Rosai-Dorfman Disease: Report of a 3-Year-Old Girl with Critical Review of Treatment Options

 

 Buy Article Permissions and Reprints

Abstract

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a newer pathological entity described in 1969. It is a self-limiting disorder of unknown aetiology. It is likely to be mistaken for lymphoma. It predominantly affects children and adolescents. However there are no publications on this disorder in the paediatric surgical literature. In this report we describe a 3-year-old girl who presented with this disease and we critically review the therapeutic options available for children. Prednisolone therapy with long-term follow-up appears to be sufficient. Surgery should be limited to biopsy and relief of compression symptoms.

Résumé

La maladie de Rosai-Dorfman (histiocytose sinusale avec lymphadénopathie massive) est une nouvelle entité pathologique décrite en 1969. Il s'agit d'un trouble spontanément régressif sans étiologie connue pouvant suggérer un lymphome, généralement observée chez les enfants ou les adolescents. Nous n'avons pas pu trouver une autre description dans la littérature pédiatrique. Nous présentons le cas d'une fillette de 3 ans qui présentait une maladie de Rosai-Dorfman et discutons des options thérapeutiques possibles chez l'enfant. La prescription de prédnisolone à long terme semble être suffisante. Une intervention chirurgicale serait limitée à une biopsie et une décompression.

Resumen

La Enfermedad de Rosai-Dorfman (Linfadenopatía masiva con histiocitosis sinusal) es una enfermedad nueva descrita en 1969. Se trata de una enfermedad autocontenida de etiología desconocida. Es fácilmente confundida con linfoma. Afecta fundamentalmente a niños y a adolescentes pero no hay publicaciones de esta enfermedad en la literatura quirúrgica pediátrica. En este trabajo describimos una niña de 3 años que se presentó con esta enfermedad y revisamos críticamente las opciones terapéuticas aplicables a niños. El tratamiento con Prednisolona y seguimiento a largo plazo parece ser suficiente. Debe limitarse la cirugía a la biopsia para aliviar los síntomas de compresión.

Zusammenfassung

Die Rosai-Dorfmansche Erkrankung (Sinus-Histiozytose mit massiver Lymphadenopathie) wurde erstmals 1969 beschrieben. Es ist eine selbstbegrenzende Erkrankung unklarer Ätiologie. Häufig wird sie als Lymphom fehldiagnostiziert. Im Wesentlichen betrifft sie Kinder und Jugendliche. In der kinderchirurgischen Literatur finden sich keine Publikationshinweise. Aus diesem Grunde wird der Fall eines dreijährigen Mädchens mit dieser Erkrankung vorgestellt und die therapeutischen Möglichkeiten diskutiert. Prednison zeigt befriedigende Langzeiterfolge. Die Chirurgie sollte auf eine Biopsie oder die Beseitigung von Kompressionssymptomen begrenzt bleiben.

References

• 1 Allen M R, Ninfo V, Viglio A, D'Angelo P, Paulli M, Arico M. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a girl previously affected by acute lymphoblastic leukemia.  Med Pediatr Oncol. 2001;  37 150-152
  CrossrefPubMedGoogle Scholar
• 2 Antonius J I, Farid S M, Baez-Giangreco A. Steroid-responsive Rosai-Dorfman disease.  Pediatr Hematol Oncol. 1996;  13 563-570
  PubMedGoogle Scholar
• 3 Baildam E M, Ewing C I, D'Souza S W, Stevens R F. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to acyclovir.  J R Soc Med. 1992;  85 179-180
  PubMedGoogle Scholar
• 4 Deshpande V, Verma K. Fine needle aspiration (FNA) cytology of Rosai Dorfman disease.  Cytopathology. 1998;  9 329-335
  CrossrefPubMedGoogle Scholar
• 5 Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.  Semin Diagn Pathol. 1990;  7 19-73
  PubMedGoogle Scholar
• 6 Foucar E, Rosai J, Dorfman R F. Sinus histiocytosis with massive lymphadenopathy. An analysis of 14 deaths occurring in a patient registry.  Cancer. 1984;  54 1834-1840
  PubMedGoogle Scholar
• 7 Govender D, Chetty R. Inflammatory pseudotumour and Rosai-Dorfman disease of soft tissue: a histological continuum?.  J Clin Pathol. 1997;  50 79-81
  PubMedGoogle Scholar
• 8 Komp D M. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).  Semin Diagn Pathol. 1990;  7 83-86
  PubMedGoogle Scholar
• 9 Lai F M, To K F, Szeto C C, Wang A Y, Ahuja A T, Choi P C, Leung C B, Kew J, Li P K. Acute renal failure in a patient with Rosai-Dorfman disease.  Am J Kidney Dis. 1999;  34 e12
  PubMedGoogle Scholar
• 10 Levine P H, Jahan N, Murari P, Manak M, Jaffe E S. Detection of human herpes virus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).  J Infect Dis. 1992;  166 291-295
  PubMedGoogle Scholar
• 11 Levine E A, Landry M M. Rosai Dorfman disease of soft tissue.  Surgery. 1994;  115 650-652
  PubMedGoogle Scholar
• 12 Lossos I S, Okon E, Bogomolski-Yahalom V, Ron N, Polliack A. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a patient with isolated renotesticular involvement after cure of Non-Hodgkin's lymphoma.  Ann Hematol. 1997;  74 41-44
  CrossrefPubMedGoogle Scholar
• 13 Lu D, Estalilla O C, Manning Jr J T, Medeiros L J. 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;  13 414-419
  CrossrefPubMedGoogle Scholar
• 14 Maia D M, Dorfman R F. Focal changes of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with nodular lymphocyte predominant Hodgkin's disease.  Hum Pathol. 1995;  26 1378-1382
  CrossrefPubMedGoogle Scholar
• 15 Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, Villiva N, Mandelli F. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review.  Am J Hematol. 2002;  69 67-71
  CrossrefPubMedGoogle Scholar
• 16 Rocken C, Wieker K, Grote H J, Muller G, Franke A, Roessner A. Rosai-Dorfman disease and generalized AA amyloidosis: a case report.  Hum Pathol. 2000;  31 621-624
  CrossrefPubMedGoogle Scholar
• 17 Rosai J, Dorfman R F. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity.  Arch Pathol. 1969;  87 63-70
  PubMedGoogle Scholar
• 18 Rosai J, Dorfman R F. Sinus histiocytosis with massive lymphadenopathy: a pseudolymphomatous benign disorder. Analysis of 34 cases.  Cancer. 1972;  30 1174-1188
  CrossrefPubMedGoogle Scholar
• 19 Tsang W Y, Yip T T, Chan J K. The Rosai-Dorfman disease histiocytes are not infected by Epstein-Barr virus.  Histopathology. 1994;  25 88-90
  PubMedGoogle Scholar
• 20 Wright D H, Richards D B. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case with widespread nodal and extra nodal dissemination.  Histopathology. 1981;  5 697-709
  PubMedGoogle Scholar
• 21 Wu M, Anderson A E, Kahn L B. A report of intracranial Rosai-Dorfman disease with literature review.  Ann Diagn Pathol. 2001;  5 96-102
  CrossrefPubMedGoogle Scholar

V. Raveenthiran

7. Medical Faculty Quarters

Annamalai Nagar 608002 Tamilnadu

India

Email: vrthiran@yahoo.co.in