原发睾丸NK／T细胞淋巴瘤六例临床病理观察

Primary testicular NK/T cell lymphoma：a clinicopathologic analysis of six cases

目的探讨原发睾丸NK/T细胞淋巴瘤（NKTCL）病理学特征、诊断及预后。方法观察首都医科大学附属北京友谊医院2007年1月至2016年12月收集的6例睾丸NKTCL镜下形态及免疫表型，并进行临床随访，结合文献报道加以分析。结果本组睾丸NKTCL患者的中位年龄45岁（范围32～65岁），均以单侧睾丸肿大为首发症状（6/6），其中5例（5/6）发生在右侧；发病时病灶均局限于睾丸，处于Ann ArborⅠ期，但进展迅速，2例（2/6）于诊断后2个月死亡，3例（3/6）得到完全缓解，1例（1/6）失访。形态学上，肿瘤细胞弥漫浸润睾丸实质，围绕和浸润生精小管，均可见明显嗜血管生长模式，部分病例（4/6）可见坏死和核碎片。肿瘤细胞多以中等大小为主，胞质少或中等量，核型扭曲。免疫表型与鼻腔NKTCL相同，表达胞质型CD3、CD56、细胞毒性分子和EB病毒编码的小RNA（EBER），所有病例均可见CD5抗原的丢失。 结论原发睾丸NKTCL十分少见，临床呈高度侵袭性，尚无统一的治疗标准，预后差，诊断睾丸淋巴瘤时需考虑NKTCL的可能。

ObjectiveTo evaluate the clinicopathological features, diagnosis and management of primary testicular NK/T cell lymphoma （NKTCL）. MethodsSix cases of primary testicular NKTCL at Beijing Friendship Hospital, Capital Medical University from January 2007 to December 2016 were retrospectively analyzed for the morphology, immunephenotype and outcome, and relevant literature was reviewed. ResultsThe median age of patients at diagnosis was 45 years（range 32-65 years）. All patients presented with testicular masses as initial symptoms （6/6）, five cases （5/6） were on the right. The lesions were confined to the testis. All patients were classified as Ann Arbor stage Ⅰ but the tumors exhibited aggressive clinical behavior. Two patients died of the disease within two months, three （3/6） had clinical remission, and one （1/6） was lost to follow-up. Morphologically, the lymphoma cells showed a diffuse growth pattern that largely effaced the interstitial tissues, and surrounded seminiferous tubules in all cases. There was also a prominent angioinvasive pattern, with focal necrosis and karyorrhexis（4/6）. Cytologically, the medium-sized neoplastic cells showed scanty to moderate amount of cytoplasm and irregular folded nuclei. The immunophenotype was similar to that of nasal NKTCL： the neoplastic cells were positive for cytoplasmic CD3, CD56, cytotoxic molecules and EBV-encoded small RNA, the loss of CD5 antigen was seen in all cases. ConclusionsPrimary testicular NKTCL is extremely rare, highly aggressive and is associated with a poor prognosis. There is no unified standard of treatment. Thus, at the time of diagnosis of testicular lymphoma, NKTCL should be included in the differential diagnosis.