Chan J K, Tsang W Y, Lau W H, Cheung M M, Ng W F, Yuen W C, Ng C S
Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
Cancer. 1996 Mar 15;77(6):1198-205.
Nonnasal CD56+ T/natural killer (NK) cell lymphomas with morphologic and immunogenetic features similar to those of the distinctive nasal T/NK cell lymphoma are uncommon and have been characterized only recently. They show predominantly extranodal presentation, high stage disease, a highly aggressive course, strong association with Epstein-Barr virus (EBV), and lack of T cell receptor gene rearrangement. Only one previously reported case had a testicular presentation, although the testis is not uncommonly involved during the course of disease in both nasal and nonnasal T/NK cell lymphomas.
Three patients with T/NK cell lymphoma who presented initially with a testicular mass are reported.
The three patients underwent orchidectomy for testicular tumor. Histologically, the testes showed diffuse dense infiltration of medium-sized or large lymphoma cells. Antiocentric growth and necrosis were prominent in two cases. The lymphoma had the following immunophenotype: CD2+ CD3 epsilon+ CD56+ compatible with T/NK cell lymphoma; two lacked staining with Leu4 (CD3), and one had weak staining. With immunohistochemical preparations, it was noted that the rete testis stained consistently for CD56, and the Leydig cells and Sertoli cells showed patchy staining. The neoplastic cells harbored EBV, as demonstrated by in-situ hybridization. Additional sites of disease were detected at the time of the diagnosis in one patient (nose) or appeared soon afterwards in all three patients (skin or gastrointestinal tract). All three patients died within 5 months.
This study confirms that testicular CD56+ T/NK cell lymphoma tends to disseminate early, pursues an aggressive course, and is strongly associated with EBV. CD56 recognizes the neural cell adhesion molecule (NCAM), which exhibits homophilic binding properties. The expression of CD56 in the normal testicular constituents can perhaps explain the tendency for T/NK cell lymphoma to localize in this organ.
具有与独特的鼻型T/NK细胞淋巴瘤相似形态学和免疫遗传学特征的非鼻型CD56⁺T/自然杀伤（NK）细胞淋巴瘤并不常见，且直到最近才得以明确。它们主要表现为结外病变、疾病分期高、病程高度侵袭性、与EB病毒（EBV）密切相关以及缺乏T细胞受体基因重排。尽管在鼻型和非鼻型T/NK细胞淋巴瘤病程中睾丸受累并不罕见，但之前仅报道过1例睾丸受累的病例。
报道了3例最初表现为睾丸肿块的T/NK细胞淋巴瘤患者。
3例患者均因睾丸肿瘤接受了睾丸切除术。组织学上，睾丸显示中等大小或大的淋巴瘤细胞弥漫性致密浸润。两例可见明显的中心外生长和坏死。淋巴瘤具有以下免疫表型：CD2⁺、CD3ε⁺、CD56⁺，符合T/NK细胞淋巴瘤；两例缺乏Leu4（CD3）染色，1例染色较弱。通过免疫组化制备发现，睾丸网持续表达CD56，而睾丸间质细胞和支持细胞呈斑片状染色。原位杂交显示肿瘤细胞携带EBV。1例患者在诊断时发现有其他疾病部位（鼻），或在所有3例患者中随后不久出现其他疾病部位（皮肤或胃肠道）。所有3例患者均在5个月内死亡。
本研究证实睾丸CD56⁺T/NK细胞淋巴瘤往往早期播散，病程侵袭性强，且与EBV密切相关。CD56识别神经细胞黏附分子（NCAM），其具有同源性结合特性。正常睾丸组织中CD56的表达或许可以解释T/NK细胞淋巴瘤定位于该器官的倾向。
