IJSR - International Journal of Scientific Research

Dr Basant Kumar; Dr Vijai D Upadhyaya; Dr Ram Nawal Rao; Dr Sheo Kumar

Volume : VIII, Issue : VIII, August - 2019

Non–Wilms‘ Renal Tumors: Difficult management experience in Third World.

Dr Basant Kumar, Dr Vijai D Upadhyaya, Dr Ram Nawal Rao, Dr Sheo Kumar

Abstract :

Background: Renal tumors other than Wilms‘ tumors are infrequent in childhood and account for less than 1% of all pediatric tumors. Differentiation in these tumors is important for better outcome. Lack of awareness and resources, late presentation, difficult diagnosis, and less oncology centers/experts, made the situation unfavorable resulting in poor outcome. Aim of this study is to present our experience with non–Wilms` malignant renal tumors resulted in fair outcome. Method: We retrospectively reviewed all patients (n = 7) treated for non Wilms` renal tumor from January 2013 to July 2108. Data collected regarding presentations, management and follow–up with special attention on diagnostic difficulties. Result: Seven patients were included in the study with histological diagnosis of Clear Cell Sarcoma (CCSK, 3), Rhabdoid Tumor (RTK, 2), Ewing`s/PNET Tumor (1) and Congenital Mesoblastic Nephroma (CMN, 1). Two were females and aged ranged from 7 – 96 months (median 36 months). 4 patients has left sided tumor. Four patients were already evaluated and received chemotherapy (DD4A regime) before referral (2 CCSK; 1 RTK and 1 CMN) in which 3 patients had pathology report of Wilm`s Tumor but no response to chemotherapy. All patients were evaluated and biopsy performed in 6 patients (Trucut–5; open–1). Only one patient (CCSK) had radiological evidence of metastasis (bone and lung) and died during treatment. CMN patient was operated and managed well but lost in follow–up after 3 months. Rest 5 patients were operated, received chemo–radiation and are alive without recurrence. Follow–up period ranged 12 – 56 months (median 38 months). Conclusion: Because of similar clinical presentations, it is almost impossible to differentiate Non–Wilms` Tumor from Wilms` Tumor and lack of resources / experts further add in it. Differentiation is mandatory because of separate treatment protocols and proper diagnosis and multimodal treatment may result in favorable outcome