Jiménez-Pérez Juan C, Jakobiec Frederick A, Zakka Fouad R, Yoon Michael K
*Ophthalmic Plastic and Reconstructive Surgery, Massachusetts Eye and Ear, †Department of Ophthalmology, Harvard Medical School, and ‡David G. Cogan Ophthalmic Pathology Laboratory, Massachusetts Eye and Ear, Boston, MA, U.S.A.
Ophthalmic Plast Reconstr Surg. 2017 Nov/Dec;33(6):e147-e150. doi: 10.1097/IOP.0000000000000889.
Primary orbital natural killer T-cell lymphoma (NKTCL) is a rare condition with only a few published cases in the literature. Over 1 month, an 81-year-old man developed progressive left periocular inflammation unresponsive to treatment. Clinical examination and imaging studies demonstrated a left lacrimal gland enlargement. Bilateral anterior uveitis and erythematous nontender cutaneous lesions were also found. Biopsies of the skin and lacrimal gland on the back revealed histopathologic and immunohistochemical findings confirming Epstein-Barr virus-positive NKTCL. Metastatic work up disclosed multifocal involvement in the pancreas, stomach, and chest wall. Palliative treatment consisting of nonanthracycline-based chemotherapy and radiation was instituted, but the patient died 5 months after the onset of symptoms. This is the first example of acute dacryoadenitis, and the second of bilateral anterior uveitis, in the setting of NKTCL. Absence of naso-sinus involvement in the current case is rare in NKTCL. Despite treatment, the prognosis remains dismal. Orbital specialists should include NKTCL in the differential diagnosis of lacrimal gland/orbital masses and perform an incisional biopsy if the clinical scenario so dictates.
原发性眼眶自然杀伤T细胞淋巴瘤（NKTCL）是一种罕见疾病，文献中仅有少数病例报道。在1个多月的时间里，一名81岁男性出现进行性左眼眶周围炎症，治疗无效。临床检查和影像学研究显示左侧泪腺肿大。还发现双侧前葡萄膜炎和非压痛性红斑皮肤病变。背部皮肤和泪腺活检的组织病理学和免疫组化结果证实为EB病毒阳性的NKTCL。转移检查发现胰腺、胃和胸壁多灶性受累。采用了以非蒽环类药物为基础的化疗和放疗的姑息治疗，但患者在症状出现后5个月死亡。这是NKTCL背景下急性泪腺炎的首个病例，也是双侧前葡萄膜炎的第二个病例。在当前病例中，鼻窦未受累在NKTCL中较为罕见。尽管进行了治疗，预后仍然很差。眼眶专科医生应将NKTCL纳入泪腺/眼眶肿块的鉴别诊断中，并根据临床情况进行切开活检。
