Treatment and follow-up of rare testis tumours.

PURPOSE: To provide recommendations for the follow-up of rare, clinically localised testis tumours, including Leydig, Sertoli or granulosa cell and spermatocytic tumours. METHODS: Medline and Embase searches to identify published clinical trials, cohort studies, reviews, clinical practise guidelines and meta-analyses to design expert opinion-based follow-up schedules. RESULTS: In four different systematic reviews, we previously identified 1375 men with Leydig, 435 with Sertoli, 239 with granulosa cell lesions and 146 with spermatocytic tumours. Local recurrence after testis-sparing surgery (TSS) was observed in 7%, < 1% and 5% of men with Leydig, Sertoli and granulosa cell tumours: no reports were available regarding recurrence after TSS in men with spermatocytic tumours. Distant recurrence was observed in 6%, 4%, 4% and 7% of the first four tumour types, respectively: metastasis was never reported in granulosa cell tumours of juvenile type. For patients with metastatic disease, complete response after surgical resection was reported in 10%, 18%, 43% and 4%. Complete response after chemotherapy was reported in 5%, 0%, 29% and 4%. There was no report of patients responding to radiotherapy alone. CONCLUSIONS: We have collated the existing data about local and distant recurrence and response to treatment in men with rare testicular tumours and propose new recommendations for follow-up with cross-sectional imaging, stratified for each histological subtype.

Clinicopathological characteristics and outcomes in men with mesothelioma of the tunica vaginalis testis: analysis of published case-series data.

PURPOSE: Mesothelioma of the tunica vaginalis testis (MTVT) is a rare tumor, and currently, there are no published treatment recommendations. METHODS: We performed a systematic literature review and synthesized clinical presentation, clinicopathological factors associated with metastatic disease, treatment options, and outcomes in men with MTVT. RESULTS: We included 170 publications providing data on 275 patients. Metastatic disease occurred in 84/275 (31%) men with malignant MTVT: Most common sites included retroperitoneal lymph nodes (LNs) (40/84, 48%), lungs (30/84, 36%), and inguinal LNs (23/84, 27%). Invasion of the spermatic cord or scrotum was the only risk factor for local recurrence [odds ratio (OR) 3.21, 95% confidence interval (CI) 1.36-7.57]. Metastatic disease was associated with age ≥ 42 years (OR 3.02, 95% CI 1.33-6.86), tumor size ≥ 49 mm (OR 6.17, 95% CI 1.84-20.74), presence of necrosis (OR 8.31, 95% CI 1.58-43.62), high mitotic index (OR 13.36, 95% CI 1.53-116.51) or angiolymphatic invasion (OR 3.75, 95% CI 1.02-13.80), and local recurrence (OR 4.35, 95% CI 2.00-9.44). Complete remission in the metastatic setting was observed in five patients, most of whom were treated with multimodal therapy. Median survival in patients with metastatic disease was 18 months (IQR 7-43). CONCLUSION: Malignant MTVT is a rare but aggressive disease. Since local recurrence is a risk factor for metastatic progression, we recommend aggressive local treatment. Survival and response to any treatment in the metastatic setting are limited.