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酷似海绵状血管瘤的罗萨伊-多夫曼病:一例报告

Rosai-Dorfman disease mimicking cavernous hemangioma: a case report.

作者信息

Sarmiento Ana Margarita A, Parulan Mariel Angelou A, Agbay Rose Lou Marie C

机构信息

Ophthalmology, Quirino Memorial Medical Center, Quezon City, Philippines.

Department of Laboratory Medicine and Pathology, The Medical City, Pasig City, Philippines.

出版信息

Orbit. 2023 Dec;42(6):612-616. doi: 10.1080/01676830.2022.2055086. Epub 2022 Apr 25.

Abstract

A 31-year-old female presented to the clinic with a 2 year history of proptosis of the right eye. She was diagnosed with cavernous hemangioma during her initial ophthalmologic consult and was advised monitoring. However, after a considerable increase in proptosis, she underwent an excision biopsy of the right orbital mass. A diagnosis of Rosai Dorfman disease was made after the histopathology report revealed a fibrosclerotic tissue with histiocytic proliferation showing emperipolesis that is mixed with numerous small lymphocytes and plasma cells. Rosai Dorfman disease is a rare disease presenting with lymphadenopathy and sinus histiocytosis. Orbital involvement can be the principal manifestation with proptosis as the most common presentation. Resection of the orbital lesion helped in the resolution of the mild ophthalmic symptoms but since the disease has other systemic associations, a complete systemic workup should be done to monitor recurrence.

摘要

一名31岁女性因右眼突出2年就诊于诊所。她在初次眼科会诊时被诊断为海绵状血管瘤,并被告知进行观察。然而,在眼球突出明显加重后,她接受了右眼眶肿物的切除活检。组织病理学报告显示为纤维硬化组织,伴有组织细胞增生并可见血细胞吞噬现象,同时混有大量小淋巴细胞和浆细胞,最终诊断为罗萨伊-多夫曼病。罗萨伊-多夫曼病是一种罕见疾病,表现为淋巴结病和窦性组织细胞增生。眼眶受累可能是主要表现,眼球突出是最常见的症状。眼眶病变切除有助于缓解轻度眼部症状,但由于该疾病还有其他全身关联,应进行全面的全身检查以监测复发情况。

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