Zhang Jing, Chen Hui, Lin Wei, Wu Zhengzheng
Shenzhen Eye Hospital, Shenzhen Key Laboratory of Ophthalmology, Affiliated Shenzhen Eye Hospital of Jinan University, Shenzhen No. 18, Zetian Road, Futian District, Shenzhen 518040, Guangdong Province, China.
Department of Ophthalmology, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China Chengdu 610072, China.
Int J Clin Exp Pathol. 2020 May 1;13(5):1081-1085. eCollection 2020.
Natural killer T-cell lymphoma (NKTCL) is a highly aggressive tumor that usually affects the nasal cavity and/or paranasal sinuses. Primary orbital NKTCL is extremely rare, with only a few cases reported in the literature. The clinical presentation of orbital involvement by NKTCL is atypical and usually misdiagnosed as orbital cellulitis or orbital pseudotumor. A 23-year-old male patient was admitted to our hospital complaining of severe eye pain and manifested as acute orbital hemorrhage. Isolated orbital natural killer T-cell lymphoma (NKTCL) was confirmed by biopsy. This patient's orbital NKTCL did not respond to CHOP (cyclophosphamide, epirubicin, vincristine, prednisone) chemotherapy, but shrank significantly after receiving 1 cycle of C-SMILE (chidamide, steroid, methotrexate, isophosphamide, L-pegaspargase, etoposide). However, he still died after 3 cycles of C-SMILE chemotherapy at a follow-up time of 4 months. Primary orbital NKTCL can present clinically as a rare acute orbital hemorrhage, and the disease is aggressive and has a poor prognosis.
自然杀伤T细胞淋巴瘤（NKTCL）是一种侵袭性很强的肿瘤，通常累及鼻腔和/或鼻窦。原发性眼眶NKTCL极为罕见，文献中仅报道了少数病例。NKTCL累及眼眶的临床表现不典型，常被误诊为眼眶蜂窝织炎或眼眶假瘤。一名23岁男性患者因严重眼痛入院，表现为急性眼眶出血。经活检确诊为孤立性眼眶自然杀伤T细胞淋巴瘤（NKTCL）。该患者的眼眶NKTCL对CHOP（环磷酰胺、表柔比星、长春新碱、泼尼松）化疗无反应，但在接受1周期C-SMILE（西达本胺、类固醇、甲氨蝶呤、异环磷酰胺、聚乙二醇天冬酰胺酶、依托泊苷）化疗后显著缩小。然而，在随访4个月时，他在接受3周期C-SMILE化疗后仍死亡。原发性眼眶NKTCL临床上可表现为罕见的急性眼眶出血，该疾病具有侵袭性且预后较差。
