Skip to main content

Advertisement

SpringerLink
Log in

Spinal Rosai–Dorfman disease: case report and literature review

  • Case Report
  • Published:
European Spine Journal Aims and scope Submit manuscript

Abstract

Objective

Sinus histiocytosis with massive lymphadenopathy or Rosai–Dorfman disease (RDD) is a rare benign disease of dubious etiology that arises predominantly in lymph nodes with generalized fever and malaise. Isolated intraspinal involvement has its unique characteristics. The purpose of this study is to present the largest series of cases in the spinal Rosai–Dorfman disease literature to increase familiarity with its clinicopathologic features, diagnosis, and treatment of RDD from spine.

Methods

We present the case of a 34-year-old man who presented with paraplegia secondary to an isolated thoracic vertebral lesion. On physical exam, the patient displayed progressive weakness and tendon hyperreflexia of the lower limbs. After a totally section, symptoms of the patient were obviously relieved and the patient remained asymptomatic and no signs of recurrences were observed after follow-up for 5 months. We also retrospectively analyzed 60 cases of patients with spinal RDD published in English since 1969. Clinical date, histopathology, and radiological feature were retrospectively analyzed.

Results

Spinal RDD should no longer be considered rare and it may occupy an increasingly prominent place in the list of differential diagnoses for intraspinal lesions. Only elaborate histopathology was diagnostic for RDD. Most of the patients were surgically treated and marked improvements were observed in their clinical conditions.

Conclusions

RDD with spinal involvement is uncommon and it is challengeable in making a certain diagnosis. Histopathologic characteristics and immunohistochemical findings are considered as the key points for the diagnosis of this disease. The optimal treatment remains controversial, and more efforts should be focused on the investigation of etiology and adjuvant therapy for relapsing cases or subresected lesions.

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

Access this article

Log in via an institution

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

References

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

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  3. Ma J, Xiao J, Wang L (2008) Extranodal Rosai-Dorfman disease with multilevel lumbar spinal lesions. J Neurosurg Spine 9(1):55–57

    Article  PubMed  Google Scholar 

  4. Hargett C, Bassett T (2005) Atypical presentation of sinus histiocytosis with massive lymphade-nopathy as an epidural spinal cord tumor: a case presentation and literature review. J Spinal Disord Tech 18(2):193–196

    Article  PubMed  Google Scholar 

  5. Raslan O et al (2008) Intracranial Rosai-Dorfman disease with relapsing spinal lesions. J Clin Oncol 26(18):3087–3089

    Article  PubMed  Google Scholar 

  6. Destombes P (1965) Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases). Bull Soc Pathol Exot Fil 58(6):1169–1175

    CAS  Google Scholar 

  7. Buchino JJ, Byrd RP, Kmetz DR (1982) Disseminated sinus histiocytosis with massive lym-phadenopathy: its pathologic aspects. Arch Pathol Lab Med 106(1):13–16

    CAS  PubMed  Google Scholar 

  8. Sandoval-Sus JD et al (2014) Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore) 93(3):165–175

    Article  Google Scholar 

  9. Kessler E et al (1976) Sinus histiocytosis with massive lymphadenopathy and spinal epidural involvement: a case report and review of the literature. Cancer 38(4):1614–1618

    Article  CAS  PubMed  Google Scholar 

  10. Haas RJ, Helmig MS, Prechtel K (1978) Sinus histiocytosis with massive lymphadenopathy and paraparesis: remission with chemotherapy. A case report. Cancer 42(1):77–80

    Article  CAS  PubMed  Google Scholar 

  11. Chan KW et al (1985) Rosai-Dorfman disease presenting as spinal tumor. A case report with ultra-structural and immunohistochemical studies. J Bone Jt Surg Am 67(9):1427–1431

    Article  CAS  Google Scholar 

  12. Unni KK (1988) Case report 457. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) presenting as lesion in the sacrum. Skelet Radiol 17(2):129–132

    Article  CAS  Google Scholar 

  13. Katz DS, Poe LB, Corona RJ (1993) Sinus histiocytosis with massive lymphadenopathy: a case of simultaneous upper respiratory tract and CNS disease without lymphadenopathy. AJNR Am J Neuroradiol 14(1):219–222

    CAS  PubMed  Google Scholar 

  14. Osenbach RK (1996) Isolated extranodal sinus histiocytosis presenting as an intramedullary spinal cord tumor with paraplegia. Case report. J Neurosurg 85(4):692–696

    Article  CAS  PubMed  Google Scholar 

  15. Bernard F et al (1999) Sinus histiocytosis (Destombes-Rosai-Dorfman disease) revealed by extran-odal spinal involvement. Arch Pediatr 6(2):173–177

    Article  CAS  PubMed  Google Scholar 

  16. Kelly WF, Bradey N, Scoones D (1999) Rosai-Dorfman disease presenting as a pituitary tumour. Clin Endocrinol (Oxf) 50(1):133–137

    Article  CAS  Google Scholar 

  17. Hollowell JP et al (2000) Rosai-Dorman disease causing cervical myelopathy. Spine (Phila Pa 1976) 25(11):1453–1456

    Article  CAS  Google Scholar 

  18. Andriko JA et al (2001) Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 14(3):172–178

    Article  CAS  PubMed  Google Scholar 

  19. Yip CC et al (2002) Orbital, adnexal, and unusual systemic involvement in Rosai-Dorfman disease. Ophthal Plast Reconstr Surg 18(3):223–227

    Article  PubMed  Google Scholar 

  20. Sato A et al (2003) Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka 31(11):1199–1204

    PubMed  Google Scholar 

  21. Bhaskar V et al (2003) Sinus histiocytosis with nodal and extranodal involvement—a case report. J Indian Med Assoc 101(1):28–30

    CAS  PubMed  Google Scholar 

  22. Chen KT (2003) Crush cytology of Rosai-Dorfman disease of the central nervous system. A report of 2 cases. Acta Cytol 47(6):1111–1115

    Article  PubMed  Google Scholar 

  23. Tubbs RS et al (2005) Spinal cord compression as a result of Rosai-Dorfman disease of the upper cervical spine in a child. Childs Nerv Syst 21(11):951–954

    Article  PubMed  Google Scholar 

  24. Purav P et al (2005) Rosai-Dorfman disease of the central nervous system. J Clin Neurosci 12(6):656–659

    Article  CAS  PubMed  Google Scholar 

  25. Al-Saad K et al (2005) Extranodal Rosai-Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression. Pediatr Dev Pathol 8(5):593–598

    Article  PubMed  Google Scholar 

  26. Kidd DP, Revesz T, Miller NR (2006) Rosai-Dorfman disease presenting with widespread intra-cranial and spinal cord involvement. Neurology 67(9):1551–1555

    Article  PubMed  Google Scholar 

  27. Robert EG, Fallon KB, Tender GC (2006) Isolated Rosai-Dorfman disease of the sacrum. Case illustration. J Neurosurg Spine 4(5):425

    Article  PubMed  Google Scholar 

  28. Bhandari A, Patel PR, Patel MP (2006) Extranodal Rosai-Dorfman disease with multiple spinal lesions: a rare presentation. Surg Neurol 65(3):308–311

    Article  PubMed  Google Scholar 

  29. Seyednejad F et al (2007) Presumed recurrence of intracranial Rosai-Dorfman disease as a cervical spine tumor. Acta Neurochir (Wien) 149(4):425–427

    Article  CAS  Google Scholar 

  30. Huang YC et al (2007) Spinal epidural Rosai-Dorfman disease preceding by relapsing uveitis: a case report with literature review. Spinal Cord 45(9):641–644

    Article  PubMed  Google Scholar 

  31. Dauendorffer JN et al (2007) Cutaneous and epidural Rosai-Dorfman disease. Ann Dermatol Venereol 134(3 Pt 1):257–260

    Article  PubMed  Google Scholar 

  32. Mitra S et al (2007) A young male with paraplegia, massive cervical adenopathy and nondiagnostic biopsy specimens. J Assoc Phys India 55:238–241

    CAS  Google Scholar 

  33. Dran G et al (2008) Rosai-Dorfman disease causing spinal cord compression: case report. Neurosurgery 62(4):E977–E978 (discussion E978)

    Article  PubMed  Google Scholar 

  34. Konca C et al (2009) Extranodal multifocal Rosai-Dorfman disease: response to 2-chlorodeoxyadenosine treatment. Int J Hematol 89(1):58–62

    Article  PubMed  Google Scholar 

  35. Wang Y et al (2010) Rosai-Dorfman disease isolated to the central nervous system: a report of six cases. Neuropathology 30(2):154–158

    Article  PubMed  Google Scholar 

  36. Abou-Zeid AH et al (2010) Isolated extradural Rosai-Dorfman disease of the thoracic spine: a rare cause of spinal cord compression: case report. Neurosurgery 67(2):E514–E515

    Article  PubMed  Google Scholar 

  37. Maiti TK et al (2011) Rosai-Dorfman disease presenting as cervical extradural lesion: a case report with review of literature. Neurol India 59(3):438–442

    Article  PubMed  Google Scholar 

  38. Ambekar S et al (2011) Isolated cranio-spinal involvement of Rosai-Dorfman disease: case report. Br J Neurosurg 25(2):297–299

    Article  PubMed  Google Scholar 

  39. Zhu H et al (2012) Imaging characteristics of Rosai-Dorfman disease in the central nervous system. Eur J Radiol 81(6):1265–1272

    Article  PubMed  Google Scholar 

  40. Chen CW et al (2012) Rosai-Dorfman disease presenting as a thoracic intradural extramedullary spinal tumor but without extraspinal manifestations. Acta Neurochir (Wien) 154(2):367–368

    Article  Google Scholar 

  41. Antuna RA et al (2012) Multiple involvement of the central nervous system in Rosai-Dorfman dis-ease. Pediatr Neurol 46(1):54–56

    Article  Google Scholar 

  42. Zhu F et al (2013) Rosai-Dorfman disease: a retrospective analysis of 13 cases. Am J Med Sci 345(3):200–210

    Article  PubMed  Google Scholar 

  43. Roy C et al (2012) Extranodal Rosai-Dorfman disease presenting as spinal extradural lesion: a case report with a review of the literature. J Cancer Res Ther 8(4):647–649

    Article  PubMed  Google Scholar 

  44. Parmar V et al (2013) Rosai-Dorfman disease presenting as cervical radiculopathy. Clin Neurol Neurosurg 115(6):808–810

    Article  PubMed  Google Scholar 

  45. Yao K et al (2013) An intramedullary cervical cord lesion in a 12-year-old girl. Neuropathology 33(5):582–585

    PubMed  Google Scholar 

  46. El MM et al (2014) Rare presentation of Rosai-Dorfman disease mimicking a cervical intramedullary spinal cord tumor. World Neurosurg. 81(2):442.e7–442.e9

    Article  Google Scholar 

  47. Wu L, Xu Y (2014) Rosai-Dorfman disease: a rare lesion with dura tail sign mimicking spinal meningioma. Spine J 14(12):3058–3059

    Article  PubMed  Google Scholar 

  48. Fu X et al (2015) Isolated spinal Rosai-Dorfman disease misdiagnosed as lymphoplasmacyte-rich meningioma by intraoperative histological examination. Brain Tumor Pathol 32(1):72–75

    Article  PubMed  Google Scholar 

  49. Sciacca S et al (2015) Rosai-Dorfman disease with spinal cord compression: a diagnostic challenge. Eur Spine J 24(Suppl 4):S529–S535

    Article  PubMed  Google Scholar 

  50. Tian Y et al (2015) Rosai-Dorfman disease involving the central nervous system: seven cases from one institute. Acta Neurochir (Wien) 157(9):1565–1571

    Article  Google Scholar 

  51. Huang BY et al (2016) Rosai-Dorfman disease of rare isolated spinal involvement: report of 4 cases and literature review. World Neurosurg 85:367.e11–367.e16

    Article  Google Scholar 

  52. Huang BY, Liu HL, Yu CJ (2016) Isolated intramedullary spinal Rosai-Dorfman disease: a case report and literature review. World Neurosurg 88:694.e11–694.e15

    Article  Google Scholar 

  53. de Oliveira LG et al (2016) Double compression caused by isolated spinal Rosai-Dorfman disease. Spine J 16(8):e521–e522

    Article  Google Scholar 

  54. Rocha-Maguey J et al (2016) A new case of cervical intramedullary sinus histiocytosis causing para-plegia and review of the literature. Surg Neurol Int 7:9

    Article  PubMed Central  PubMed  Google Scholar 

  55. Kozak B et al (2015) Rosai-Dorfman disease isolated to the thoracic epidural spine. J Radiol Case Rep 9(11):6–16

    PubMed Central  PubMed  Google Scholar 

  56. Levine PH et al (1992) Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 166(2):291–295

    Article  CAS  PubMed  Google Scholar 

  57. Mehraein Y et al (2006) Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol 59(12):1320–1326

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  58. Becroft DM et al (1973) Benign sinus histiocytosis with massive lymphadenopathy: transient immunological defects in a child with mediastinal involvement. J Clin Pathol 26(7):463–469

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  59. Udono H et al (1999) Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg 91(2):335–339

    Article  CAS  PubMed  Google Scholar 

  60. McAlister WH, Herman T, Dehner LP (1990) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Pediatr Radiol 20(6):425–432

    Article  CAS  PubMed  Google Scholar 

  61. Geara AR et al (2004) Rosai-Dorfman disease mimicking neurofibromatosis: case presentation and review of the literature. Clin Radiol 59(7):625–630

    Article  CAS  PubMed  Google Scholar 

  62. Toh CH et al (2005) Rosai-Dorfman disease with dural sinus invasion. Report of two cases. J Neurosurg 102(3):550–554

    Article  PubMed  Google Scholar 

  63. Adeleye AO et al (2013) Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 32(6):572–578

    Article  Google Scholar 

  64. Lopes LF et al (2003) Emperipolesis in a case of B-cell lymphoma: a rare phenomenon outside of Rosai-Dorfman disease. Ann Diagn Pathol 7(5):310–313

    Article  PubMed  Google Scholar 

  65. Sita G et al (1996) Rosai-Dorfman syndrome with extranodal localizations and response to glucocorticoids: a case report. Haematologica 81(2):165–167

    CAS  PubMed  Google Scholar 

  66. Raveenthiran V et al (2003) Rosai-Dorfman disease: report of a 3-year-old girl with critical review of treatment options. Eur J Pediatr Surg 13(5):350–354

    Article  CAS  PubMed  Google Scholar 

  67. Alqanatish JT et al (2010) Rituximab treatment in a child with rosai-dorfman disease and systemic lupus erythematosus. J Rheumatol 37(8):1783–1784

    Article  PubMed  Google Scholar 

  68. Pagel JM et al (2007) Therapeutic use of Rituximab for sinus histiocytosis with massive lymphade-nopathy (Rosai-Dorfman disease). Am J Hematol 82(12):1121–1122

    Article  PubMed  Google Scholar 

  69. Utikal J et al (2007) Imatinib as a treatment option for systemic non-langerhans cell histiocytoses. Arch Dermatol 143(6):736–740

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Author notes

    Authors and Affiliations

    1. Department of Orthopedics, Huashan Hospital, Fudan University, No. 12 Wulumuqi Middle Road, Shanghai, China

      Haocheng Xu, Fan Zhang, Feizhou Lu & Jianyuan Jiang

    2. The Fifth People’s Hospital of Shanghai, Fudan University, Shanghai, China

      Feizhou Lu

    Authors
    1. Haocheng Xu
      View author publications

      You can also search for this author in PubMed Google Scholar

    2. Fan Zhang
      View author publications

      You can also search for this author in PubMed Google Scholar

    3. Feizhou Lu
      View author publications

      You can also search for this author in PubMed Google Scholar

    4. Jianyuan Jiang
      View author publications

      You can also search for this author in PubMed Google Scholar

    Corresponding author

    Correspondence to Feizhou Lu.

    Ethics declarations

    Conflict of interest

    None of the authors has any potential conflict of interest.

    Ethical approval

    All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

    Informed consent

    Informed consent was obtained from all individual participants included in the study.

    Additional information

    H. Xu and F. Zhang contributed equally to this study.

    Rights and permissions

    Reprints and permissions

    About this article

    Check for updates. Verify currency and authenticity via CrossMark

    Cite this article

    Xu, H., Zhang, F., Lu, F. et al. Spinal Rosai–Dorfman disease: case report and literature review. Eur Spine J 26 (Suppl 1), 117–127 (2017). https://doi.org/10.1007/s00586-017-4975-0

    Download citation

    • Received:

    • Accepted:

    • Published:

    • Issue Date:

    • DOI: https://doi.org/10.1007/s00586-017-4975-0

    Keywords

    Search

    Navigation