosis: a case report. Hum Pathol. 2000;31(5):621-4.

Yamada S, Uemura M, Tokumoto M. Hypercalcemia induced by Rosai-Dorfman disease in a hemodialysis patient: histological evidence of extrarenal calcitriol overproduction. Intern Med. 2014;53(24):2783-7.

Karaosmanoglu AD, Onur MR, Shirkhoda A, Ozmen M, Hahn PF. Unusual benign solid neoplasms of the kidney: cross-sectional imaging findings. Diagn Interv Radiol. 2015;21(5):376-81.

Haroche J, Abla O. Uncommon histiocytic disorders: Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease. Hematology Am Soc Hematol Educ Program. 2015;2015:571-8.

Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile JF et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood. 2018;131(26):2877-90.






Case Reports