原发性眼附属器非霍奇金淋巴瘤的特征

Characteristics of ocular adnexal non-Hodgkin lymphoma

目的:探讨原发于眼附属器非霍奇金淋巴瘤的临床表现与病理特征。方法:对18例住院手术并经病理学检查确认为原发于眼附属器非霍奇金淋巴瘤患者的临床资料进行回顾性分析,对该病发生部位、临床及影像学表现、病理学特征进行总结。结果:全部患者均表现为眼部占位性病变,其中发生于眼眶者12例(含泪腺3例),占66.7%;发生于结膜者3例,占16.7%;发生于下睑者3例,占16.7%。全部病例中,8例患者被临床和影像学检查误诊为"炎性假瘤"。8例被诊为眼部占位性病变性质待查。经病理学检查,16例(88.9%)诊断为黏膜相关淋巴组织型的结外边缘带B细胞淋巴瘤,2例诊断为NK/T细胞性非霍奇金淋巴瘤。结论:原发于眼附属器的非霍奇金淋巴瘤主要表现为无痛性肿块,多发生于眼眶,临床和影像学确诊难度大,大多数糖皮质激素治疗有效,容易被误诊为"炎性假瘤"。黏膜相关淋巴组织B细胞淋巴瘤是最常见的病理类型。

Objective To investigate the clinical manifestation and pathological features of primary ocular adnexal non-Hodgkin lymphoma. Methods Data of 18 patients with biopsy-proven ocular adnexal non-Hodgkin lymphoma at Xiangya Hospital were reviewed. The disease site, clinical manifestation, imaging and pathological features of the tumor were summarized. Results All patients had typical presentation of an adnexal mass. Twelve （66. 7% ） patients had orbital involvement, 3 （16.7%） had conjunctival, and 3 （ 16.7 % ） had lymphoma involving the eyelids. Eight patients were misdiagnosed as ＂ inflammatory pseudotumour＂ before the operation according to their clinical and imaging examination, another 8 patients were diagnosed as ＂ocular adnexal turnout with unknown nature＂ before the operation. According to the pathologic diagnosis, 16 patients （ 88.9 % ） had marginal zone lymphomas of mucosa-associated lymphoid tissue （MZL-MALT） and 2 （11. 1% ） had NK/T-cell lymphoma. Conclusion The typical presentation of ocular adnexal lymphoma is a painless mass. Orbital connective tissue is the most involved anatomical site. The diagnosis of ocular adnexal non-Hodgkin lymphoma is difficult which could easily be misdiagnosed as ＂ inflammatory pseudotumour＂. MZL-MALT is the most common pathologic type.