Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease

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), r...

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Main Authors: Ying Zhao, Feng-Mei Cai, Hui Liu, Man Niu, Ling-Ran Zhi, Cui-Hong Liu, Ya-Lou Wang, Jia Wang, Hui-Fang Wang
Format: Article
Language:English
Published: Press of International Journal of Ophthalmology (IJO PRESS) 2022-01-01
Series:Guoji Yanke Zazhi
Subjects:
Online Access:http://ies.ijo.cn/cn_publish/2022/1/202201035.pdf
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author Ying Zhao
Feng-Mei Cai
Hui Liu
Man Niu
Ling-Ran Zhi
Cui-Hong Liu
Ya-Lou Wang
Jia Wang
Hui-Fang Wang
author_facet Ying Zhao
Feng-Mei Cai
Hui Liu
Man Niu
Ling-Ran Zhi
Cui-Hong Liu
Ya-Lou Wang
Jia Wang
Hui-Fang Wang
author_sort Ying Zhao
collection DOAJ
description 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.
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spelling doaj.art-e9e9d2f1dacc49b28ced9dfab81230d12022-12-21T18:44:15ZengPress of International Journal of Ophthalmology (IJO PRESS)Guoji Yanke Zazhi1672-51232022-01-0122115816210.3980/j.issn.1672-5123.2022.1.35202201035Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous diseaseYing Zhao0Feng-Mei Cai1Hui Liu2Man Niu3Ling-Ran Zhi4Cui-Hong Liu5Ya-Lou Wang6Jia Wang7Hui-Fang Wang8Pathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaEye Plastic Surgery and Orbital Disease Center, Xi'an People's Hospital(Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaPathological Diagnosis Center; Xi'an People's Hospital (Xi'an Fourth Hospital), Xi'an 710004, Shaanxi Province, ChinaAIM: 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.http://ies.ijo.cn/cn_publish/2022/1/202201035.pdfadult orbital xanthogranulomatous diseasenecrobiotic xanthogranulomaerdheim-chester disease
spellingShingle Ying Zhao
Feng-Mei Cai
Hui Liu
Man Niu
Ling-Ran Zhi
Cui-Hong Liu
Ya-Lou Wang
Jia Wang
Hui-Fang Wang
Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
Guoji Yanke Zazhi
adult orbital xanthogranulomatous disease
necrobiotic xanthogranuloma
erdheim-chester disease
title Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
title_full Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
title_fullStr Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
title_full_unstemmed Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
title_short Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
title_sort clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease
topic adult orbital xanthogranulomatous disease
necrobiotic xanthogranuloma
erdheim-chester disease
url http://ies.ijo.cn/cn_publish/2022/1/202201035.pdf
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