AIM: To examine the clinicopathological characteristics of adult orbital xanthogranulomatous disease(AOXGD).

METHODS: From January 2015 to January 2021, the researchers collected postoperative pathological diagnoses cases of AOXGD from Xi'an People's Hospital(Xi'an Fourth Hospital), retrospectively analyzed clinical pathological data, and reviewed related literature.

RESULTS: A total of five AOXGD cases were collected, including three cases of adult-onset xanthogranuloma(AOX), one case of necrobiotic xanthogranuloma(NBX), and one case of Erdheim-Chester disease(ECD). The five patients were composed of middle-aged and elderly patients with local orbital lesions were treated. Microscopic examination revealed that the orbital skin and subcutaneous tissue had foam-like non-Langerhans histiocytes that exhibited diffused or nested infiltration. Immunohistochemistry revealed that the cells were positive for CD68, CD163, FXⅢa, lysozyme, negative for S100, CD1a, Langerin, HMB-45, MDM2. One ECD patient had orbital lesions as the first symptom and exhibited a combination of retroperitoneal lesions, right atrium pseudotumor, and long bones, heart, kidney lesions. The diagnosis of AOXGD depends on clinical manifestations and pathological features. The treatment was based on subtypes and clinical manifestations to select corresponding strategies, which mainly include the administration of glucocorticoids, immunosuppressants, and surgical treatment.

CONCLUSION: AOXGD is relatively rare in clinical practice. AOXGD can manifest as a local orbital disease or an orbital disease combined with a systemic disease. In the clinical and pathological work of ophthalmology, the understanding and identification of this group of diseases should be strengthened, and correct diagnosis and standard treatment should be promoted.