眼眶自然杀伤-T细胞淋巴瘤的临床及病理观察

摘要

Objective To investigate clinical manifestation,diagnosis and treatment of orbital natural killer(NK)-T cell lymphoma.Methods It was a retrospective case series.Seven orbital NK-T cell lymphoma patients confirmed by surgical biopsies were collected during the past 22 years.We reviewed the records,surgical and treatment procedures.Surgical specimens were studied with HE staining,immunohistochemical staining and molecular biological analysis.Results These patients had proptosis,eye motive inhibition or fixation and visual acuity was decreased or even without light perception.Skin of inner canthus and eyelids appeared red and swollen,with ulceration and cavity formation.CT scan revealed that the tumor showed uneven density and an unclear border.Tremendous lymphocyte infiltration and tissue necrosis in the tumor were observed in the biopsy tissue.LCA,CD45RO and CD57 immunohistochemical staining revealed positive results.Clonal T-cell-receptor gene rearrangements of two patients showed negative results and the Epstein-Barr virus was detected.Conclusions Orbital NK-T cell lymphoma is a rare disease.The characteristics of this disease include a highly aggressive clinical course.severe destruction and a poor prognosis.The final diagnosis depends on HE staining,immunohistochemical staining and molecular biological examination.%目的 探讨眼眶自然杀伤(NK)-T细胞淋巴瘤患者的临床表现、诊断和治疗方法.方法 为回顾性系列病例研究.收集1982年至2004年间7例住院手术、经病理检查证实为眼眶NK-T细胞淋巴瘤患者的临床和病理检查资料,包括手术方式、治疗方法、肿瘤病理标本的HE、免疫组织化学染色和分子生物学检查结果.结果 7例患者均表现为眼球突出、活动受限或固定,视力下降或失明,内眦部和眼睑皮肤红肿、溃烂和腔洞形成,CT检查可见眶内侧有边界欠清、密度不均的肿块.病理活检可见肿瘤内有大量淋巴细胞浸润和坏死组织,CD3、CD45、RO、CD57及Ki-67免疫组织化学染色阳性.2例患者T细胞受体基因重排呈阴性表达.而EB病毒的mRNA检查呈阳性.眶内肿瘤切除、局部放射及全身化学治疗效果均欠佳.结论 眼眶NK-T细胞淋巴瘤少见,其特点是发病急,病变进展快且破坏性大,预后差.最后确诊需借助于组织病理学、免疫组织化学或分子生物学检查结果.