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目的：总结、归纳睾丸鞘膜恶性间皮瘤的临床特点及诊治方法，提高早期诊断及治疗的意识。方法报告1例睾丸鞘膜恶性间皮瘤患者，结合相关文献探讨睾丸鞘膜恶性间皮瘤临床表现及病理特点。结果行右侧睾丸、睾丸鞘膜及部分精索切除术，术后病理示：右侧睾丸恶性肿瘤，考虑来源于间皮。术后2个月随访患者已死亡。结论睾丸鞘膜恶性间皮瘤临床罕见，临床症状不典型，缺少特异性肿瘤标记物，诊断主要依据病理，生物学行为进展快，治疗以根治性手术为主，辅以术后放化疗可以延长患者寿命，预后差。“,”Objective To study the clinical and pathological features, diagnosis and treatment of malignant mesothelioma of the tunica vaginalis testis. Methods A case of malignant mesothelioma of the tunica vaginalis testis was reported and the related literatures were reviewed. Results The patient was A 63 years old and his B-ultrasound result showed obvious liquid anechoic area around the testis, the tunica vaginalis was resected radically. Pathological analysis of testis tunica vaginalis demonstrated malignant mesothelium-derived carcinoma. Two months later, the patient died. Conclusion Malignant mesothelioma of the tunica vaginalis is a rare disease of the testis with potentially aggressive behavior. Due to the lack of tumor clinic characteristic symptoms and specific tumor maker, correct preoperative diagnosis is extremely difficult. Radical surgical treatment is a major treatment and after operation chemoradiotherapy can improve survival of the patients. Totally the patient has an indicator of poor prognosis.