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.