Abstract Listings 2025

Kimura’s disease: a mysterious orbital mass

Author: Estel·la Rojas Pineda
Base Hospital / Institution: Barraquer Ophthalmology Centre

ePoster presentation

Abstract ID: 25-146

Purpose

To present a rare entity of uncertain origin, with only about 200 histopathologically diagnosed cases reported worldwide.


Methods

Case report.


Results

A 40-year-old male from Saudi Arabia, with no relevant medical history, presented with a painless, slowly growing mass in the right upper eyelid. On examination, a large lesion was noted in the superomedial quadrant of the right orbit. It had smooth borders, a semi-soft consistency, was adherent to deep planes, and was non-tender to palpation. The lesion caused downward and outward displacement of the globe without diplopia. Exophthalmometry measured 24 mm in the right eye and 21 mm in the left. Examination of both the anterior and posterior segments was unremarkable. Visual acuity was 20/20 in both eyes.

Orbital MRI revealed a well-defined, round, 2.5 cm lesion, iso- to hyperintense, and heterogeneous. Surgical excision was performed via an anterior orbitotomy through an internal eyelid crease approach, and a biopsy was obtained.

Histopathological analysis showed capillary vessel proliferation with prominent endothelial cells, lymphoid aggregates, eosinophilic infiltrates, and scattered histiocytic cells. Immunohistochemistry was positive for CD1a, CD31, CD34, CD68, and S-100 protein, findings consistent with Kimura disease.


Conclusion

Kimura disease is a rare condition, more commonly affecting young males from East Asian countries. It involves the subcutaneous tissues and lymph nodes of the head and neck, including the eyelids and orbit. Diagnosis relies on imaging (MRI/CT) and histological confirmation. It may present with eosinophilia and elevated IgE levels. Up to 60% of cases are associated with nephrotic syndrome. The mainstay of treatment is surgical excision, though corticosteroids, immunosuppressants, or radiotherapy may also be used. Despite a recurrence rate of up to 40%, the overall prognosis is favorable.


Additional Authors

First name Last name Base Hospital / Institution
Cristina Arbó Bascuñana Barraquer Ophthalmology Centre
Sandra Planella Coll Barraquer Ophthalmology Centre

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