Abstract
Late relapses of Wilms tumour are extremely uncommon but still represent possible events. Even more rarely Wilms tumours present as extrarenal neoplasms, for which it could be difficult to diagnose and treat them promptly.We present a unique case of late recurrence of Wilms tumour 16 years after the primary diagnosis, with location in the gynaecological system. The relapse presented as a vaginal mass, and it gradually raised up to involve the majority of pelvic organs. We accurately studied the tumour extension, even realising a 3D preoperative reconstruction, and we managed to evaluate the patient with a multidisciplinary team involving general surgeons, urologists, gynaecologists and plastic surgeons. We finally decided for an extended surgical approach and realised a complete pelvic exenteration. Three months after surgery, the patient is in a good general condition, without major surgical complications and with no radiological signs of pelvic tumour relapse.