Woog John J, Kim Yoon Duck, Yeatts R Patrick, Kim Stella, Esmaeli Bita, Kikkawa Don, Lee H B Harold, Korn Bobby S, Punja Karim, Habermann Thomas M, Colgan Joseph P, Salomao Diva, Cameron J Douglas
Mayo Clinic, Rochester, Minnesota, USA.
Ophthalmology. 2006 Jan;113(1):140-7. doi: 10.1016/j.ophtha.2005.09.036. Epub 2005 Dec 19.
To review the clinical, radiological, and histopathologic features in 8 patients with natural killer/T-cell lymphoma (NKTL) involving the orbit and/or ocular adnexa, and to describe the responses of these patients to various treatment regimens.
Retrospective observational case series.
Eight patients (5 male, 3 female) with NKTL involving the orbit and/or ocular adnexa were identified from 1999 through 2005. The mean age at presentation was 45 years (range, 26-65).
We retrospectively identified patients with NKTL of the ocular adnexa treated in the authors' medical centers from 1999 through 2004 using computerized diagnostic index retrieval. The clinical records and radiologic studies were analyzed to define modes of presentation and progression, response to therapy, and areas of anatomic involvement. Histopathologic findings, including the presence of CD3, CD56, and Epstein-Barr virus-encoded mRNA in each patient, were reviewed.
Time of survival from presentation to last known follow-up and tumor-related death.
Four of the 8 patients (50%) with NKTL involving the orbit or ocular adnexa had systemic involvement at presentation. Five of the 8 patients (62.5%) had concurrent sinonasal involvement, whereas 3 (37.5%) had orbital involvement alone. All lesions demonstrated CD3, CD56, and/or Epstein-Barr virus positivity on immunopathology studies. Therapy consisted of various chemotherapeutic regimens typically employed in the treatment of non-Hodgkins lymphoma, steroids, surgical intervention, and radiation. Seven (87.5%) patients died 5 weeks to 13 months after presentation, and 1 (12.5%) is alive without disease (5-year follow-up).
Natural killer/T-cell orbital lymphoma is a rare Epstein-Barr virus-associated neoplasm that may occur with or without associated sinonasal involvement. Our series, the largest cohort reported to date, demonstrates the high lethality of this condition despite aggressive conventional therapy, suggesting that new treatment options should be considered early in the course of treatment of patients with this disorder.
回顾8例累及眼眶和/或眼附属器的自然杀伤/T细胞淋巴瘤（NKTL）患者的临床、放射学及组织病理学特征，并描述这些患者对各种治疗方案的反应。
回顾性观察病例系列。
1999年至2005年期间确诊的8例累及眼眶和/或眼附属器的NKTL患者（5例男性，3例女性）。就诊时的平均年龄为45岁（范围26 - 65岁）。
我们通过计算机诊断索引检索，回顾性地确定了1999年至2004年在作者所在医疗中心接受治疗的眼附属器NKTL患者。分析临床记录和放射学研究，以确定临床表现和进展模式、对治疗的反应以及解剖学受累部位。回顾了组织病理学结果，包括每位患者中CD3、CD56和爱泼斯坦 - 巴尔病毒编码mRNA的存在情况。
从就诊到最后一次已知随访以及肿瘤相关死亡的生存时间。
8例累及眼眶或眼附属器的NKTL患者中，4例（50%）在就诊时已有全身受累。8例患者中有5例（62.5%）同时累及鼻窦，而3例（37.5%）仅累及眼眶。所有病变在免疫病理学研究中均显示CD3、CD56和/或爱泼斯坦 - 巴尔病毒阳性。治疗包括通常用于治疗非霍奇金淋巴瘤的各种化疗方案、类固醇、手术干预和放疗。7例（87.5%）患者在就诊后5周至13个月死亡，1例（12.5%）无病存活（5年随访）。
自然杀伤/T细胞眼眶淋巴瘤是一种罕见的与爱泼斯坦 - 巴尔病毒相关的肿瘤，可伴有或不伴有鼻窦受累。我们的系列研究是迄今为止报道的最大队列，表明尽管采用积极的传统治疗，这种疾病的致死率仍然很高，提示在这种疾病患者的治疗过程中应尽早考虑新的治疗选择。
