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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:498-500, March 2004
© 2004 American Society of Neuroradiology

Case Report
HEAD AND NECK

Lacrimal Location of Sinus Histiocytosis (Destombes-Rosai-Dorfman Disease)

Robin Azoulay^a, Hervé Brisse^b, Paul Fréneaux^c, Solène Ferey^a, Gabriel Kalifa^a and Catherine Adamsbaum^a

^a Department of Pediatric Radiology, Hôpital St-Vincent-de Paul
^b Department of Radiology, Institut Curie, Paris, France
^c Department of Pathology, Institut Curie, Paris, France

Address reprint requests to Catherine Adamsbaum, Hôpital St-Vincent-de-Paul, 82, Avenue Denfert-Rochereau, 75674 Paris, France

Summary: A 7-year-old West African male patient presented with recurrence of multiple cervical lymphadenopathy. Cervical node biopsy disclosed a sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman-Destombes disease). During follow-up, the patient developed a left orbital mass corresponding to an enlarged lacrimal gland shown by CT. The histologic features of the gland were consistent with the diagnosis, and with steroid treatment, the gland decreased in size. Although rare, the diagnosis of sinus histiocytosis with massive lymphadenopathy has to be considered in cases of lacrimal gland enlargement.

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