Çocuklarda primer böbrek tümörlerinin klinik özellikleri, tedavi yaklaşımları ve tedavi sonuçları

Year 2021, Volume: 31 Issue: 4, 375 - 379, 15.12.2021

Buket Kara Mehmet Sarıkaya , Kübra Ertan Nevzat Serdar Uğraş İlhan Çiftçi Güler Yavaş Yavuz Köksal

https://doi.org/10.54005/geneltip.1036599

Abstract

Amaç: Bu çalışmanın amacı, çocuklarda primer malign böbrek tümörlerinin klinik özellikleri, tedavi yaklaşımları ve tedavi sonuçlarının değerlendirilmesidir.

Gereç ve Yöntem: 2006 ile 2020 yılları arasında primer malign böbrek tümör tanısı alan ve izlenen çocuk hastaların onkoloji dosyaları geriye yönelik incelendi. Hastaların demografik ve klinik özellikleri ile tedavi yaklaşımları ve izlemleri not edildi.

Bulgular: Bu yıllar arasında izlenen 950 malign hastalıklı çocuğun 49’unda(%5,2) primer malign böbrek tümörü vardı. Hastaların yaşı üç gün ile 13 yıl arasında değişiyordu (ortanca, 3 yıl). Erkek kız oranı 25/24'dü. En sık görülen semptom ve bulgu karında kitle idi. İki hasta da bilateral hastalık vardı (%4,1). Patolojik tanılar, Wilms tümörü (n = 44, % 89,8), mezoblastik nefroma (n=2, % 4,1), böbreğin clear hücreli sarkomu (n=2, % 4,1) ve böbreğin primer sinovyal sarkomuydu (n=1, %2). Wilms tümörlü hastalardan sadece ikinde diffüz anaplazi vardı. Hastalarımızın risk grupları düşük (n = 4, % 8.3), orta (n = 35, % 72.9) ve yüksek riskli (n = 9, % 18.8) idi. Hastaların takip süreleri iki ay ile 15 yıl (ortanca, 5 yıl) arasında değişiyordu. Primer böbrek tümörlerinde genel ve olaysız sağ kalım oranları sırasıyla % 72,7 ve% 59,7 idi. Wilms tümörlü hastaların genel ve olaysız sağ kalım oranları %79 ve %63'dü.

Sonuç: Primer malign böbrek tümörlerinde özellikle de Wilms tümöründe multidisipliner yaklaşımlarla başarı oranları yükselmiştir. Bundan sonra hem tedavi başarısının daha da artırılması hem de tedavi ilişkili yan etkilerin azaltılması üzerinde durulmalıdır.

Keywords

çocuk, primer malign böbrek tümörleri, tedavi yaklaşımları, tedavi sonuçları

The clinical features, treatment approaches and outcomes of primary renal tumors in children

Abstract

Abstract

Objective: The aim of this study is to evaluate the clinical features, treatment approaches and outcomes of primary malign renal tumors in children.

Material and Methods: The oncology charts of children diagnosed and followed up with primary malign renal tumors between 2006 and 2020 were retrospectively analyzed. Demographic and clinical characteristics, treatment approaches and follow-up of the patients were noted.

Results: In this period, 49 of 950 children with malignant disease had primary renal kidney tumors (5.2%). The patients' age ranged from three days to 13 years (median, 3 years). The male to female ratio was 25/24. The most common symptom and signs were abdominal mass. Both patients had bilateral disease (4.1%). Pathological diagnoses were Wilms tumor (n = 44, 89.8%), mesoblastic nephroma (n = 2, 4.1%), clear cell sarcoma of the kidney (n = 2, 4.1%), and primary synovial sarcoma of the kidney (n = 1, 2%). Only two of the patients with Wilms tumor had diffuse anaplasia. The risk groups of our patients were low (n=4, 8.3%), intermediate (n=35, 72.9%) and high risk (n=9, 18.8%). Follow-up periods ranged from two months to 15 years (median, 5 years). Overall and event free survival rates were 72.7% and 59.7%, respectively. Overall and event-free survival rates of Wilms tumor were 79% and 63%.

Conclusion: Multidisciplinary approaches have increased success rates in primary malignant kidney tumors, especially in Wilms tumor. After that, it should be focused on both increasing the success of the treatment and reducing the treatment-related side effects.

Keywords

children, primary renal tumors, treatment approach, outcome

References

• Kutluk T, Yesilipek MA. Turkish national pediatric cancer registry 2002-2008 (Turkish Pediatric Oncology Group and Turkish Pediatric Hematology Society). Pediatr Blood Cancer. 2009; 53: 851.
• Kutluk T, Sahiner UM, Akyuz C, et al. A hospital based pediatric cancer registry, Hacettepe experience 1971-2004. Pediatr Blood Cancer. 2009; 53: 851.
• Varan A. Wilms' tumor in children: an overview. Nephron Clin Pract. 2008; 108: c83-90.
• Bozlu G, Çıtak EÇ. Evaluation of renal tumors in children. Turk J Urol. 2018; 44: 268-73.
• Qureshi SS, Bhagat M, Verma K, et al. Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution. J Pediatr Urol. 2020;16: 475.e1-475.e9.
• Varan A, Kalkan N, Yalcin B, et al. Wilms tumor associated with genetic syndromes and congenital malformations. Eur J Pediatr 2017; 176: 1451.
• Akyüz C, Yalçin B, Yildiz I, et al. Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG). Pediatr Hematol Oncol. 2010; 27: 161-78.
• Vujanić GM, Sandstedt B, Harms D, Kelsey A, Leuschner I, de Kraker J; SIOP Nephroblastoma Scientific Committee. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood. Med Pediatr Oncol. 2002; 38: 79-82.
• Graf N, van Tinteren H, Bergeron C, et al. Characteristics and outcome of stage II and III non-anaplastic Wilms' tumour treated according to the SIOP trial and study 93-01. Eur J Cancer. 2012; 48(17): 3240-8.
• Warwick AB, Dome JS. Renal Tumors. In: Lanzkowsky P, Lipton JM and Fish JD, eds. Lanzkowsky’s Manual of Pediatric Hematology and Oncology. 6th ed. Amsterdam: Elsevier, 2016; 491-504.
• Kutluk T, Varan A, Büyükpamukçu N, et al. Improved survival of children with Wilms tumor. J Pediatr Hematol Oncol. 2006; 28: 423-6.
• Yildiz I, Yüksel L, Ozkan A, et al. Multidisciplinary approach to Wilms' tumor: 18 years of experience. Jpn J Clin Oncol. 2000; 30: 17-20.
• Erginel B, Vural S, Akın M, et al. Wilms' tumor: a 24-year retrospective study from a single center. Pediatr Hematol Oncol. 2014; 31: 409-14.