ABSTRACT
Objective To investigate the clinical manifestations,imaging and pathological features and treatment prognosis of primary renal sarcoma in adults.Methods A retrospective analysis was performed on the clinical data of 48 patients with primary renal sarcoma from January 2009 to December 2018 in the first affiliated hospital and cancer center of Sun Yat-Sen university.There were 30 males and 18 females.Their aged ranged from 27 to 76 yrs with an average age of 50 yrs.A total of 24 patients presented with lumbar and abdominal pain.Abdominal mass was found in 2 cases.Gross hematuria was noticed in 4 cases.Febrile was recorded in 2 cases and 21 cases were diagnosed by physical examination.31 tumors located in the left kidney and 17 tumors located in the right kidney.The tumor diameter ranged from 3 to 16 cm with an average diameter of 8 cm.All patients underwent ultrasound or CT/MRI examination of the urinary system before surgery.The ultrasound showed the undistributed echo inside the tumor with the undistinguished border.The image of necrosis and liquefaction could be seen in some cases.The CT/MRI examination showed the lesion site with the necrosis,liquefaction or cystic changing.The mass exhibited the unregular enhancement with undistinguished border line.The mean diameter of liposcarcoma was 10.2 cm.CT scan demonstrated the relative low density of tumor,which was hard to be identified with AML.The average diameter of leiomyosarcoma was 6.5 cm.The enhanced CT scan showed the low density of tumor,compared with renal parenchyma.46 patients underwent radical nephrectomy,2 patients underwent renal tumor biopsy.And postoperative follow-up was performed.Results Pathological diagnosis revealed that 19 cases with liposarcoma,9 cases with leiomyosarcoma and 4 cases with synovial sarcoma,especially 4 cases with Ewing' s sarcoma and 12 cases with other sarcoma.36 cases were followed up and survived for 4 to 64 months.The average survival time was 28 months.The longest mean survival time was seen in patients with liposarcoma,which was 32 months (ranging 11 to 64 months).The mean survival time of synovial sarcoma group was 25 months (ranging 5-58 months).The mean survival time of Ewing's sarcoma group was 22 months(ranging 12-46 months).and the survival time of leiomyosarcoma group was the shortest 20 months (ranging 4-36 months).Conclusion Renal sarcoma is rare and highly malignant.It needs to be diagnosed with clinical manifestations,imaging and pathological data together.
ABSTRACT
ObjectiveTo review the clinical features,management and prognosis of renal Ewing's sarcoma (ES) of a single case report.MethodsA single case of renal ES was reported.A 33-year-old male presented with a mass in the left kidney found during a three day medical examination.B-ultrasound examination showed a lesion with rich blood flow signals and well defined margins in the inferior portion of the left kidney.The CT scan revealed a solid mass of 5.1 cm × 4.7 cm in the inferior portion of the kidney with un-even enhancement by contrast.A possible diagnosis of renal carcinoma was given prior to surgery.No metastasis was proven.A literature review of ES was then conducted.ResultsA left retroperitoneoscopic radical nephrectomy was successfully performed.Gross pathologic examination showed a solid tumor with necrosis,localized at the inferior pole of the left kidney.The histopathological examination revealed the tumor consisted of small round tumor cells,which were positive for CD99,vimentin and PAS,but negative for WT-1.A diagnosis of ES of the kidney was then determined.The patient received alternating short cycle ( CTX + VCR + THP) and long cycle ( IFO + VP-16) adjuvant chemotherapy for 6 cycles after the operation.There has been no evidence of recurrence at the 14-month follow up.ConclusionsES of the kidney is a rare disease with no specific clinical feature in most cases.Diagnosis of renal ES must be confirmed with histological features.Surgery combined with radiotherapy and chemotherapy is the main method of therapy for renal ES.The prognosis of renal ES is poor.