Massimiliano Silveri1, Gian Luigi Natali2, Giovanni Cobellis3, Agnese Roberti4, Sabrina Rossi5, Lorenzo Gregori2, Giovanni Torino3. 1. Department of Surgery, Bambino Gesù Children's Hospital, Rome, Italy. 2. Department of Imaging, Bambino Gesù Children's Hospital, Rome, Italy. 3. Pediatric Urology and Surgery Unit, G. Salesi Children's Hospital, Ancona, Italy. 4. Pediatric Urology Unit, Santobono-Pausilipon Children's Hospital, Naples, Italy. 5. Department of Pathology, Bambino Gesù Children's Hospital, Rome, Italy.
Abstract
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.
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.