Marchino Tizana, Ibáñez Núria, Prieto Sebastián, Novelli Silvana, Szafranska Justyna, Mozos Anna, Graell Xavier, Buil José A
*Department of Ophthalmology, Santa Creu i Sant Pau Hospital; †Department of Orbital and Ophthalmic Plastic Surgery, Institut Català de Retina; ‡Department of Hematology and §Department of Pathology, Santa Creu i Sant Pau Hospital, Barcelona, Spain.
Ophthalmic Plast Reconstr Surg. 2014 Sep-Oct;30(5):e131-4. doi: 10.1097/IOP.0b013e3182a65026.
Natural killer/T-cell lymphoma (NKTCL) and its presentation with extranodal orbital involvement as a single lesion are extremely rare. The aim of this article was to describe the presentation, diagnosis, and systemic treatment of a primary orbital NKTCL. A 67-year-old Caucasian woman presented with left exophthalmos, pain, periorbital swelling, and limited extrinsic ocular motility. Orbital cellulitis was suspected, but finally orbital biopsy was performed due to no response to initial antibiotic and anti-inflammatory standard treatment. The pathologic diagnosis was NKTCL. Systemic evaluations were negative. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy was initiated, but after 2 cycles of treatment, tumoral progression was observed. SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) rescue chemotherapy was then administered. Lymphoma progression was inevitable. She died 10 months later. Although more nasal NKTCL cases have been described, the nonnasal primary orbital NKTCL is an uncommon neoplasm with high mortality rate, despite the recent use of more potent chemotherapy regimens.
自然杀伤/T细胞淋巴瘤（NKTCL）及其以结外眼眶受累作为单一病灶的表现极为罕见。本文旨在描述1例原发性眼眶NKTCL的表现、诊断及全身治疗情况。1例67岁白种女性患者，出现左眼突出、疼痛、眶周肿胀及眼球外展运动受限。最初怀疑为眼眶蜂窝织炎，但由于对初始抗生素及抗炎标准治疗无反应，最终进行了眼眶活检。病理诊断为NKTCL。全身评估结果为阴性。开始给予CHOP（环磷酰胺、多柔比星、长春新碱、泼尼松）化疗，但在2个周期治疗后，观察到肿瘤进展。随后给予SMILE（地塞米松、甲氨蝶呤、异环磷酰胺、L-天冬酰胺酶、依托泊苷）挽救化疗。淋巴瘤进展不可避免。10个月后患者死亡。尽管已有更多鼻型NKTCL病例的报道，但非鼻型原发性眼眶NKTCL是一种罕见肿瘤，尽管最近使用了更强效的化疗方案，其死亡率仍很高。
