Zhang Wan-Lin, Ma Shuang, Jug Rachel, Li Fan, Wang Endi, Zhao Huan-Yu, Xu Hong-Tao, Cai Lin, Yu Cheng-Qian, Shen Shuai, Yang Lian-He
Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences, China Medical University.
Department of Neurology, Sheng Jing Hospital of China Medical University, Shenyang, Liaoning, China.
Medicine (Baltimore). 2018 Mar;97(12):e0181. doi: 10.1097/MD.0000000000010181.
Primary testicular natural killer (NK)/T-cell lymphoma is an extremely rare and highly aggressive lymphoid malignancy. At present, only 20 cases have been reported.
A 32-year-old Chinese man complained of discomfort and swelling of his right testicle for 3 months. Physical examination revealed a 10 × 10 × 9.5 cm mass on the right side of the scrotum area.
Pathologic evaluation showed effacement of normal testicular parenchymal architecture by small-to-medium-sized lymphoid cells with irregular nuclear profiles, and immunohistochemical studies positively expressed CD2, CD56, cytoplasmic CD3, granzyme B, perforin, and TIA-1. Therefore, the patient was diagnosed with primary testicular NK/T-cell lymphoma.
The patient underwent CHOP (cyclophosphamide (CTX), pirarubicin (THP-ADM), vincristine (VCR), and prednisolone (PDN)) chemotherapy.
The patient relapsed 5 months after his initial presentation and died after an infection and gastrointestinal bleed.
Clinicopathological assessment of this rare case highlights the clinical and pathological features required to diagnose testicular NK/T-cell lymphoma. In addition, it highlights the dismal survival of these patients. We hope it may serve as a reference aiding prompt clinical diagnosis, which can hopefully improve the survival and quality of life of these patients.
原发性睾丸自然杀伤(NK)/T细胞淋巴瘤是一种极其罕见且侵袭性很强的淋巴恶性肿瘤。目前,仅报道过20例。
一名32岁中国男性主诉右侧睾丸不适及肿胀3个月。体格检查发现阴囊区域右侧有一个10×10×9.5cm的肿块。
病理评估显示中小淋巴细胞使正常睾丸实质结构消失,细胞核形态不规则,免疫组化研究显示CD2、CD56、细胞质CD3、颗粒酶B、穿孔素和TIA-1呈阳性表达。因此,该患者被诊断为原发性睾丸NK/T细胞淋巴瘤。
患者接受了CHOP(环磷酰胺(CTX)、吡柔比星(THP-ADM)、长春新碱(VCR)和泼尼松龙(PDN))化疗。
患者在初次就诊后5个月复发,最终因感染和胃肠道出血死亡。
对该罕见病例的临床病理评估突出了诊断睾丸NK/T细胞淋巴瘤所需的临床和病理特征。此外,它还凸显了这些患者令人沮丧的生存率。我们希望它能作为参考,有助于临床快速诊断,有望提高这些患者的生存率和生活质量。