Stella Simona, Ceresoli Giovanni Luca, Dallari Barbara, Barile Rosalba, Maisenti Fabio, Rugarli Sabrina, Marinaccio Alessandro, Consonni Dario, Mensi Carolina
Occupational Health Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.
Department of Medical Oncology, Humanitas Gavazzeni Clinic, 24125 Bergamo, Italy.
Cancers (Basel). 2024 Nov 26;16(23):3956. doi: 10.3390/cancers16233956.
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.
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.
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.
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.
睾丸鞘膜间皮瘤(MTVT)是一种极其罕见且侵袭性强的癌症。MTVT的诊断和管理较为复杂,目前尚无标准治疗方案。
我们于1982年1月1日至2024年3月14日利用PubMed进行了一项系统的文献综述,以收集所有可用的病例报告和病例系列。对患者的特征进行了描述性分析,包括临床表现、诊断检查、治疗管理以及既往石棉暴露情况。评估了采用不同治疗方法的患者的生存时间。
总体而言,我们的分析纳入了289例MTVT患者。最常见的临床表现是阴囊/睾丸肿胀或肿块(187例患者,65%)以及鞘膜积液(159例,55%)。三分之二的病例报告了影像学评估,主要是超声检查或CT扫描。根治性手术(216例患者,75%),包括睾丸切除术,在某些情况下还包括半阴囊切除术和腹股沟淋巴结切除术,是最常用的治疗方法。少数患者(49例,17%)术后接受了辅助治疗(放疗、化疗或两者联合),但没有生存改善的证据。
目前尚无MTVT的标准指南。准确的影像学分期后进行根治性手术应是主要治疗方法。辅助治疗的作用仍不明确。由于其罕见性,MTVT应在转诊中心进行治疗,患者数据应收集到专门的登记册中,以提高对这种极其罕见疾病的认识,并建立最佳的诊断和治疗管理。