Skip to main content

Advertisement

SpringerLink
Log in

Intracranial recurrences of Rosai–Dorfman disease in the sellar region: two illustrative cases

  • Case Report
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Rosai–Dorfman disease [RDD] is a condition of an unknown etiology, characterized by proliferation of histiocytes. Not uncommonly extranodal lesions occur, but sellar involvement is very rare. Treatment of this lesion is complicated, especially in cases of recurrences at multiple intracranial sites. We report two cases of RDD in the sellar region with intracranial recurrences to illustrate the clinicopathological features and considerations of the optimal management.

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
Fig. 6
Fig. 7
Fig. 8
Fig. 9

References

  1. Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S (2010) Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 32:572–578

    Article  PubMed  Google Scholar 

  2. Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV (2001) Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 14:172–178

    Article  PubMed  CAS  Google Scholar 

  3. Antonius JI, Farid SM, Baez-Giangreco A (1996) Steroid-responsive Rosai-Dorfman disease. Pediatr Hematol Oncol 13:563–570

    Article  PubMed  CAS  Google Scholar 

  4. Deodhare SS, Ang LC, Bilbao JM (1998) Isolated intracranial involvement in Rosai-Dorfman disease: a report of two cases and review of the literature. Arch Pathol Lab Med 122:161–165

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  6. Geara AR, Ayoubi MA, Achram MC, Chamseddine NM (2004) Rosai-Dorfman disease mimicking neurofibromatosis: case presentation and review of the literature. Clin Radiol 59:625–630

    Article  PubMed  CAS  Google Scholar 

  7. Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG (2004) Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg 18:293–297

    Article  PubMed  CAS  Google Scholar 

  8. Gupta DK, Suri A, Mahapatra AK, Mehta VS, Garg A, Sarkar C, Ahmad FU (2006) Intracranial Rosai-Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: a case report and review of literature. Childs Nerv Syst 22:1194–1200

    Article  PubMed  Google Scholar 

  9. Hadjipanayis CG, Bejjani G, Wiley C, Hasegawa T, Maddock M, Kondziolka D (2003) Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery. Case report. J Neurosurg 98:165–168

    Article  PubMed  Google Scholar 

  10. Kaminsky J, Koerbel A, Mittelbronn M, Beschorner R, Ernemann U, Tatagiba M (2005) Rosai-Dorfman disease involving the cranial base, paranasal sinuses and spinal cord. Clin Neuropathol 24:194–200

    PubMed  CAS  Google Scholar 

  11. Kattner KA, Stroink AR, Roth TC, Lee JM (2000) Rosai-Dorfman disease mimicking parasagittal meningioma: case presentation and review of literature. Surg Neurol 53:452–457, discussion 457

    Article  PubMed  CAS  Google Scholar 

  12. Kayali H, Onguru O, Erdogan E, Sirin S, Timurkaynak E (2004) Isolated intracranial Rosai-Dorfman disease mimicking meningioma. Clin Neuropathol 23:204–208

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  15. Kitai R, Sato K, Kubota T, Kabuto M, Kawano H, Kobayashi H, Tsuji T (1996) Meningeal sinus histiocytosis mimicking lymphoplasmacyte-rich meningioma. Case report. J Neurosurg 84:1051–1054

    Article  PubMed  CAS  Google Scholar 

  16. Komp DM (1990) The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 7:83–86

    PubMed  CAS  Google Scholar 

  17. Konishi E, Ibayashi N, Yamamoto S, Scheithauer BW (2003) Isolated intracranial Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). AJNR Am J Neuroradiol 24:515–518

    PubMed  Google Scholar 

  18. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES (1992) Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 166:291–295

    PubMed  CAS  Google Scholar 

  19. McPherson CM, Brown J, Kim AW, DeMonte F (2006) Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case report. J Neurosurg 104:840–844

    Article  PubMed  Google Scholar 

  20. Middel P, Hemmerlein B, Fayyazi A, Kaboth U, Radzun HJ (1999) Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder. Histopathology 35:525–533

    Article  PubMed  CAS  Google Scholar 

  21. Mohadjer Y, Holds JB, Rootman J, Wilson MW, Gigantelli JW, Custer PL (2006) The spectrum of orbital Rosai-Dorfman disease. Ophthalmic Plast Reconstr Surg 22:163–168

    Article  Google Scholar 

  22. Ng HK, Poon WS (1995) Sinus histiocytosis with massive lymphadenopathy localized to the sella. Br J Neurosurg 9:551–555

    Article  PubMed  CAS  Google Scholar 

  23. Petzold A, Thom M, Powell M, Plant GT (2001) Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatry 71:538–541

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  25. Rotondo F, Munoz DG, Hegele RG, Gray B, Khatun N, Bonert M, Kovacs K (2010) Rosai-Dorfman disease involving the neurohypophysis. Pituitary 13:256–259

    Article  PubMed  Google Scholar 

  26. Sato A, Sakurada K, Sonoda Y, Saito S, Kayama T, Jokura H, Yoshimoto T, Nakazato Y (2003) Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka 31:1199–1204

    PubMed  Google Scholar 

  27. Sharma MS, Padua MD, Jha AN (2005) Rosai-Dorfman disease mimicking a sphenoid wing meningioma. Neurol India 53:110–111

    Article  PubMed  Google Scholar 

  28. Shaver EG, Rebsamen SL, Yachnis AT, Sutton LN (1993) Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. J Neurosurg 79:769–773

    Article  PubMed  CAS  Google Scholar 

  29. Toh CH, Chen YL, Wong HF, Wei KC, Ng SH, Wan YL (2005) Rosai-Dorfman disease with dural sinus invasion. Report of two cases. J Neurosurg 102:550–554

    Article  PubMed  Google Scholar 

  30. Trudel M (1984) Dural involvement in sinus histiocytosis with massive lymphadenopathy. Case report. J Neurosurg 60:850–852

    Article  PubMed  CAS  Google Scholar 

  31. Ture U, Seker A, Bozkurt SU, Uneri C, Sav A, Pamir MN (2004) Giant intracranial Rosai-Dorfman disease. J Clin Neurosci 11:563–566

    Article  PubMed  Google Scholar 

  32. Woodcock RJ Jr, Mandell JW, Lipper MH (1999) Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings–a case report. Radiology 213:808–810

    PubMed  Google Scholar 

  33. Wu M, Anderson AE, Kahn LB (2001) A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol 5:96–102

    Article  PubMed  CAS  Google Scholar 

  34. Yang XP, Yu CS, Li KC, Piao YS, Lu DH (2007) Isolated intracranial Rosai–Dorfman disease: a case report with CT and MR findings. Eur J Radiol Extra 61:77–80

    Article  Google Scholar 

  35. Z'Graggen WJ, Sturzenegger M, Mariani L, Keserue B, Kappeler A, Vajtai I (2006) Isolated Rosai-Dorfman disease of intracranial meninges. Pathol Res Pract 202:165–170

    Article  PubMed  Google Scholar 

Download references

Conflicts of interest

None.

Author information

Authors and Affiliations

  1. Department of Pathology, Chinese PLA General Hospital, No.28 Fu Xing Road, Hai Dian District, Beijing, 100853, China

    Fulin Wang, Xin Song & Wei Chen

  2. Department of Neurosurgery, Chinese PLA General Hospital, Beijing, China

    Guangyu Qiao

  3. Department of Radiology, Chinese PLA General Hospital, Beijing, China

    Xin Lou

Authors
  1. Fulin Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Guangyu Qiao
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Xin Lou
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Xin Song
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Wei Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Fulin Wang.

Additional information

Comment

Rosai–Dorfman disease usually presents as a histiocytic infiltration of lymphoid tissue, but may also involve the orbit, nasopharynx, respiratory pathways, gastrointestinal tract, endocrine glands (particularly the thyroid), bone, and skin. Neurologic manifestations are encountered in approximately 4%. The report of Wang and colleagues on two cases of Rosai–Dorfman disease in this journal is interesting for more than one reason: the intracranial location of the disease and the young age of the patients. RDD, by itself, is a rare disease, although extranodal manifestations are not uncommon. RDD disease, involving the central nervous system (CNS), seems to be more common than previously thought, and should be considered in the differential diagnoses of dural-based lesions. By now, well, over 100 cases of RDD, involving the CNS, have been reported in the literature. In the CNS, RDD may occur at various locations. The presenting symptoms depend on the site of involvement and include cranial nerve deficits, seizures, and increased intracranial pressure. The present report adds loss of pituitary function to this list. Intracranial involvement by RDD may present as a subarachnoid, subdural, or epidural mass at the convexity or in the suprasellar, parasagittal, or petroclival regions, causing headaches and hearing or visual loss. On imaging, intracranial RDD usually manifests as one or more enhancing, dural-based, extra-axial masses with perilesional cerebral edema. Therefore, the radiological appearance of the disease closely mimics the presentation of meningioma. Histology and immunophenotyping are essential to reach a correct diagnosis. In RDD on microscopic examination, the polymorphic infiltrate consists of histiocytes, lymphocytes, and plasma cells. Emperipolesis, signifying the phagocytosis of lymphocytes, may be more difficult to identify in extranodal sites, such as the CNS. Emperipolesis is present in only 70% of cases, and although characteristic, may not be a prominent feature. Immunohistochemical staining is useful in differentiating RDD from other histiocytic lesions. The histiocytes of RDD are thought to be activated macrophages, derived from circulating monocytes, and they are usually strongly reactive to S100 and CD68 protein, while not reactive with CD1a. The histological differential diagnosis includes Langerhans histiocytosis, which could be ruled out in the 10-year-old girl of the present report. Furthermore, inflammatory pseudotumor or lymphoproliferative disorders should be considered in the differential diagnosis. In RDD, the lymphocytes are bland, polyclonal, and show a mixture of B and T phenotypes. While resection followed by corticosteroids is generally the therapy of choice, the authors of the present report recommend adding postoperative radiation therapy. Although a variety of treatment modalities have been reported, including steroid therapy and radiation, surgical excision, radical if possible, is likely to be the appropriate treatment. Most instances of RDD, involving the CNS, are treated surgically. According to the relatively small series reported in the literature, only few patients succumb to the disease, following surgery, despite the fact that lesions may remain present. In case of subtotal resection, local low dose radiation is advocated, and a long-term follow-up showed that intracranial tumor regrowth, or recurrence of symptoms occurred in 14% of patients who received this therapy (mean follow-up of 10 years). However, because of the lack of substantial numbers, therapy remains controversial and long-term follow-up is necessary to record late relapses.

Johan Kros

Rotterdam, Netherlands

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wang, F., Qiao, G., Lou, X. et al. Intracranial recurrences of Rosai–Dorfman disease in the sellar region: two illustrative cases. Acta Neurochir 153, 859–867 (2011). https://doi.org/10.1007/s00701-010-0895-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-010-0895-z

Keywords

Search

Navigation