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.

Objectives Workers in the construction industry have been exposed to asbestos in various occupations. In Italy, a National Mesothelioma Registry has been implemented more than 20 years ago. Using cases selected from this registry and exploiting existing control data sets, we estimated relative risks for pleural mesothelioma (PM) among construction workers. Design Case–control study. Setting Cases from the National Mesothelioma Registry (2000–2018), controls from three previous case–control studies. Methods We selected male PM incident cases diagnosed in 2000–2018. Population controls were taken from three studies performed in six Italian regions within two periods (2002–2004 and 2012–2016). Age-adjusted and period-adjusted unconditional logistic regression models were fitted to estimate odds ratios (OR) for occupations in the construction industry. We followed two approaches, one (primary) excluding and the other (secondary) including subjects employed in other non-construction blue collar occupations for >5 years. For both approaches, we performed an overall analysis including all cases and, given the incomplete temporal and geographic overlap of cases and controls, three time or/and space restricted sensitivity analyses. Results The whole data set included 15 592 cases and 2210 controls. With the primary approach (4797 cases and 1085 controls), OR was 3.64 (2181 cases) for subjects ever employed in construction. We found elevated risks for blue-collar occupations (1993 cases, OR 4.52), including bricklayers (988 cases, OR 7.05), general construction workers (320 cases, OR 4.66), plumbers and pipe fitters (305 cases, OR 9.13), painters (104 cases, OR 2.17) and several others. Sensitivity analyses yielded very similar findings. Using the secondary approach, we observed similar patterns, but ORs were remarkably lower. Conclusions We found markedly increased PM risks for most occupations in the construction industry. These findings are relevant for compensation of subjects affected with mesothelioma in the construction industry.

Sinonasal cancers (SNCs) are rare malignancies associated with occupational exposures. The aim of this study was to analyse the survival of SNC patients using data from the population-based SNC registry of the Lombardy region (10 million people), Italy. We included epithelial SNC cases registered in 2008–2020 and followed-up for vital status until 31 July 2023. Multivariate flexible parametric models with time-dependent covariates were fitted to calculate excess hazard ratios (EHRs) and 95% confidence intervals (CIs) of death. Based on 827 cases (553 males, 274 females) and 514 deaths (345 males, 169 females), the 5-year observed survival was 49% and the net survival was 57%. Age had a substantial impact on survival, particularly within the first year (EHR, 1.35; 95% CI, 1.12–1.51 per 10 years). Compared with the nasal cavity, the EHR for paranasal sinuses was 4.70 (95% CI, 2.96–7.47) soon after diagnosis. Compared with squamous cell carcinomas, the EHR was 0.69 (95% CI, 0.52–0.91) for adenocarcinomas, 1.68 (95% CI, 1.20–2.35) for undifferentiated and unspecified carcinomas, and 1.78 (95% CI, 1.07–2.95) for neuroendocrine carcinomas. Age and cancer site showed time-dependent effects on prognosis, especially within the first month after diagnosis. Prognosis was also markedly affected by cancer morphology. No associations were found for gender and period of diagnosis.

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.

Background: Pericardial mesothelioma (PM) is an extremely rare cancer with a poor prognosis and no consensus on diagnostic and therapeutic management. We conducted a registry-based study on PM cases in Italy diagnosed between 1993 and 2021. Methods: Based on data from the Italian National Mesothelioma Registry (ReNaM), we performed a descriptive analysis of PM cases including clinical presentation, diagnostic work-up, asbestos exposure, and therapeutic management. Overall survival was calculated. Hazard ratios (HRs) and 95% confidence intervals (CI) for selected variables were estimated with univariate and multivariate Cox models. Results: We identified 72 cases (46 men and 26 women). Median age was 66 years (range 22–89). The most frequent histological subtypes were epithelioid and unspecified mesothelioma. Almost two-thirds of cases had been exposed to asbestos. The most common clinical presentation was pericardial effusion. Overall median survival was 2.8 months (95% CI 1.2–6.6) and older age at diagnosis was a negative prognostic factor. Clinical and treatment data were available for 47 patients (65%). Approximately one out of two patients underwent surgery (palliative, in one-third of cases). Adjuvant therapy was administered to seven patients (15%). In cases with treatment information, sarcomatoid subtype (HR 2.74, 95% CI: 1.06–7.06) was a negative prognostic factor; adjuvant therapy was associated with better survival (HR 0.38, 95% CI 0.14–1.02), but confounding by indication cannot be excluded. Conclusions: We confirmed the very poor prognosis of PM. An international network collecting PM cases with treatment information is needed to improve patient quality of life and survival.

Background: The presence of a second primary cancer (SPC) in patients with pleural mesothelioma (PM) may impact overall survival and suggest a common mechanism of carcinogenesis or an underlying germline genetic alteration. Methods: We evaluated the occurrence of SPCs within PM cases collected from 2000 to 2018 by the Lombardy Mesothelioma Registry and their prognostic implications. Kaplan–Meier analysis was performed to estimate median survival times, together with univariate and multivariate Cox regression models to estimate hazard ratios (HR) and 95% confidence intervals (CI) of death. Results: The median overall survival (OS) of the entire study population (N = 6646) was 10.9 months (95% CI: 10.4–11.2); patient age and histotype were the strongest prognostic factors. No substantial survival difference was observed by the presence of an SPC (10.5 months in 1000 patients with an SPC vs. 10.9 months in 5646 patients in the non-SPC group, HR 1.03, p = 0.40). Shorter OS in the SPC group was only observed in 150 patients with the non-epithelioid subtype (median OS of 5.4 vs. 7.1 months, HR 1.21, p = 0.03). Conclusions: The diagnosis of an SPC did not influence the outcome of PM patients in the overall study population but was associated with shorter OS in non-epithelioid cases. Further studies are needed to clarify the role of SPCs as markers of genetic susceptibility in mesothelioma.