Histiocytosis and adult-onset orbital xanthogranuloma in 2023: a review of the literature and mini case series.

Purpose: Within the large umbrella of histiocytosis are a few similar yet heterogenous entities involving the orbit and periocular tissues with or without systemic infiltration, termed adult onset xanthogranuloma or orbital xanthogranuloma. Due to rarity of these conditions, different classifications in use, diverse clinical presentations and still unknown etiology, the aim of this paper was to provide an up-to-date literature review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities.

Methods: We present a review of literature and small case series comprising four patients diagnosed and treated in the period from 2001 until 2023 in our hospital.