Ekrem ÜNAL,
Ebru YILMAZ,
Alper ÖZCAN,
Bilgen IŞIK,
Musa KARAKÜKCÜ,
Cüneyt TURAN,
Hülya AKGÜN,
Figen ÖZTÜRK,
Abdulhakim COŞKUN,
Mehmet Akif ÖZDEMİR,
Türkan PATIROĞLU
8148
Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey
Background/aim: Non-Wilms renal tumors NWRTs are rarely encountered in children. The aim of this study is to determine the treatment strategies, prognosis, outcomes, and survival of children with NWRTs at Erciyes University in Kayseri, Turkey.Materials and methods: Medical records of all patients n = 20 treated for NWRTs over a 23-year period 1995-2018 were reviewed retrospectively.Results: There was male predominance female/male: 7/13 ; the median age at diagnosis was 3.2 years old 0.1-13.5 years old . The major histological groups included mesoblastic nephroma MBN , n: 5, 25% , malignant rhabdoid tumor MRT , n: 5, 25% , renal cell carcinoma, n: 3, 15% , inflammatory myofibroblastic tumor n: 2, 10% , multilocular cystic renal tumors n: 2, 10% , metanephric adenoma n: 1, 5% , renal neuroblastoma n: 1, 5% , and bilateral renal Ewing sarcoma/primitive neuroectodermal tumor ES/PNET n: 1, 5% . All of the patients with NWRTs had radical nephrectomy except the child with bilateral renal ES/PNET. Six children died because of progressive disease; the mortality rate was 30% n: 6 . Conclusion: We have made the first report of bilateral renal involvement of ES/PNET in the English medical literature. Physicians dealing with pediatric renal masses should be alert to the high mortality rate in children with MRT, MBN, and ES/PNET and they should design substantial management plans for NWRTs.
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- 1. Akyüz C, Yalçin B, Yildiz I, Hazar V, Yörük A et al. Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG). Pediatric Hematology and Oncology 2010 (27): 161-178. doi: 0.3109/08880010903447375
- 2. Broecker B. Non-Wilms’ renal tumors in children. Urologic Clinics of North America 2000 (27): 463-469.
- 3. Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I et al. Part I: Primary malignant non-Wilms’ renal tumours in children. Lancet Oncology 2007 (8): 730-737. doi: 10.1016/S1470- 2045(07)70241-3
- 4. Ahmed HU, Arya M, Levitt G, Duffy PG, Sebire NJ et al. Part II: Treatment of primary malignant non-Wilms’ renal tumours in children. Lancet Oncology 2007 (8): 842-848. doi: 10.1016/ S1470-2045(07)70276-0
- 5. Zhuge Y, Cheung MC, Yang R, Perez EA, Koniaris LG et al. Pediatric non-Wilms renal tumors: subtypes, survival, and prognostic indicators. Journal of Surgical Research 2010 (163): 257-263. doi: 10.1016/j.jss.2010.03.061
- 6. Saula PW, Hadley GP. Pediatric non-Wilms’ renal tumors: a third world experience. World Journal of Surgery 2012 (36): 565-572. doi: 10.1007/s00268-011-1410-2
- 7. Warmann SW, Nourkami N, Frühwald M, Leuschner I, Schenk JP et al. Primary lung metastases in pediatric malignant nonWilms renal tumors: data from SIOP 93-01/GPOH and SIOP 2001/GPOH. Klinische Pädiatrie 2012 (224): 148-152. doi: 10.1055/s-0032-1304600
- 8. Bozlu G, Çıtak EÇ. Evaluation of renal tumors in children. Turkish Journal of Urology 2018 (44): 268-273. doi: 10.5152/ tud.2018.70120
- 9. Dogan MS, Doganay S, Koc G, Gorken SB, Unal E et al. Inflammatory myofibroblastic tumor of the kidney and bilateral lung nodules in a child mimicking Wilms tumor with lung metastases. Journal of Pediatric Hematology/Oncology 2015 (37): e390-393. doi: 10.1097/MPH.0000000000000353
- 13. Gupta R, Mathur SR, Singh P, Agarwala S, Gupta SD. Cellular mesoblastic nephroma in an infant: report of the cytologic diagnosis of a rare paediatric renal tumor. Diagnostic Cytopathology 2009 (37): 377-380. doi: 10.1002/dc.21028
- 14. Van den Heuvel-Eibrink MM, van Tinteren H, Rehorst H, Coulombe A, Patte C et al. Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group. Pediatric Blood & Cancer 2011 (56): 733-737. doi: 10.1002/ pbc.22922
- 15. Tomlinson GE, Breslow NE, Dome J, Guthrie KA, Norkool P et al. Rhabdoid tumor of the kidney in the National Wilms’ Tumor Study: age at diagnosis as a prognostic factor. Journal of Clinical Oncology 2005 (23): 7641-7645. doi: 10.1200/ JCO.2004.00.8110
- 16. Indolfi P, Spreafico F, Collini P, Cecchetto G, Casale F et al. Metastatic renal cell carcinoma in children and adolescents: a 30-year unsuccessful story. Journal of Pediatric Hematology/Oncology 2012 (34): e277-281. doi: 10.1097/ MPH.0b013e318267fb12
- 17. Varan A, Akyuz C, Sari N, Buyukpamukçu N, Cağlar M et al. Renal cell carcinoma in children: experience of a single center. Nephron Clinical Practice 2007 (105): c58-61. doi: 10.1159/000097599
- 18. Perlman EJ. Pediatric renal cell carcinoma. Surgical Pathology Clinics 2010; 3: 641-651. doi: 10.1016/j.path.2010.06.011
- 19. Cajaiba MM, Dyer LM, Geller JI, Jennings LJ, George D et al. The classification of pediatric and young adult renal cell carcinomas registered on the children’s oncology group (COG) protocol AREN03B2 after focused genetic testing. Cancer 2018 (124): 3381-3389. doi: 10.1002/cncr.31578
- 20. Pothadiyil AJ, Bhat S, Paul F, Mampatta J, Srinivas M. Inflammatory myofibroblastic tumor of the kidney: a rare renal tumor. Journal of Clinical and Diagnostic Research 2016 (10): ED17-ED18. doi: 10.7860/JCDR/2016/22465.8856
- 21. Bektas S, Okulu E, Kayigil O, Ertoy Baydar D. Inflammatory myofibroblastic tumor of the perirenal soft tissue misdiagnosed as renal cell carcinoma. Pathology, Research and Practice 2007 (203): 461-465. doi: 10.1016/j.prp.2007.02.002