Skip to main content

Advertisement

Log in

Extranodal Rosai-Dorfman Disease: a rare presentation involving anterior chest wall in a middle-aged female

  • Case Report
  • Published:
Skeletal Radiology Aims and scope Submit manuscript

Abstract

Rosai-Dorfman Disease is a rare benign disorder involving overproduction of immune cells, causing swollen lymph nodes and, in rare cases, the sternum. The sternal involvement may cause chest pain and masses. Diagnosis is confirmed through clinical examination, biopsy, and imaging. Treatment options may include surgery, radiation, or steroids. In this case study, we present an unusual example of extranodal Rosai-Dorfman Disease involving the sternum, bilateral clavicles and first three ribs, and pectoral muscle with no associated lymphadenopathy or systemic symptoms in a 57-year-old female. The etiology, pathology, immunohistochemistry, imaging findings, and treatment options of this unique disease are discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

Data Availability

The data presented in this case report contains patient information that is confidential. This confidentiality is maintained to protect the privacy and rights of the individuals whose information is included in the report. Interested parties seeking access to the data should contact the University of Alabama at Birmingham, Heersink School of Medicine, to initiate the necessary permissions and procedures.

References

  1. Khoury JD, Solary E, Abla O, Akkari Y, Alaggio R, Apperley JF, Bejar R, Berti E, Busque L, Chan JKC, Chen W, Chen X, Chng WJ, Choi JK, Colmenero I, Coupland SE, Cross NCP, De Jong D, Elghetany MT, Takahashi E, Emile JF, Ferry J, Fogelstrand L, Fontenay M, Germing U, Gujral S, Haferlach T, Harrison C, Hodge JC, Hu S, Jansen JH, Kanagal-Shamanna R, Kantarjian HM, Kratz CP, Li XQ, Lim MS, Loeb K, Loghavi S, Marcogliese A, Meshinchi S, Michaels P, Naresh KN, Natkunam Y, Nejati R, Ott G, Padron E, Patel KP, Patkar N, Picarsic J, Platzbecker U, Roberts I, Schuh A, Sewell W, Siebert R, Tembhare P, Tyner J, Verstovsek S, Wang W, Wood B, Xiao W, Yeung C, Hochhaus A. The 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms. Leukemia. 2022;36(7):1703–19. https://doi.org/10.1038/s41375-022-01613-1.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Campo E, Jaffe ES, Cook JR, Quintanilla-Martinez L, Swerdlow SH, Anderson KC, Brousset P, Cerroni L, de Leval L, Dirnhofer S, Dogan A, Feldman AL, Fend F, Friedberg JW, Gaulard P, Ghia P, Horwitz SM, King RL, Salles G, San-Miguel J, Seymour JF, Treon SP, Vose JM, Zucca E, Advani R, Ansell S, Au WY, Barrionuevo C, Bergsagel L, Chan WC, Cohen JI, d’Amore F, Davies A, Falini B, Ghobrial IM, Goodlad JR, Gribben JG, Hsi ED, Kahl BS, Kim WS, Kumar S, LaCasce AS, Laurent C, Lenz G, Leonard JP, Link MP, Lopez-Guillermo A, Mateos MV, Macintyre E, Melnick AM, Morschhauser F, Nakamura S, Narbaitz M, Pavlovsky A, Pileri SA, Piris M, Pro B, Rajkumar V, Rosen ST, Sander B, Sehn L, Shipp MA, Smith SM, Staudt LM, Thieblemont C, Tousseyn T, Wilson WH, Yoshino T, Zinzani PL, Dreyling M, Scott DW, Winter JN, Zelenetz AD. The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee. Blood. 2022;140(11):1229–53. https://doi.org/10.1182/blood.2022015851.

    Article  PubMed Central  CAS  Google Scholar 

  3. Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, Requena-Caballero L, Jordan MB, Abdel-Wahab O, Allen CE, Charlotte F, Diamond EL, Egeler RM, Fischer A, Herrera JG, Henter JI, Janku F, Merad M, Picarsic J, Rodriguez-Galindo C, Rollins BJ, Tazi A, Vassallo R, Weiss LM, Histiocyte Society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–81. https://doi.org/10.1182/blood-2016-01-690636.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  4. Bruce-Brand C, Schneider JW. Schubert P Rosai-Dorfman Disease: an overview. J Clin Pathol. 2020;73:697–705.

    Article  PubMed  CAS  Google Scholar 

  5. Sall A, Touré AO, Ndiaye FS, Sène A, Sall FB, Faye BF, Seck M, Diop S. Rosai Dorfman disease diagnosed by fine-needle aspiration cytology in a young man with HIV infection. Clin Case Rep. 2015;3(10):879–83. https://doi.org/10.1002/ccr3.391.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Ravindran A, Goyal G, Go RS, Rech KL, Mayo Clinic Histiocytosis Working Group. Rosai-Dorfman Disease displays a unique monocyte-macrophage phenotype characterized by expression of OCT2. Am J Surg Pathol. 2021;45(1):35–44. https://doi.org/10.1097/PAS.0000000000001617.

    Article  PubMed  Google Scholar 

  7. Goyal G, Young JR, Koster MJ, Tobin WO, Vassallo R, Ryu JH, Davidge-Pitts CJ, Hurtado MD, Ravindran A, SartoriValinotti JC, Bennani NN, Shah MV, Rech KL, Go RS, Mayo Clinic Histiocytosis Working Group. The Mayo Clinic histiocytosis working group consensus statement for the diagnosis and evaluation of adult patients with histiocytic neoplasms: Erdheim-Chester disease, Langerhans cell histiocytosis, and Rosai-Dorfman Disease. Mayo Clin Proc. 2019;94(10):2054–71. https://doi.org/10.1016/j.mayocp.2019.02.023.

    Article  PubMed  Google Scholar 

  8. Goyal G, Ravindran A, Young JR, Shah MV, Bennani NN, Patnaik MM, Nowakowski GS, Thanarajasingam G, Habermann TM, Vassallo R, Sher T, Parikh SA, Rech KL, Go RS, Mayo Clinic Histiocytosis Working Group. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman Disease. Haematologica. 2020;105(2):348–57. https://doi.org/10.3324/haematol.2019.219626.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  9. Abeykoon JP, Rech KL, Young JR, Ravindran A, Ruan GJ, Dasari S, Morlote DM, King RL, Rummage C, Zanwar S, Acosta-Medina AM, Tobin WO, Shah MV, Bennani NN, Vassallo R, Ryu JH, Koster MJ, Davidge-Pitts CJ, Witzig TE, Goyal G, Go RS, Mayo Clinic-University of Alabama at Birmingham Histiocytosis Working Group. Outcomes after treatment with cobimetinib in patients with Rosai-Dorfman Disease based on KRAS and MEK alteration status. JAMA Oncol. 2022;8(12):1816–20. https://doi.org/10.1001/jamaoncol.2022.4432.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Andrew B. Ross, Kirkland W. Davis, Darya Buehler, Brian Y. Chan (2019) Primary Rosai-Dorfman Disease of bone: a report of two cases. Case Rep Radiol, 2019:1720131. 6 pages. https://doi.org/10.1155/2019/1720131

  11. Izubuchi Y, Suzuki K, Imamura Y, Katayama H, Ohshima Y, Matsumine A. Primary Rosai-Dorfman Disease of bone arising in the infantile ilium: a case report. Exp Ther Med. 2020;19:2983–8. https://doi.org/10.3892/etm.2020.8568.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Mosheimer BA, Oppl B, Zandieh S, et al. Bone involvement in Rosai-Dorfman Disease (RDD): a case report and systematic literature review. Curr Rheumatol Rep. 2017;19:29. https://doi.org/10.1007/s11926-017-0656-6.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Adam R, Harsovescu T, Tudorache S, Moldovan C, Pogarasteanu M, Dumitru A, Orban C. Primary bone lesions in Rosai-Dorfman disease, a rare case and diagnostic challenge—case report and literature review. Diagnostics. 2022;12(4):783. https://doi.org/10.3390/diagnostics12040783.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Soft tissue and bone pathology: Rosai Dorfman disease presented at Case of the week at NYU Langone Health, Department of Pathology, by: Lucy Wang, DO (Resident) and Jonathan Melamed, MD (Attending), July 24, 2019. https://med.nyu.edu/departmentsinstitutes/pathology/sites/default/files/pathology-cotw-soft-tissue-and-bonepathology-rosai-dorfman-disease.pdf.

  15. Parekh A, Osmani F, Aardsma N, Groth J, Gonzalez M (2019) Osseous Rosai-Dorfman disease presenting as a solitary lesion of the distal radius published: 2018. 44(1):P70.E1–70.E5. https://doi.org/10.1016/j.jhsa.2018.03.019

  16. Arun VP. Primary skeletal Rosai Dorfman disease of distal tibia. J Orthop Oncol. 2021;7:151.

    Google Scholar 

  17. Duijsens HM, Vanhoenacker FM, ter Braak BP, Hogendoorn PC, Kroon HM. Primary intraosseous manifestation of Rosai-Dorfman Disease: 2 cases and review of literature. JBR-BTR. 2014;97(2):84–9. https://doi.org/10.5334/jbr-btr.18.

    Article  PubMed  CAS  Google Scholar 

  18. Vithran DTA, Wang JZ, Xiang F, Wen J, Xiao S, Tang WZ, Chen Q. Osseous Rosai-Dorfman Disease of tibia in children: a case report. World J Clin Cases. 2021;9(6):1416–23. https://doi.org/10.12998/wjcc.v9.i6.1416].

    Article  PubMed  PubMed Central  Google Scholar 

  19. Joshi V, Offer G, Richards C, Rathinam S. Chest wall resection and reconstruction for Rosai-Dorfman Disease masquerading as a chest wall sarcoma. Ann R Coll Surg Eng. 2018;100:e28-30.

    Article  CAS  Google Scholar 

  20. Demicco EG, Rosenberg AE, Björnsson J, Rybak LD, Unni KK, Nielsen GP. Primary Rosai-Dorfman Disease of bone: a clinicopathologic study of 15 cases. Am J Surg Pathol. 2010;34(9):1324–33. https://doi.org/10.1097/PAS.0b013e3181ea50b2.

    Article  PubMed  Google Scholar 

  21. Li H, Li D, Xia J, Huang H, Jiao N, Zheng Z, Zhao Y, Guo X. Radiological features of Rosai-Dorfman Disease: case series and review of the literature. Clin Radiol. 2022;77(11):e799–805. https://doi.org/10.1016/j.crad.2022.07.008.

    Article  PubMed  CAS  Google Scholar 

  22. La Barge III, DV, Salzman KL, Harnsberger HR, Ginsberg LE, Hamilton BE, Wiggins III HR, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): imaging manifestations in the head and neck. AJR. 2008;191:W299-306.

    Article  PubMed  Google Scholar 

  23. Xu Q, Fu L, Liu C. Multimodality imaging-based evaluation of Rosai-Dorfman disease in the head and neck: a retrospective observational study. Medicine (Baltimore). 2017;96:e9372.

    Article  PubMed  Google Scholar 

  24. Hingwala D, Neelima R, Kesavadas C, et al. Advanced MRI in Rosai-Dorfman disease: correlation with histopathology. J Neuroradiol. 2011;38:113–7.

    Article  PubMed  CAS  Google Scholar 

  25. Thomas B, Somasundaram S, Thamburaj K, Kesavadas C, Gupta AK, Bodhey NK, et al. Clinical applications of susceptibility weighted MR imaging of the brain - a pictorial review. Neuroradiology. 2008;50:10e516.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Mostafa Abozeed.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Glossary

CD

Scluster of differentiation

IHC

Immunohistochemistry

OCT2

Octamer binding transcription factor 2

SUV

standardized uptake value

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Agarwal, A., Ravindran, A., Donahue, J. et al. Extranodal Rosai-Dorfman Disease: a rare presentation involving anterior chest wall in a middle-aged female. Skeletal Radiol 53, 589–594 (2024). https://doi.org/10.1007/s00256-023-04427-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00256-023-04427-z

Keywords

Navigation