Bi Y W, Cai R R, Wang S Y, Zhu X Z
Department of Pathology, Eye & ENT Hospital of Fudan University, Shanghai 200031, China.
Department of Pathology, Cancer Hospital of Fudan University, Shanghai 200032, China.
Zhonghua Yan Ke Za Zhi. 2021 Sep 11;57(9):689-695. doi: 10.3760/cma.j.cn112142-20201023-00702.
To investigate the clinicopathologic features and differential diagnosis of ocular Kimura disease (KD) and epithelioid hemangioma. It was a retrospective case series study. The data of 10 patients with ocular KD and 3 patients with ocular epithelioid hemangioma from the Pathology Department of Eye & ENT Hospital of Fudan University from January 2010 to December 2019 were retrospectively analyzed, including clinical manifestations, morphology and immunophenotypes. Among patients with ocular KD, there were 9 males and 1 female with an age from 7 to 75 years (mean, 30 years). There were 6 unilateral cases and 4 bilateral cases. The disease mainly involved the orbit in 3 patients, the lacrimal gland in 5 patients and the eyelid in 2 patients. The ophthalmic presentation included a palpable periorbital or eyelid mass with eyelid swelling and ptosis, proptosis and displacement of the eyeball, and ocular dysmotility. Three patients had a history of lymphadenopathy. The disease course ranged from 6 months to 7 years (mean, 34 months). All the patients had elevated peripheral blood eosinophilia. Three patients of ocular epithelioid hemangioma were all males with age from 25 to 60 years old. They were all unilateral cases with 1 right eye and 2 left eyes. The disease respectively involved eyelid and orbit, the eyebrow skin and the inner canthus skin. They presented with eyelid swelling, orbital mass or subcutaneous nodule for 5 months to 2 years. All patients (11 eyes) with KD underwent incisional or excisional biopsy. The histopathology revealed follicular hyperplasia of lymphoid tissue with active germinal centers in orbital fibroadipose tissue. There were massive interfollicular eosinophils with eosinophilic microabscesses. Some swelling endothelial cells of proliferating vessels were seen. All the 3 patients (3 eyes) with ocular epithelioid hemangioma underwent excision of the lesions. Histopathological examination showed proliferation of small and medium blood vessels. The vessels were lined by endothelial cells with abundant eosinophilic cytoplasm which protruded into the lumen. The endothelial cells were positive for CD31, factor Ⅷ-related antigen and E26 transformation-specific related gene immunohistochemically. There was a moderate amount of lymphocytes, plasma cells and eosinophils surrounding blood vessels without eosinophilic microabscess. Both ocular KD and epithelioid hemangioma are more commonly seen in males and share the common histopathological features of vascular proliferation, swelling endothelial cells and eosinophilic infiltration. KD is an allergic benign lymphoid tissue proliferation characteristic of massive eosinophilic infiltration, whereas epithelioid hemangioma is a benign neoplasm of blood vessels with plump and epithelioid endothelial cells. .
探讨眼部木村病（KD）和上皮样血管瘤的临床病理特征及鉴别诊断。这是一项回顾性病例系列研究。回顾性分析了2010年1月至2019年12月复旦大学附属眼耳鼻喉科医院病理科10例眼部KD患者和3例眼部上皮样血管瘤患者的数据，包括临床表现、形态学和免疫表型。眼部KD患者中，男性9例，女性1例，年龄7至75岁（平均30岁）。单侧病例6例，双侧病例4例。3例主要累及眼眶，5例累及泪腺，2例累及眼睑。眼部表现包括可触及的眶周或眼睑肿块伴眼睑肿胀和上睑下垂、眼球突出和移位以及眼球运动障碍。3例有淋巴结病病史。病程6个月至7年（平均34个月）。所有患者外周血嗜酸性粒细胞均升高。3例眼部上皮样血管瘤患者均为男性，年龄25至60岁。均为单侧病例，右眼1例，左眼2例。病变分别累及眼睑和眼眶、眉部皮肤和内眦皮肤。表现为眼睑肿胀、眶内肿块或皮下结节5个月至2年。所有KD患者（11只眼）均行切开或切除活检。组织病理学显示眼眶纤维脂肪组织中淋巴组织滤泡增生，生发中心活跃。滤泡间有大量嗜酸性粒细胞及嗜酸性微脓肿。可见一些增生血管的肿胀内皮细胞。3例眼部上皮样血管瘤患者（3只眼）均行病变切除。组织病理学检查显示中小血管增生。血管内衬有内皮细胞，胞质丰富嗜酸性，突向管腔。免疫组化显示内皮细胞CD31、Ⅷ因子相关抗原和E26转化特异性相关基因阳性。血管周围有中等量淋巴细胞、浆细胞和嗜酸性粒细胞，无嗜酸性微脓肿。眼部KD和上皮样血管瘤均多见于男性，具有血管增生、内皮细胞肿胀和嗜酸性浸润等共同组织病理学特征。KD是一种以大量嗜酸性浸润为特征的过敏性良性淋巴组织增生，而上皮样血管瘤是一种具有丰满上皮样内皮细胞的血管良性肿瘤。
