ABSTRACT
@#AIM: To examine the clinicopathological characteristics of adult orbital xanthogranulomatous disease(AOXGD).<p> 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. <p>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. <p>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.
ABSTRACT
·Adult orbital xanthogranulomatous disease is a group of rare orbital and ocular adnexal disorders, which is classified as class II non - Langerhans histiocytic proliferations. This disease can be classified into 4 subtypes based mainly on systemic involvement: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease and adult-onset asthma and periocular xanthogranuloma. Diagnosis depends on its characteristic clinical manifestations and pathologic features. Therapeutic approaches are derived from anecdotal evidences, which include corticosteroid, immunosuppressive agent, surgical debulking and chemotherapy. The management of this disease varies with different subtypes, as well as associated systemic presentations. This review summarizes advances of etiopathogenesis, clinical features, diagnosis and treatment.
ABSTRACT
PURPOSE: To report a case of adult orbital xanthogranulomatous disease presented as bilateral swelling and yellowish eyelids in a 42-year-old woman who was misdiagnosed with xanthelasma at a dermatologic clinic. CASE SUMMARY: A 42-year-old woman presented yellowish and swollen eyelids in both eyes of 2.5 year duration. She had no past history of systemic diseases or other ophthalmologic problems. MRI showed heterogeneous eyelid masses and hypertrophic changes of the lacrimal glands in both eyes. There were no abnormalities on chest X-ray exams. The blood cholesterol and low density lipoprotein levels were increased. Incisional biopsy showed many foamy histiocytes, lymphocytes with germinal centers, several touton giant cells, and negative S100/CD 1 staining; all being features consistent with adult onset xanthogranulomatous disease. CONCLUSIONS: In cases with bilateral swelling and yellowish colored eyelids, adult orbital xanthogranulomatous disease should be evaluated through biopsy and other systemic examinations.