Orbital and Periorbital Epithelioid Hemangioma (Angiolymphoid Hyperplasia with Eosinophilia): A multicentre study of 25 cases.

PURPOSE: To characterize clinicoradiological features and outcomes of peri-orbital and orbital epithelioid hemangioma (EH). DESIGN: Retrospective, multicentre, observational case series SUBJECTS: Twenty-five (29 eyes) cases of biopsy-proven orbital and peri-orbital EH. METHODS: 25 cases (29 eyes) of orbital and peri-orbital EH managed across six tertiary centres over a 28-year period were reviewed. Previously reported radiological findings were pooled and compared with the present cohort. MAIN OUTCOME MEASURES: Clinicoradiological patterns, types of intervention, and treatment outcomes including completeness of excision and presence of tumor residue, in orbital and peri-orbital EH. RESULTS: The median age at presentation was 38 years (IQR: 18-49) with no gender predilection. Of the 21 (25 eyes) cases with imaging, orbital involvement predominated in 20 (80%) eyes, with most lesions located in the superior quadrant (n=14, 70%). Lacrimal gland (LG) was involved in 14/20 (70%) eyes. Extraocular muscles were involved in 10/20 (50%) eyes, the most common being the superior rectus-levator muscle complex (n=7, 35%). A 'hypervascular' phenotype was identified in 9/25 (36%) eyes, characterized by heterogenous contrast enhancement with internal flow voids on magnetic resonance imaging, and an intrinsic vascular blush on angiography. Interventions included excisional biopsy (n=13, 62%), debulking (n=3, 14%), incisional biopsy (n=5, 24%), and primary sclerotherapy (n=1, 5%). Excisional biopsy was curative (p=0.0005) with tumor residue being more common in infiltrative, ill-defined lesions on imaging (p=0.0196). CONCLUSION: Orbital and periorbital EH demonstrates a radiological fingerprint, characterized by superior orbital tropism with predilection for LG, with a 'hypervascular' radiological phenotype identified in one-third of cases. Complete excision, where feasible, remains curative.