眼部Kimura病和上皮样血管瘤的临床病理学特点及鉴别诊断

The clinicopathologic features and differential diagnosis of ocular Kimura disease and epithelioid hemangioma

目的探讨眼部Kimura病和上皮样血管瘤的临床病理学特点及鉴别诊断。方法回顾性系列病例研究。选取2010年1月至2019年12月复旦大学附属眼耳鼻喉科医院收治及会诊并经组织病理学诊断为眼部Kimura病的10例患者和上皮样血管瘤的3例患者的临床及病理学检查资料,对临床表现、实验室检查、病理形态学特点、免疫表型等进行分析。结果10例眼部Kimura病患者中男性9例,女性1例;年龄7~75岁,平均年龄30岁;单眼病变6例,双眼病变4例;病变累及眼眶软组织为主3例,累及泪腺为主5例,累及眼睑为主2例;主要临床表现为眶周或眼睑皮下扪及质韧肿物,可伴眼睑肿胀下垂及眼球突出移位,运动受限;3例曾有全身其他部位淋巴结肿大病史;病程6个月至7年,平均34个月;10例外周血嗜酸性粒细胞绝对值或百分比均升高。3例眼部上皮样血管瘤均为男性,均单眼发病(右眼1例,左眼2例),年龄25~60岁,病变累及眼睑和眼眶1例、眉弓及内眦部皮肤各1例,表现为眼睑肿胀、眼眶肿物或皮下结节,病程5个月至2年。10例(11只眼)眼部Kimura病患者行肿物切除或部分切除活检术,组织病理学检查均显示泪腺区或眼眶纤维肌肉脂肪组织内或眼睑皮下淋巴组织增生,反应性淋巴滤泡形成,内含活跃的生发中心,滤泡间及腺体周围大量嗜酸性粒细胞浸润,伴嗜酸性微脓肿形成,部分增生的血管内皮细胞肿胀。3例(3只眼)眼部上皮样血管瘤均行肿物切除术,组织病理学检查均显示小至中等大小血管增生,血管内衬胞质丰富红染的突入管腔的上皮样内皮细胞,均表达CD31、第八因子相关抗原、E26转录特异性相关基因等血管内皮标志物,血管周围可见中等量的淋巴细胞、浆细胞和嗜酸性粒细胞的浸润,但无嗜酸性微脓肿形成。结论眼部Kimura病和上皮样血管瘤均为男性好发,组织病理学形态均表现为血管增生、内皮肿胀及嗜酸性粒细胞浸润,但Kimura病是一种以大量嗜酸性粒细胞浸润为特征的良性淋巴组织增生,而上皮样血管瘤是一种以血管内皮显著肿胀为特征的良性血管性肿瘤,可以此进行鉴别诊断。(中华眼科杂志,2021,57:689-695)

Objective To investigate the clinicopathologic features and differential diagnosis of ocular Kimura disease(KD)and epithelioid hemangioma.Methods 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.Results 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,factorⅧ-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.Conclusions 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.(Chin J Ophthalmol,2021,57:689-695)