Background: Perivascular epithelioid cell tumours (PEComas) represent a rare group of mesenchymal tumours with an unpredictable outcome. They are usually observed in middle-aged women whilst they are very rare in children, with limited cases described. Due to the rare anatomical location in kidneys, a correct diagnosis and treatment is really challenging.
Methods: We observed and treated a case of kidney's PEComa in a 14-year-old boy.
Results: An individualized approach based on anatomical (3D CT-reconstructions) and histopathological (US-guided preliminary biopsy) features lead us to perform a successful robotic-sparing surgery enabling the preservation of two-thirds of the kidney involved.
Conclusions: A meticulous preoperative planning in selected patients can lead to a minimally invasive approach even in some paediatric kidney's neoplasms.
Keywords: metanephric stromal tumour; partial nephrectomy; rare tumours; renal tumours; robotic.
© 2020 John Wiley & Sons Ltd.