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.
Key words
Rosai-Dorfman disease - sinus histiocytosis with massive lymphadenopathy - pseudolymphoma - lymph node enlargement
Mots-clés
Maladie de Rosai-Dorfman - histocytose sinusale avec lymphadénopathie massive - pseudolymphome - lymphadénopathie
Palabras clave
Enfermedad de Rosai-Dorfman - linfadenopatía masiva con histiocitosis sinusal - pseudolinfoma - adenopatía
Schlüsselwörter
Rosai-Dorfmansche Erkrankung - massive Lymphadenopathie - Sinus-Histiozytose - Pseudolymphoma - Therapie
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V. Raveenthiran
7. Medical Faculty Quarters
Annamalai Nagar 608002 Tamilnadu
India
Email: vrthiran@yahoo.co.in
