Abstract Listings 2025

Purpose

The aim is to provide a case series and a review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities.

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Methods

We present a review of the literature and our case series of four patients diagnosed and treated from 2001 – 2023 in our center. Clinical files of patients with adult-onset xanthogranulomatous disease of the orbit and ocular adnexa (AOXGD) were reviewed retrospectively. Clinical, laboratory, radiological, histopathological, and immunohistochemical findings were reexamined.

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Results

Reviewing medical records of our patients with AOXGD we found significant overlap between histiocytosis and different immune disorders. A broad workup should be considered in these patients as they can harbour severe immune disfunctions and hematologic disorders. Preferred treatment modality depends on a histopathologic type of AOXGD, clinical presentation and systemic involvement and should be conducted multidisciplinary.

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Conclusion

Diagnosing AOXGD can often be delayed because of its rarity and diverse clinical findings. Development of molecular genetic tests, detection of BRAF V600E mutation, different types of kinase mutations and mutations in transcriptional regulatory genes have shed a new light on the etiopathogenesis and potential targeted treatment.

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Additional Authors

First name	Last name	Base Hospital / Institution
Maja	Bakula	University hospital center Zagreb
Lorena Karla	Šklebar	University hospital center Zagreb, Medical School, University of Zagreb
Antonia	Jakovčević	Medical School, University of Zagreb
Krešimir	Mandić	University hospital center Zagreb, Medical School, University of Zagreb
Jasenka	Petrović Jurčević	Private Eye Clinic, Ivanić Grad
Ivan	Padjen	University hospital center Zagreb, Medical School, University of Zagreb

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