Background: Mesothelioma of the tunica vaginalis testis (MTVT) is an extremely rare and aggressive cancer. The diagnosis and management of MTVT is complex, and no standard treatment protocol is available. Methods: We conducted a systematic literature review from 1 January 1982 to 14 March 2024 using PubMed to collect all the available case reports and case series. A descriptive analysis of patient characteristics with clinical presentation, diagnostic work-up, therapeutic management, and past asbestos exposure was performed. Survival times of patients treated with different therapeutic approaches were evaluated. Results: Overall, 289 patients with MTVT were included in our analysis. The most common clinical presentations were scrotal/testicular swelling or mass (187 patients, 65%) and the presence of hydrocele (159, 55%). Imaging evaluation, mostly with ultrasonography or CT scan, was reported in two-thirds of cases. Radical surgery (216 patients, 75%) with orchiectomy and, in select cases, hemiscrotectomy and inguinal lymphadenectomy was the most frequent therapeutic approach. A minority of patients (49, 17%) received adjuvant therapy after surgery (radiotherapy, chemotherapy, or a combination of the two), with no evidence of survival improvement. Conclusions: No standard guidelines for MTVT are available so far. Radical surgery following accurate radiological staging should be the mainstay of treatment. The role of adjuvant treatments remains undefined. Due to its rarity, MTVT should be treated in referral centers, and patients’ data should be collected in a dedicated register in order to improve the knowledge of this exceedingly rare disease and establish optimal diagnostic and therapeutic management.
背景：睾丸鞘膜间皮瘤是一种极为罕见且侵袭性强的恶性肿瘤。其诊断与治疗过程复杂，目前尚无标准化治疗方案。方法：我们通过PubMed数据库对1982年1月1日至2024年3月14日期间所有已发表的病例报告和病例系列进行系统性文献综述。对患者的临床特征、诊断流程、治疗方案及既往石棉接触史进行描述性分析，并评估不同治疗方式患者的生存时间。结果：本研究共纳入289例睾丸鞘膜间皮瘤患者。最常见的临床表现是阴囊/睾丸肿胀或肿块（187例，65%）以及鞘膜积液（159例，55%）。约三分之二的病例报告了影像学评估结果（主要为超声或CT检查）。根治性手术（216例，75%）是最常用的治疗方式，包括睾丸切除术，部分病例联合半阴囊切除术和腹股沟淋巴结清扫术。少数患者（49例，17%）术后接受辅助治疗（放疗、化疗或两者联合），但未观察到生存获益证据。结论：目前尚无睾丸鞘膜间皮瘤的标准诊疗指南。基于精确影像学分期的根治性手术应作为主要治疗手段，辅助治疗的作用尚未明确。鉴于该疾病的罕见性，建议在专科诊疗中心进行治疗，并建立专项登记系统收集患者数据，以增进对这种极罕见疾病的认知，最终确立最优化的诊疗策略。
肿瘤(癌症)患者之家