Domenico Fichera1, Roberta Luciano2, Alessandro Nini3, Massimo Freschi2, Claudio Doglioni2, Roberto Bertini3, Francesco Montorsi3, Umberto Capitanio3. 1. Division of Experimental Oncology. URI. Urological Research Institute. Renal cancer Unit. IRCCS San Raffaele Scientific Institute. Milan. Italy. 2. Unit of Pathology. University Vita-Salute San Raffaele. IRCCS San Raffaele Scientific Institute. Milan. Italy. 3. Division of Experimental Oncology. URI. Urological Research Institute. Renal cancer Unit. IRCCS San Raffaele Scientific Institute. Milan. Italy.Unit of Urology. University Vita-Salute San Raffaele. IRCCS San Raffaele Scientific Institute. Milan. Italy.
Abstract
OBJECTIVES: Primary renal liposarcoma is an unusual malignant mesenchymal tumor. In this context, all the previous reports were based on cases with insufficient data regarding the natural history of the disease. We decided to fill this gap, reporting the largest single institution series of patients with primary RL and a review of the already available literature. METHODS: We describe 3 cases with radiologically and histologically-confirmed RL out of 3,224 surgeries performed for primary kidney cancers over 28 years (1987-2015, 0.09%) at a single tertiary care center. RESULTS: Patients underwent open radical nephrectomy with an anterior transperitoneal access with complete resection of the retroperitoneal mass and retroperitoneal lymph node dissection; all patients died from tumor progression after a mean time of 45 months. CONCLUSIONS: In conclusion, RL is a very rare mesenchymal renal tumor, with sporadic cases reported. We reported the largest case series of primary RL. The most appropriate approach for RL is nephrectomy with complete resection of all the neoplastic tissue. Stringent follow-up scheme is required due to a high rate of disease recurrence and progression. The role of adjuvant and salvage therapy remains to be investigated.
OBJECTIVES:Primary renal liposarcoma is an unusual malignant mesenchymal tumor. In this context, all the previous reports were based on cases with insufficient data regarding the natural history of the disease. We decided to fill this gap, reporting the largest single institution series of patients with primary RL and a review of the already available literature. METHODS: We describe 3 cases with radiologically and histologically-confirmed RL out of 3,224 surgeries performed for primary kidney cancers over 28 years (1987-2015, 0.09%) at a single tertiary care center. RESULTS:Patients underwent open radical nephrectomy with an anterior transperitoneal access with complete resection of the retroperitoneal mass and retroperitoneal lymph node dissection; all patients died from tumor progression after a mean time of 45 months. CONCLUSIONS: In conclusion, RL is a very rare mesenchymal renal tumor, with sporadic cases reported. We reported the largest case series of primary RL. The most appropriate approach for RL is nephrectomy with complete resection of all the neoplastic tissue. Stringent follow-up scheme is required due to a high rate of disease recurrence and progression. The role of adjuvant and salvage therapy remains to be investigated.