A rare case study: Adult orbital xanthogranulomatous disease

ABSTRACT

This study contributes to the existing literature by exploring potential treatment options for the rare adult-onset subtype of orbital xanthogranulomatous disease, with the goal of improving diagnosis, management, and patient outcomes.

This case report presents a 28-year-old woman with bilateral periorbital swelling with prominent, firm yellow-orange lesions that had existed for over a year. These masses resulted in bilateral superior gaze restriction. Although high levels of rheumatoid factor were noted two months prior to surgery, all other biomarkers were within normal limits, which resulted in an inconclusive diagnosis until a biopsy of the mass was taken. The periorbital subcutaneous mass was treated surgically and excised successfully. Through histopathological examination, the presence of histiocytic infiltrate and multinucleated giant cells led to the diagnosis of adult orbital xanthogranulomatous disease (AOXD). Based on the histopathologic findings and absence of systemic involvement, the patient’s findings are consistent with adult-onset orbital xanthogranuloma (AOX) subtype. Adult-onset orbital xanthogranuloma is the rarest subtype of adult orbital xanthogranulomatous disease with varying treatment options, as there is no consensus on the most effective therapeutic course.

Keywords: Adult orbital xanthogranulomatous disease; histiocytes; multi-nucleated giant cells; histiocytic infiltrate