Rosai-Dorfman disease with exclusive nasal involvement: Case report

Authors

  • Isabel Pinto Serviço de Otorrinolaringologia do Centro Hospitalar Vila Nova de Gaia/Espinho
  • Sandra Gerós Serviço de Otorrinolaringologia do Centro Hospitalar Vila Nova de Gaia/Espinho
  • Maria Shekhovtsova Serviço de Anatomia Patológica do Centro Hospitalar Vila Nova de Gaia/Espinho
  • Diamantino Helena Serviço de Otorrinolaringologia do Centro Hospitalar Vila Nova de Gaia/Espinho
  • Artur Condé Serviço de Otorrinolaringologia do Centro Hospitalar Vila Nova de Gaia/Espinho

DOI:

https://doi.org/10.34631/sporl.774

Keywords:

Rosai-Dorfman Disease, Nose, Paranasal Sinuses

Abstract

Rosai-Dorfman disease is a histiocytosis classically characterized by painless cervical lymphadenopathy. Nevertheless, in 43% of cases it may involve extranodal sites. It is an entity with a high clinical variability that can evolve from a self-limited episode to death.

A 64-year-old female with complains of nasal obstruction presented on the physical examination a septum deviation and nasal polyposis grade III bilaterally. A computed tomography was requested and highlighted an almost complete opacification of the paranasal sinuses, as well as signs of bone erosion. The patient underwent endoscopic sinus surgery. The cyto-histopathological examination revealed Rosai-Dorfman disease. Laboratory tests and imaging study were performed and allowed the exclusion of primary pathology and other sites of the disease.

This case was particularly interesting because of the surgical challenge it posed, since the disease caused a significant alteration in the nasal anatomy.

References

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

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

-Oussama Abla et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood. 2018 Jun 28;131(26):2877-2890. doi: 10.1182/blood-2018-03-839753.

-Cocker RS, Kang J, Kahn LB. Rosai-Dorfman disease. Report of a case presenting as a midline thyroid mass. Arch Pathol Lab Med. 2003 Apr;127(4):e197-200. Doi: 10.1043/0003-9985(2003)1272.0.CO;2.

-Gaitonde S. Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: an overview. Arch Pathol Lab Med. 2007 Jul;131(7):1117-21. Doi: 10.1043/1543-2165(2007)131[1117:MESHWM]2.0.CO;2.

-Pettinato G, Manivel J, d'Amore ES, Petrella G. Fine needle aspiration cytology and immunocytochemical characterization of the histiocytes in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome). Acta Cytol. 1990 Nov-Dec;34(6):771-7.

Published

2020-03-10

How to Cite

Pinto, I., Gerós , S. ., Shekhovtsova, M., Helena , D. ., & Condé, A. (2020). Rosai-Dorfman disease with exclusive nasal involvement: Case report. Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery, 57(2), 75–78. https://doi.org/10.34631/sporl.774

Issue

Section

Case Report