Background: Mesothelioma of the tunica vaginalis testis (MTVT) is an exceedingly rare tumor. We performed a registry-based study on MTVT patient management and survival in Italy. Methods: Cases were extracted from the dataset of the Italian National Mesothelioma Registry. A descriptive analysis of patient characteristics, including asbestos exposure, clinical presentation, diagnostic work-up and therapeutic management, was performed. Overall survival was evaluated. We calculated hazard ratios (HR) and 95% confidence intervals (CI) for selected variables by fitting univariate and multivariable Cox models. Results: Overall, 104 patients with MTVT were included. Median age was 72 years (range 17–92). Epithelioid histotype was the most frequent. Previous asbestos exposure was identified in two thirds of cases. Data on diagnostic and therapeutic management were available for 74 patients (71%). The most frequent presentations were scrotal swelling/mass, hydrocele and inguinal pain. All patients underwent surgery, mostly with orchi-funicolectomy. Adjuvant therapy was administered to 15 patients (20%). Overall median survival was 26.2 months (95% CI 22.1–52.1); 3-, 5- and 10-year survival was 49%, 30% and 18%. Older age at diagnosis and presence of distant metastasis (HR 1.91, CI: 0.85–4.26) were negative prognostic factors. Adjuvant therapy was associated with higher mortality (HR 2.54, CI: 1.25–5.15), indicating a more advanced stage at diagnosis. Conclusions: Surgery remains the mainstay of treatment for MTVT; adjuvant therapy in our study did not improve outcome. Data from cancer registries are essential for rare cancers, but they should be integrated routinely with additional diagnostic and therapeutic information.
背景：睾丸鞘膜间皮瘤是一种极为罕见的肿瘤。我们在意大利开展了一项基于登记数据的关于睾丸鞘膜间皮瘤患者治疗与生存情况的研究。方法：病例数据来源于意大利国家间皮瘤登记数据库。对患者特征进行了描述性分析，包括石棉暴露史、临床表现、诊断检查及治疗方案。评估了总体生存率。通过拟合单变量和多变量Cox模型，计算了选定变量的风险比及其95%置信区间。结果：共纳入104例睾丸鞘膜间皮瘤患者。中位年龄72岁（范围17-92岁）。上皮样组织学类型最为常见。三分之二的病例有既往石棉暴露史。74例患者（71%）可获得诊断与治疗管理数据。最常见的临床表现为阴囊肿胀/肿块、鞘膜积液和腹股沟疼痛。所有患者均接受手术治疗，主要为睾丸精索切除术。15例患者（20%）接受了辅助治疗。总体中位生存期为26.2个月（95% CI 22.1-52.1）；3年、5年和10年生存率分别为49%、30%和18%。诊断时年龄较大和存在远处转移（HR 1.91，CI：0.85-4.26）是负面预后因素。辅助治疗与更高的死亡率相关（HR 2.54，CI：1.25-5.15），表明诊断时已处于更晚期阶段。结论：手术仍是睾丸鞘膜间皮瘤的主要治疗手段；本研究中辅助治疗并未改善预后。癌症登记数据对罕见肿瘤研究至关重要，但应常规整合更多诊断与治疗信息。
肿瘤(癌症)患者之家