Rosai–Dorfman Disease

Chapter

Abstract

Rosai–Dorfman disease (RDD) is a benign non-Langerhans cell histiocytic disorder that usually presents with massive lymphadenopathy and sinus histiocytosis, although at least 40% of patients can have extranodal involvement. Recent studies found NRAS, KRAS, and ARAF mutations in a subset of patients with RDD, but no BRAF-V600E mutations have been reported yet. RDD is often associated with autoimmune or malignant disease, and rare cases of hereditary RDD have been reported as well. In most cases, RDD is self-limited and observation is the standard approach. However, 7–12% of patients die from the disease. Treatment is restricted to patients with life-threatening, multiple-relapsing, or autoimmune-associated disease.

Keywords

Rosai–Dorfman Sinus histiocytosis Massive lymphadenopathy S100 emperipolesis Treatment Classification 

References

  1. 1.
    Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy. Arch Pathol. 1969;87(1):63–70.PubMedGoogle Scholar
  2. 2.
    Destombes P. Ade’nites avec surcharge lipidique, de l’enfant ou de l’adulte jeune, observe’es aux Antilles et au Mali. Bull Soc Pathol Exot. 1965;58(6):1169–75.Google Scholar
  3. 3.
    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.PubMedGoogle Scholar
  4. 4.
    Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127(22):2672–81.PubMedPubMedCentralCrossRefGoogle Scholar
  5. 5.
    Mahzoni P, Zavareh MHT, Bagheri M, Neda Hani N, Moqtader B. Intracranial ROSAI-DORFMAN disease. J Res Med Sci. 2012;17(3):304–7.PubMedPubMedCentralGoogle Scholar
  6. 6.
    Warrier R, Chauhan A, Jewan Y, Bansal S, Craver R. Rosai-Dorfman disease with central nervous system involvement. Clin Adv Hematol Oncol. 2012;10(3):196–8.PubMedGoogle Scholar
  7. 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. 2013;36:1–5.Google Scholar
  8. 8.
    Paulli M, Bergamaschi G, Tonon L, et al. Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (rosai-dorfman disease). Br J Haematol. 1195;91(2):415–8.CrossRefGoogle Scholar
  9. 9.
    Dalia S, Sagatys E, Sokol L, Kubal T. Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Control. 2014;21(4):322–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Delacretaz F, Meuge-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.PubMedCrossRefGoogle Scholar
  11. 11.
    di Dio F, Mariotti I, Coccolini E, Bruzzi P, Predieri B, Lughetti L. Unusual presentation of Rosai-Dorfman disease in a 14-month-old Italian child: a case report and review of the literature. BMC Pediatr. 2016;16:62. doi: 10.1186/s12887-016-0595-9.PubMedPubMedCentralCrossRefGoogle Scholar
  12. 12.
    Vaiselbuh SR, Bryceson YT, Allen CE, Whitlock JA, Abla O. Updates on histiocytic disorders. Pediatr Blood Cancer. 2014;61(7):1329–35.PubMedCrossRefGoogle Scholar
  13. 13.
    Maric I, Pittaluga S, Dale JK, et al. Histologic features of sinus histiocytosis with massive lymphadenopathy in patients with autoimmune lymphoproliferative syndrome. Am J Surg Pathol. 2005;29(7):903–11.PubMedCrossRefGoogle Scholar
  14. 14.
    Lu D, Estalilla OC, Manning JT Jr, Medeiros LJ. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature. Mod Pathol. 2000;13(4):414–9.PubMedCrossRefGoogle Scholar
  15. 15.
    Long E, Lassalle S, Cheikh-Rouhou R, Hofman V, Lacour JP, Hofman P. Intestinal occlusion caused by Rosai-Dorfman disease mimicking colonic diverticulitis. Pathol Res Pract. 2007;203:233–7.PubMedCrossRefGoogle Scholar
  16. 16.
    Ambati S, Chamyan G, Restrepo R, et al. Rosai-Dorfman disease following bone marrow transplantation for pre-B cell acute lymphoblastic leukemia. Pediatr Blood Cancer. 2008;51(3):433–5.PubMedCrossRefGoogle Scholar
  17. 17.
    O'Malley DP, Duong A, Barry TS, et al. Co-occurrence of Langerhans cell histiocytosis and Rosai-Dorfman disease: possible relationship of two histiocytic disorders in rare cases. Mod Pathol. 2010;23:1616–23.PubMedCrossRefGoogle Scholar
  18. 18.
    Cohen-Aubart F, Haroche J, De Risi T, et al. La maladie de Rosai–Dorfman-Destombes est une histiocytose inflammatoire polymorphe: etude phenotypique multicentrique de 47 patients. Orale Communication at the 71st Congress of the Socie’te´ Nationale Franc¸aise de Medecine Interne (SNFMI). June 10–12, 2015. Besancon.Google Scholar
  19. 19.
    Chakraborty R, Hampton OA, Shen X, et al. Mutually exclusive recurrent somatic mutations in MAP2K1 and BRAF support a central role for ERK activation in LCH pathogenesis. Blood. 2014;124(19):3007–15.PubMedPubMedCentralCrossRefGoogle Scholar
  20. 20.
    Diamond EL, Durham BH, Haroche J, Yao Z, Ma J, Parikh SA, et al. Diverse and targetable kinase alterations drive histiocytic neoplasms. Cancer Discov. 2016;6(2):154–65.PubMedCrossRefGoogle Scholar
  21. 21.
    Zhao M, Li C, Zheng J, et al. Extranodal Rosai-Dorfman disease involving appendix and mesenteric nodes with protracted course: a report of a rare case lacking relationship to IgG4-related disease and review of the literature. Int J Clin Exp Pathol. 2013;6(11):2569–77.PubMedPubMedCentralGoogle Scholar
  22. 22.
    Wimmer DB, Ro JY, Lewis A, et al. Extranodal Rosai-Dorfman disease associated with increased numbers of immunoglobulin g4 plasma cells involving the colon: case report with literature review. Arch Pathol Lab Med. 2013;137(7):999–1004.PubMedCrossRefGoogle Scholar
  23. 23.
    Liu L, Perry AM, Cao W, et al. Relationship between Rosai-Dorfman disease and IgG4-related disease: study of 32 cases. Am J Clin Pathol. 2013;140(3):395–402.PubMedCrossRefGoogle Scholar
  24. 24.
    Menon MP, Evbuomwan MO, Rosai J, Jaffe ES, Pittaluga S. A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease? Histopathology. 2014;64(3):455–9.PubMedCrossRefGoogle Scholar
  25. 25.
    Iwabuchi H, Kakihara T, Tanaka A, Uchiyama M, Shibuya H, Umezu H. Congenital Rosai-Dorfman disease without lymphadenopathy. Pediatr Pathol Mol Med. 2003;22:399–403.PubMedCrossRefGoogle Scholar
  26. 26.
    Chow CP, Ho HK, Chan GCF, Cheung AN, Ha SY. Congenital Rosai-Dorfman disease presenting with anemia, thrombocytopenia and hepatomegaly. Pediatr Blood Cancer. 2009;52:415–7.PubMedCrossRefGoogle Scholar
  27. 27.
    Morgan NV, Morris MR, Cangul H, Gleeson D, Straatman-Iwanowska A, Davies N, et al. Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease. PLoS Genet. 2010;6(2):e1000833.PubMedPubMedCentralCrossRefGoogle Scholar
  28. 28.
    Avitan-Hersh E, Mandel H, Indelman M, et al. A case of H syndrome showing immunophenotype similarities to Rosai–Dorfman disease. Am J Dermatopathol. 2011;33(1):47–51.PubMedCrossRefGoogle Scholar
  29. 29.
    Colmenero I, Molho-Pessach V, Torrelo A, Zlotogorski A, Requena L. Emperipolesis: an additional common histopathologic finding in H syndrome and Rosai-Dorfman disease. Am J Dermatopathol. 2012;34(3):315–20.PubMedCrossRefGoogle Scholar
  30. 30.
    Rossbach HC, Dalence C, Wynn T, Tebbi C. Faisalabad histiocytosis mimics Rosai–Dorfman disease: brothers with lymphadenopathy, intrauterine fractures, short stature and sensorineural deafness. Pediatr Blood Cancer. 2006;47(5):629–32.PubMedCrossRefGoogle Scholar
  31. 31.
    Jaffe R. Histiocytic proliferations in childhood. In: Proytcheva M, editor. Diagnostic pediatric hematopathology. Illinois: Feinberg School of Medicine, Northwestern University, Cambridge University Press; 2011. p. 504–39.CrossRefGoogle Scholar
  32. 32.
    Picarsic J, Chikwava K. In: Hsi ED, editor. Disorders of histiocytes hematopathology. 3rd ed. Philadelphia: Elsevier Saunders; 2017. (in press).Google Scholar
  33. 33.
    Picarsic J, Jaffe R. Nosology and pathology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am. 2015;29(5):799–823.PubMedCrossRefGoogle Scholar
  34. 34.
    Utikal J, Ugurel S, Kurzen H, et al. Imatinib as a treatment option for systemic non-Langerhans cell histiocytoses. Arch Dermatol. 2007;143:736–40.PubMedCrossRefGoogle Scholar
  35. 35.
    Sodhi KS, Suri S, Nijhawan R, Kang M, Gautam V. Rosai-Dorfman disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy. Br J Radiol. 2005;78(933):845–7.PubMedCrossRefGoogle Scholar
  36. 36.
    Litzner BR, Subtil A, Vidal CI. Combined cutaneous Rosai-Dorfman disease and localized cutaneous Langerhans cell histiocytosis within a single subcutaneous nodule. Am J Dermatopathol. 2015;37(12):936–9.PubMedPubMedCentralCrossRefGoogle Scholar
  37. 37.
    Kuo TT, Chen TC, Lee LY, Lu PH. IgG4-positive plasma cells in cutaneous Rosai-Dorfman disease: an additional immunohistochemical feature and possible relationship to IgG4-related sclerosing disease. J Cutan Pathol. 2009;36(10):1069–73.PubMedCrossRefGoogle Scholar
  38. 38.
    Al-Khateeb TH. Cutaneous Rosai-Dorfman disease of the face: a comprehensive literature review and case report. J Oral Maxillofac Surg. 2016;74:528–40.PubMedCrossRefGoogle Scholar
  39. 39.
    Varan A, Sen H, Akalan N, et al. Pontine Rosai-Dorfman disease in a child. Child Nerv Syst. 2015;31:971–5.CrossRefGoogle Scholar
  40. 40.
    Ludemann W, Banan R, Samii A, et al. Cerebral Rosai-Dorfman disease. Child Nerv Syst. 2015;31:529–32.CrossRefGoogle Scholar
  41. 41.
    Gupta K, Bagdi N, Sunitha P, et al. Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child: a case report and review of the literature. Br J Radiol. 2011;84:e138–41.PubMedPubMedCentralCrossRefGoogle Scholar
  42. 42.
    Raslan O, Ketonen LM, Fuller GN, Schellingerhout D. Intracranial Rosai-Dorfman disease with relapsing spinal lesions. J Clin Oncol. 2008;26:3087–9.PubMedCrossRefGoogle Scholar
  43. 43.
    Zhu H, Qiu LH, Dou YE, et al. Imaging characteristics of Rosai-Dorfman disease in the central nervous system. Eur J Radiol. 2012;81:1265–72.PubMedCrossRefGoogle Scholar
  44. 44.
    Morandi X, Godey B, Riffaud L, et al. Isolated Rosai-Dorfman disease of the fourth ventricle. Case illustration. J Neurosurg. 2000;92:890.PubMedCrossRefGoogle Scholar
  45. 45.
    Konishi E, Ibayashi N, Yamamoto S, Scheithauer BW. Isolated intracranial Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). Am J Neuroradiol. 2003;24:515–8.PubMedGoogle Scholar
  46. 46.
    Andriko JAW, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol. 2001;14:172–8.PubMedCrossRefGoogle Scholar
  47. 47.
    Ramos AA, Vega MAA, Alles JVD, Garcia JA, Martinez AM. Multiple involvement of the central nervous system in Rosai-Dorfman disease. Pediatr Neurol. 2012;46:54–6.CrossRefGoogle Scholar
  48. 48.
    Petzold A, Thom M, Powell M, Plant GT. Relapsing intracranial Rosai-Dorfman disease. J Neurol Neurosurg Psychiatry. 2001;71:538–41.PubMedPubMedCentralCrossRefGoogle Scholar
  49. 49.
    Md T, Khair A, Begum F, Akhter S, Farooq S, Kamal M. Isolated intracranial Rosai-Dorfman disease. Case Reports in Neuro Med. 2016;1972594:4.Google Scholar
  50. 50.
    Tran HM, Chinichian S, Storkersen K, Tokuhara K. An unusual case of extranodal Rosai-Dorfman disease manifesting as an Epibulbar mass. Case Rep Ophthalmol. 2015;6:351–5.PubMedPubMedCentralCrossRefGoogle Scholar
  51. 51.
    Nalini A, Jitender S, Anantaram G, Santosh V. Rosai-Dorfman disease: case with extensive dural involvement and cerebrospinal fluid pleocytosis. J Neurol Sci. 2012;314:152–4.PubMedCrossRefGoogle Scholar
  52. 52.
    Yetsir S, Cekin E, Tosun F, Yildrim A. Rosai-Dorfman disease associated with neurosensorial hearing loss in two siblings. Int J Pediatr Otorino. 2004;68:1095–100.CrossRefGoogle Scholar
  53. 53.
    Ahsan SF, Madgy DN, Poulik J. Otolaryngologic manifestations of Rosai-Dorfman disease. Int J Pediatr Otorino. 2001;59:221–7.CrossRefGoogle Scholar
  54. 54.
    Kozak B, Talbott J, Uzelac A, Rehani B. Rosai-Dorfman disease isolated to the thoracic epidural spine. Radiol Case. 2015;9:6–16.Google Scholar
  55. 55.
    Chen CW, Kachramanoglou C, Revesz T, Choi D. Rosai-Dorfman disease presenting as a thoracic intradural extramedullary spinal tumor but without extraspinal manifestations. Acta Neurochir (Wein). 2012;154:367–8.CrossRefGoogle Scholar
  56. 56.
    Chen HH, Zhou SH, Wang SQ, Teng XD, Fan J. Factors associated with recurrence and therapeutic strategies for sinonasal Rosai-Dorfman disease. Head Neck. 2012;34:1504–13.PubMedCrossRefGoogle Scholar
  57. 57.
    Duan HG, Zheng CQ, Wang DH, et al. Extranodal sinonasal Rosai-Dorfman disease: a clinical study of 10 cases. Eur Arch Otorhinolaryngol. 2015;272:2313–8.PubMedCrossRefGoogle Scholar
  58. 58.
    Vujhini SK, Kolte SS, Satarkar RN, Srikanth S. Fine needle aspiration diagnosis of Rosai-Dorfman disease involving thyroid. J Cytol. 2012;29:83–5.PubMedPubMedCentralCrossRefGoogle Scholar
  59. 59.
    Ma YL, Liang ZP, Xu SE, et al. Rosai-Dorfman disease (RDD) in the paraglottic space: report of a case and review of the literature. Int J Clin Exp Pathol. 2015;8:13532–8.PubMedPubMedCentralGoogle Scholar
  60. 60.
    Wenig BM, Abbondazo SL, Childers EL, Kapadia SB, Heffner DR. Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck. Hum Pathol. 1993;24:483–92.PubMedCrossRefGoogle Scholar
  61. 61.
    El-Kersh K, Perez RL, Guardiola J. Pulmonary IgG4+ Rosai-Dorfman disease. BMJ Case Rep. 2013;2013:bcr2012008324. doi: 10.1136/bcr-2012-008324.PubMedPubMedCentralGoogle Scholar
  62. 62.
    Campana M, Goupil de Bouille J, De Muret A, et al. Pulmonary manifestations revealing Rosai-Dorfman disease. Sarcoidosis Vasc Diffuse Lung Dis. 2015;32:275–7.Google Scholar
  63. 63.
    Roberts SS, Attanoos RL. IgG4+ Rosai-Dorfman disease of the lung. Histopathology. 2010;56:662–4.PubMedCrossRefGoogle Scholar
  64. 64.
    Boissiere L, Patey M, Toubas O, et al. Tracheobronchial involvement of Rosai-Dorfman disease. Medicine. 2016;95:1–5.CrossRefGoogle Scholar
  65. 65.
    Pulsoni A, Anghel G, Falcucci P, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol. 2002;69(1):67–71.PubMedCrossRefGoogle Scholar
  66. 66.
    Ocheni S, Ibegbulam OG, Okafor OC, et al. Usefulness of oral corticosteroids in Rosai-Dorfman disease. Eur J Cancer Care (Engl). 2007;16:286–8.CrossRefGoogle Scholar
  67. 67.
    O’Gallagher K, Dancy L, Sinha A, Sado D. Rosai-Dorfman disease of the heart. Intractable Rare Dis Res. 2016;5:1–5.PubMedPubMedCentralCrossRefGoogle Scholar
  68. 68.
    Daruwalla V, Parekh K, Tahir H, Collins JD, Carr J. Extranodal Rosai-Dorfman disease involving the left atrium: cardiac MRI, CT, and PET scan findings. Case Rep Radiol. 2015;2015:753160.PubMedPubMedCentralGoogle Scholar
  69. 69.
    Krishnan A, Nassar A, Nieh P. Rosai-Dorfman disease presenting as extranodal mass. Urology. 2005;66:1317–9.CrossRefGoogle Scholar
  70. 70.
    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 the literature. Pan Afr Med J. 2016;24(64):1–5.Google Scholar
  71. 71.
    Sasaki K, Pemmaraju N, Westin JR, et al. A single case of Rosai-Dorman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single-agent cladribine. Front Oncol. 2014;297:1–3.Google Scholar
  72. 72.
    Rocken C, Wieker K, Grote HJ, Muller G, Franke A, Roessner A. Rosai-Dorman disease and generalized AA amyloidosis: a case report. Hum Pathol. 2000;31:621–4.PubMedCrossRefGoogle Scholar
  73. 73.
    Harik L, Nassar A. Extranodal Rosai-Dorfman disease of the kidney and coexistent poorly differentiated prostatic adenocarcinoma. Arch Pathol Lab Med. 2006;130:1223–6.PubMedGoogle Scholar
  74. 74.
    Yamada S, Uemura M, Tokumoto M, et al. Hypercalcemia induced by Rosai-Dorfman disease in a hemodialysis patient: histological evidence of Extrarenal Calcitriol overproduction. Intern Med. 2014;53:2783–7.PubMedCrossRefGoogle Scholar
  75. 75.
    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;21:376–81.PubMedPubMedCentralCrossRefGoogle Scholar
  76. 76.
    Izadyar S, Samiei F, Gholamrezanezhad A. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) – imaging manifestations of renal involvement. Nuclear Med Rev. 2014;17:44–6.CrossRefGoogle Scholar
  77. 77.
    Rodriguez Torres C, Fantova GR, Martinez IE, Castillo Escudero JJ, Arias CDA. Rosai-Dorfman disease with atypical intrascrotal involvement. Int J Urol. 2015;22:794–6.PubMedCrossRefGoogle Scholar
  78. 78.
    Flores-Carrillo VM, Santaella-Torres F, Sanchez-Martinez LC, et al. Rosai-Dorfman disease presented with involvement of the adrenal gland. A clinical case reported. Rev Med Inst Mex Seguro Soc. 2014;52:224–7.PubMedGoogle Scholar
  79. 79.
    Lauwers GY, Perez-Atayde A, Dorfman RF, Rosai J. The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. Hum Pathol. 2000;31:380–5.PubMedCrossRefGoogle Scholar
  80. 80.
    Baran B, Karaca C, Soyer OM, et al. Rosai-Dorfman disease with diffuse gastrointestinal involvement. Eur J Gastroenterol Hepatol. 2013;25:869–74.PubMedCrossRefGoogle Scholar
  81. 81.
    Ide M, Asao T, Yoshida T, et al. Rosai-Dorfman disease of the colon presented as small solitary polypoid lesion. Rare Tumors. 2010;31(2):e2.Google Scholar
  82. 82.
    Hollowood K, Stamp G, Zouvani I, Fletcher CD. Extranodal follicular dendritic cell sarcoma of the gastrointestinal tract. Morphologic, immunohistochemical and ultrastructural analysis of two cases. Am J Clin Pathol. 1995;103:90–7.PubMedCrossRefGoogle Scholar
  83. 83.
    Esquivel J, Krishnan J, Jundi M, et al. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: first case report. Hepatogasroenterol. 1999;46:1202–5.Google Scholar
  84. 84.
    Zivin SP, Atieh M, Mosier M, et al. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: second case report. J Gastrointest Surg. 2009;13:806–9.PubMedCrossRefGoogle Scholar
  85. 85.
    Podberezin M, Angeles R, Guzman G, Peace D, Gaitonde S. Primary pancreatic sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) an unusual extranodal manifestation clinically simulating malignancy. Arch Pathol Lab Med. 2010;134:276–8.PubMedGoogle Scholar
  86. 86.
    Romero Arenas MA, Singhi AD, Hruban RH, et al. Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence. J Gastrointest Cancer. 2012;43:626–9.PubMedPubMedCentralCrossRefGoogle Scholar
  87. 87.
    Shaikh F, Awan O, Mohiuddin S, Farooqui S, Kahn SA, McCartney W. 18F-FDG PET /CT imaging of extranodal Rosai-Dorfman disease with hepatopancreatic involvement: a pictorial and literature review. Cureus. 2015;7:e392. doi: 10.7759/cureus.392.PubMedPubMedCentralGoogle Scholar
  88. 88.
    Mantilla JG, Goldberg-Stein S, Wang Y. Extranodal Rosai-Dorfman disease. Clinicopathologic series of 10 patients with radiologic correlation and review of the literature. Am J Clin Path. 2016;145:211–21.PubMedCrossRefGoogle Scholar
  89. 89.
    Melikyan AC, Kovringina AM, Gilyazildinova EA, Gittis MK. A case of sinus histiocytosis with massive lymphadenopathy in a patient with diffuse large cell lymphoma and chronic hepatitis B infection. Ter Arch. 2012;84:66–70.Google Scholar
  90. 90.
    Kgomo MK, Elnagar AA, Jeske C, Nagel J. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and cirrhosis of the liver: a case report and literature review. S Afr Med J. 2016;106:463–4.CrossRefGoogle Scholar
  91. 91.
    Di Tommaso L, Rahal D, Bossi P, Roncalli M. Hepatic Rosai-Dorfman disease with coincidental lymphoma: report of a case. Int J Surg Pathol. 2010;18:540–3.PubMedCrossRefGoogle Scholar
  92. 92.
    Patel MH, Jambhekar KR, Pandey T, Ram R. A rare case of extranodal Rosai-Dorfman disease with isolated multifocal osseous manifestation. Indian J Radiol Imaging. 2015;25:284–7.PubMedPubMedCentralCrossRefGoogle Scholar
  93. 93.
    Tsang JS, Anthony MP, Wong MP, Wong CS. The use of FDG-PET/CT in extranodal Rosai-Dorfman disease of bone. Skelet Radiol. 2012;41:715–7.CrossRefGoogle Scholar
  94. 94.
    Deshayes E, Le Berre JP, Joanneau E, Vasiljevic A, Raverot G, Seve P. 18F-FDG PET/CT findings in a patient with isolated intracranial Rosai-Dorfman disease. Clin Nucl Med. 2013;38:e50–2.PubMedCrossRefGoogle Scholar
  95. 95.
    Albano D, Bosio G, Bertagna F. 18F-FDG PET/CT of Rosai-Dorfman disease. Clin Nucl Med. 2015;40:e40–2.CrossRefGoogle Scholar
  96. 96.
    Gupta P, Babyn P. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease. Pediatr Radiol. 2008;38:721–8. quiz 821-822PubMedCrossRefGoogle Scholar
  97. 97.
    Demicco EG, Rosenberg AE, Bjornsson J, et al. Primary Rosai-Dorfman disease of bone: a clinicopathological study of 15 cases. Am J Surg Pathol. 2010;34:1324–33.PubMedCrossRefGoogle Scholar
  98. 98.
    Paryani NN, Daugherty LC, O’Connor MI, Jiang L. Extranodal Rosai-Dorfman disease of the bone treated with surgery and radiotherapy. Rare Tumors. 2014;6(5531):132–4.CrossRefGoogle Scholar
  99. 99.
    Huang Q, Chang KL, Weiss LM. Extranodal Rosai-Dorfman disease involving the bone marrow: a case report. Am J Surg Pathol. 2006;30:1189–92.PubMedCrossRefGoogle Scholar
  100. 100.
    Huang BY, Liu HL, Yu CJ. Isolated Intramedullary spinal Rosai-Dorfman disease: a case report and literature review. World Neurosurg. 2016;88:694. e.11-694.e15PubMedCrossRefGoogle Scholar
  101. 101.
    Oner AY, Akpek S, Tali T. Rosai-Dorfman disease with epidural and spinal bone marrow involvement: magnetic resonance imaging and diffusion-weighted imaging features. Acta Radiol. 2007;48:331–4.PubMedCrossRefGoogle Scholar
  102. 102.
    Lima FB, Barcelos PS, Constâncio AP, Nogueira CD, Melo-Filho AA. Rosai-Dorfman disease with spontaneous resolution: case report of a child. Rev Bras Hematol Hemoter. 2011;33:312–4.PubMedPubMedCentralCrossRefGoogle Scholar
  103. 103.
    Purav P, Ganapathy K, Mallikarjuna VS, et al. Rosai-Dorfman disease of the central nervous system. J Clin Neurosci. 2005;12:656–9.PubMedCrossRefGoogle Scholar
  104. 104.
    Forest F, N'guyen AT, Fesselet J, et al. Meningeal Rosai-Dorfman disease mimicking meningioma. Ann Hematol. 2014;93:937–40.PubMedGoogle Scholar
  105. 105.
    Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR, et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore). 2014;93:165–75.CrossRefGoogle Scholar
  106. 106.
    Cooper SL, Chavis PS, Fortney JA, Watkins JM, Caplan MJ, Jenrette JM 3rd. A case of orbital Rosai-Dorfman disease responding to radiotherapy. J Pediatr Hematol Oncol. 2008;30:744–8.PubMedCrossRefGoogle Scholar
  107. 107.
    Maklad AM, Bayoumi Y, Tunio M, Alshakweer W, Dahar MA, Akbar SA. Steroid-resistant extranodal rosai-dorfman disease of cheek mass and ptosis treated with radiation therapy. Case Rep Hematol. 2013;2013:428297. doi: 10.1155/2013/428297.PubMedPubMedCentralGoogle Scholar
  108. 108.
    Toguri D, Louie AV, Rizkalla K, Franklin J, Rodrigues G, Venkatesan V. Radiotherapy for steroid-resistant laryngeal Rosai-Dorfman disease. Curr Oncol. 2011;18:e158–62.PubMedPubMedCentralCrossRefGoogle Scholar
  109. 109.
    Z'Graggen WJ, Sturzenegger M, Mariani L, Keserue B, Kappeler A, Vajtai I. Isolated Rosai-Dorfman disease of intracranial meninges. Pathol Res Pract. 2006;202:165–70.PubMedCrossRefGoogle Scholar
  110. 110.
    Shulman S, Katzenstein H, Abramowsky C, Broecker J, Wulkan M, Shehata B. Unusual presentation of Rosai-Dorfman disease (RDD) in the bone in adolescents. Fetal Pediatr Pathol. 2011;30:442–7.PubMedCrossRefGoogle Scholar
  111. 111.
    McPherson CM, Brown J, Kim AW, DeMonte F. Regression of intracranial rosai-dorfman disease following corticosteroid therapy. Case report. J Neurosurg. 2006;104:840–4.PubMedCrossRefGoogle Scholar
  112. 112.
    Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S. Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res. 2010;32:572–8.PubMedCrossRefGoogle Scholar
  113. 113.
    Petrushkin H, Salisbury J, O’Sullivan E. Intralesional steroid for orbital manifestations of Rosai-Dorfman disease. Clin Exp Ophthalmol. 2015;43:483–5.PubMedCrossRefGoogle Scholar
  114. 114.
    Ottaviano G, Doro D, Marioni G, et al. Extranodal Rosai-Dorfman disease: involvement of eye, nose and trachea. Acta Otolaryngol. 2006;126:657–60.PubMedCrossRefGoogle Scholar
  115. 115.
    Sakallioglu O, Gok F, Kalman S, et al. Minimal change nephropathy in a 7-year-old boy with Rosai-Dorfman disease. J Nephrol. 2006;19:211–4.PubMedGoogle Scholar
  116. 116.
    Saboo SS, Jagannathan JP, Krajewski KM, et al. Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery. J Clin Oncol. 2011;29:e772–5.PubMedCrossRefGoogle Scholar
  117. 117.
    Horneff G, Jurgens H, Hort W, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine. Med Pediatr Oncol. 1996;27:187–92.PubMedCrossRefGoogle Scholar
  118. 118.
    Scheel MM, Rady PL, Tyring SK, et al. Sinus histiocytosis with massive lymphadenopathy: presentation as giant granuloma annulare and detection of human herpesvirus 6. J Am Acad Dermatol. 1997;37:643–6.PubMedCrossRefGoogle Scholar
  119. 119.
    Jabali Y, Smrcka V, Pradna J. Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report. Int J Surg Pathol. 2005;13:285–9.PubMedCrossRefGoogle Scholar
  120. 120.
    Inoue S, Onwuzurike N. Venorelbine and methotrexate for the treatment of Rosai-Dorfman disease. Pediatr Blood Cancer. 2005;45(1):84–5.PubMedCrossRefGoogle Scholar
  121. 121.
    Arnao V, Riolo M, Savettieri G, Aridon P. Mercaptopurine treatment in an adult man with orbital and intracranial Rosai-Dorfman disease. Case Rep Neurol Med. 2016;2016:1030478. doi: 10.1155/2016/1030478. 5 pagesPubMedPubMedCentralGoogle Scholar
  122. 122.
    Rivera D, Pérez-Castillo M, Fernández B, Stoeter P. Long-term follow-up in two cases of intracranial Rosai–Dorfman disease complicated by incomplete resection and recurrence. Surg Neurol Int. 2014;5:30. doi: 10.4103/2152-7806.128003.PubMedPubMedCentralCrossRefGoogle Scholar
  123. 123.
    Visser J, Dyer MJ. Refractory, atypical Rosai-dorfman disease successfully treated with a Cytarabine-containing Langerhans cell Histiocytosis treatment regimen-a case report. Pediatr Blood Cancer. 2014;61(11):2142.Google Scholar
  124. 124.
    Liu P, Wang P, Du J, Zhang J. Successful treatment of refractory cutaneous Rosai-Dorfman disease with vincristine. J Dermatol. 2015;42:97–8.PubMedCrossRefGoogle Scholar
  125. 125.
    Nadal M, Kervarrec T, Machet MC, Petrella T, Machet L. Cutaneous Rosai-Dorfman disease located on the breast: rapid effectiveness of methotrexate after failure of topical corticosteroids. Acitretin and thalidomide. Acta Derm Venereol. 2015;95:758–9.PubMedCrossRefGoogle Scholar
  126. 126.
    Le Guenno G, Galicier L, Uro-Coste E, et al. Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system. J Neurosurg. 2012;117:486–9.PubMedCrossRefGoogle Scholar
  127. 127.
    Lohr HF, Godderz W, Wolfe T, et al. Long-term survival in a patient with Rosai-Dorfman disease treated with interferon-alpha. Eur J Cancer. 1995;31A:2427–8.PubMedCrossRefGoogle Scholar
  128. 128.
    Le Guenno G, Galicier L, Fieschi C, et al. Dramatic efficiency of pegylated interferon in sinus histiocytosis with massive lymphadenopathy. Br J Dermatol. 2011;164:213–5.PubMedCrossRefGoogle Scholar
  129. 129.
    Konca C, Ozkurt ZN, Deger M, et al. Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment. Int J Hematol. 2009;89:58–62.PubMedCrossRefGoogle Scholar
  130. 130.
    Aouba A, Terrier B, Vasiliu V, et al. Dramatic clinical efficacy of cladribine in Rosai-Dorfman disease and evolution of the cytokine profile: towards a new therapeutic approach. Haematologica. 2006;91(12 Suppl):ECR52.PubMedGoogle Scholar
  131. 131.
    Tasso M, Esquembre C, Blanco E, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine. Pediatr Blood Cancer. 2006;47:612–5.PubMedCrossRefGoogle Scholar
  132. 132.
    Simko SJ, Tran HD, Jones J, et al. Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai-Dorfman disease. Pediatr Blood Cancer. 2014;61:479–87.PubMedCrossRefGoogle Scholar
  133. 133.
    Petschner F, Walker UA, Schmitt-Graff A, et al. “Catastrophic systemic lupus erythematous” with Rosai-Dorfman sinus histiocytosis: successful treatment with anti-CD20/rituximab. Dtsch Med Wochenschr. 2001;126:998–1001.PubMedCrossRefGoogle Scholar
  134. 134.
    Pagel JM, Lionberger J, Gopal AK, et al. Therapeutic use of rituximab for sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Am J Hematol. 2007;82:1121–2.PubMedCrossRefGoogle Scholar
  135. 135.
    Appel S, Boehmler AM, Grunebach F, et al. Imatinib mesylate affects the development and function of dendritic cells generated from CD34 peripheral blood progenitor cells. Blood. 2004;103:538–44.PubMedCrossRefGoogle Scholar
  136. 136.
    Gebhardt C, Averbeck M, Paasch U, et al. A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol. 2009;145:571–4.PubMedCrossRefGoogle Scholar
  137. 137.
    Li X, Hong Y, An Q, et al. Successful treatment of Rosai-Dorfman disease with low-dose oral thalidomide. JAMA Dermatol. 2013;149:992–3.PubMedCrossRefGoogle Scholar
  138. 138.
    Wang F, Zhou H, Luo DQ, Han JD, Chen MK. Dermatoscopic findings in cutaneous Rosai-Dorfman disease and response to low-dose thalidomide. J Dtsch Dermatol Ges. 2014;12:350–2.PubMedGoogle Scholar
  139. 139.
    Chen E, Pavlidakey P, Sami N. Rosai-Dorfman disease successfully treated with thalidomide. JAAD Case Rep. 2016;2:369–72.PubMedPubMedCentralCrossRefGoogle Scholar
  140. 140.
    Rubinstein M, Assal A, Scherba M, et al. Lenalidomide in the treatment of Rosai Dorfman disease – a first in use report. Am J Hematol. 2016;91:E1. doi: 10.1002/ajh.24225.PubMedCrossRefGoogle Scholar
  141. 141.
    Cooper SL, Arceci RJ, Gamper CJ, Teachey DT, Schafer ES. Successful treatment of recurrent autoimmune cytopenias in the context of sinus histiocytosis with massive lymphadenopathy using sirolimus. Pediatr Blood Cancer. 2016;63:358–60.PubMedCrossRefGoogle Scholar
  142. 142.
    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.PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Pediatrics, Division of Hematology/OncologyHospital for Sick Children, University of TorontoTorontoCanada
  2. 2.Department of PathologyUniversity of Pittsburgh School of Medicine, Children’s Hospital of Pittsburgh of UPMCPittsburghUSA

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