Hostname: page-component-cd9895bd7-gbm5v Total loading time: 0 Render date: 2024-12-28T02:55:45.107Z Has data issue: false hasContentIssue false
  • English
  • Français

Polypoid intranasal mass caused by Rosai–Dorfman disease: a diagnostic pitfall

Published online by Cambridge University Press:  03 August 2009

M Ilie ,
N Guevara ,
L Castillo  and
P Hofman
M Ilie
Affiliation:
Clinical and Experimental Pathology Laboratory, Hôpital Louis Pasteur, Nice, France
N Guevara
Affiliation:
Department of Oto-Rhino-Laryngology, Hôpital Louis Pasteur, Nice, France
L Castillo
Affiliation:
Department of Oto-Rhino-Laryngology, Hôpital Louis Pasteur, Nice, France
P Hofman*
Affiliation:
Clinical and Experimental Pathology Laboratory, Hôpital Louis Pasteur, Nice, France
*
Address for correspondence: Dr Paul Hofman, Laboratoire de Pathologie Clinique et Expérimentale, 30 Avenue de la Voie Romaine, Hôpital Louis Pasteur, BP 69, 06002, Nice, France. Fax: +33 4 92 03 87 50 E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

Rosai–Dorfman disease is a rare, idiopathic, histiocytic proliferative disorder with a distinctive microscopic appearance, which was formerly thought to be a disease process limited to lymph nodes. However, extranodal involvement has been documented in less than half of the reported patients, but rarely without associated lymphadenopathy.

Case report:

We report the case of a 43-year-old Senegalese woman who presented with a polypoid, intranasal mass caused by Rosai–Dorfman disease. A diagnosis of a granulomatous process, including rhinoscleroma, was initially discussed. The correct diagnosis was made histologically by demonstrating aggregates of histiocytes with large amounts of cytoplasm, emperipolesis and protein S100 antigen expression. Despite using ancillary methods (molecular biology and electron microscopy), we failed to demonstrate any associated pathogen.

Conclusion:

Diagnosis of Rosai–Dorfman disease can be very difficult, in particular in adults from Africa with pure, isolated, intranasal localisation, in whom clinical and radiological features may mimic other infectious or neoplastic disorders. The diagnosis is made based on the histological presence of large histiocytes with lymphophagocytosis. Moreover, immunohistochemical analysis of these histiocytes using anti-protein S100 antibody shows strong positivity.

Keywords

Rosai-Dorfman DiseaseNasal PolypParanasal Sinuses
Type
Clinical Record
Information
The Journal of Laryngology & Otology , Volume 124 , Issue 3 , March 2010 , pp. 345 - 348
Copyright
Copyright © JLO (1984) Limited 2009

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Foucar, E, Rosai, J, Dorfman, RF, Eyman, JM.Immunologic abnormalities and their significance in sinus histiocytosis with massive lymphadenopathy. Am J Pathol 1984;82:515–25Google ScholarPubMed
2Foucar, E, Rosai, J, Dorfman, RF.Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7:19–73Google ScholarPubMed
3Montgomery, EA, Meis, JM, Frizzera, G.Rosai-Dorfman disease of soft tissue. Am J Surg Pathol 1992;16:122–9CrossRefGoogle ScholarPubMed
4Chang, Y-C, Tsai, M-H, Chen, C-L, Tsai, C-H, Lee, AY-S.Nasal Rosai-Dorfman disease with intracranial involvement: a case report. Am J Otolaryngol 2003;24:183–6CrossRefGoogle ScholarPubMed
5Dodson, KM, Powers, CN, Reiter, ER.Rosai-Dorfman disease presenting as synchronous nasal and intracranial masses. Am J Otolaryngol 2003;24:426–30CrossRefGoogle ScholarPubMed
6El-Banhawy, OA, Farahat, HG, El-Desoky, I.Facial asymmetry with nasal and orbital involvement in a case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Int J Pediatric Otorhinol 2005;69:1141–5CrossRefGoogle Scholar
7Faruk Unal, O, Koçan, EG, Sungur, A, Kaya, S.Rosai-Dorfman disease with multi-organ involvement in head and neck region. Int J Pediatric Otorhinol 2004;68:581–4CrossRefGoogle Scholar
8Goodnight, JW, Wang, MB, Sercarz, JA, Fu, YS.Extranodal Rosai-Dorfman disease of the head and neck. Laryngoscope 1996;106:253–6CrossRefGoogle ScholarPubMed
9Gregor, RT, Ninnin, D.Rosai-Dorfman of the paranasal sinuses. J Laryngol Otol 1994;108:152–5CrossRefGoogle ScholarPubMed
10Hagemann, M, Zbägren, P, Stauffer, E, Caversaccio, M.Nasal and paranasal sinus manifestation of Rosai-Dorfman disease. Rhinology 2005;43:229–32Google ScholarPubMed
11Ottaviano, G, Doro, D, Marioni, G, Mirabelli, P, Marchese-Ragona, R, Tognon, S et al. Extranodal Rosai-Dorfman disease: involvement of eye, nose and trachea. Acta Otolaryngol 2006;126:657–60CrossRefGoogle ScholarPubMed
12Wenig, BM, Abbondanzo, SL, Childers, EL, Kapadia, SB, Heffner, DR.Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck. Hum Pathol 1993;24:483–92CrossRefGoogle ScholarPubMed
13Yoon, AJ, Parisien, M, Feldman, F, Young-In Lee, F.Extranodal Rosai-Dorfman disease of bone, subcutaneous tissue and paranasal sinus mucosa with a review of its pathogenesis. Skeletal Radiol 2005;34:653–7CrossRefGoogle ScholarPubMed
14Schwartz, DA, Geyer, SJ. Klebsiella and rhinoscleroma. In: Connor, DH, Chandler, FW, Schwartz, DA, Manz, HJ, Lack, EE, eds. Pathology of Infectious Diseases. Stamford, Connecticut: Appleton & Lange, 1997:589–90Google Scholar
15Kasper, HU, Hegenbarth, V, Buhtz, P.Rhinoscleroma associated with Rosai-Dorfman reaction of regional lymph nodes. Pathol Int 2004;54:101–4CrossRefGoogle ScholarPubMed
16Chan, TV, Spiegel, JH.Klebsiella rhinoscleromatis of the membranous nasal septum. J Laryngol Otol 2007;121:998–1002CrossRefGoogle ScholarPubMed
17Delacretaz, F, Meuge-Moraw, C, Anwar, D, Borish, B, Chave, JP.Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a HIV positive patient. Virchows Arch 1991;419:251–4CrossRefGoogle Scholar
18Levine, PH, Jahan, N, Murari, P, Manak, M, Jaff, ES.Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 1992;166:291–5CrossRefGoogle ScholarPubMed