SUNA EMİR, Sonay İncesoy ÖZDEMİR, Hacı Ahmet DEMİR, DERYA ÖZYÖRÜK, Esra KARAKUŞ, Tuğrul TİRYAKİ, Faik ÇETİNDAĞ

Pediatric bladder/prostate rhabdomyosarcoma: Eight cases from a single center

Emir S, İncesoy-Özdemir S, Demir HA, Özyörük D, Karakuş E, Tiryaki T, Çetindağ F. Pediatric bladder/prostate rhabdomyosarcoma: eight cases from a single center. Turk J Pediatr 2016; 58: 254-258.In this study, clinical characteristics, treatment modalities and outcome of patients diagnosed with bladder/prostate rhabdomyosarcoma (BP RMS) were evaluated retrospectively. Files of 8 children diagnosed with BP RMS and treated between 2004-2014 were reviewed for clinical characteristics, treatment modalities and outcome. Seven males and one female were diagnosed with BP RMS between 2004-2014. Median age was 33.5 months (range, 2 to 176 months). At presentation the main clinical symptoms were hematuria in 5 patients, and constipation, oliguria and prolonged jaundice in 1 patient each. All patients were non-metastatic and only one had an embryonal histology. Primary resection before chemotherapy was performed on only one patient. Six patients were treated initially with VAC chemotherapy for 12 weeks, two patients were treated PIAV (ifosfamide, cisplatin, doxorubicin, vincristine). Local relapse or progressive disease occurred in 5 of 8 patients, and two of these patients underwent primary or secondary tumor resection without radiotherapy. Three patients developed a local relapse after combination of radiochemotherapy and tumor resection. Radical surgical treatment was performed in 3 patients with local relapse. Only one patient underwent partial cystectomy. Six of 8 patients were alive and under follow-up without disease at a median survival of 53 months (range, 13 to 78 months). BP RMS requires a multidisciplinary treatment approach. There is a general consensus that chemotherapy is the mainstay of treatment in BP RMS, but the method to be used for local control is controversial, and may vary from case to case in this heterogeneous disease.

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1. Stein R, Frees S, Schröder A, et al. Radical surgery and different types of urinary diversion in patients with rhabdomyosarcoma of bladder or prostate--a single institution experience. J Pediatr Urol 2013; 9: 932-939.

2. Hishiki T, Saito T, Mitsunaga T, et al. Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas. Pediatr Surg Int 2013; 29: 1077-1082.

3. Denes FT, Duarte RJ, Cristofani LM, Lopes RI. Pediatric genitourinary oncology. Front Pediatr 2013; 1: 48.

4. Seitz G, Dantonello TM, Int-Veen C, et al. Treatment efficiency, outcome and surgical treatment problems in patients suffering from localized embryonal bladder/prostate rhabdomyosarcoma: a report from the Cooperative Soft Tissue Sarcoma trial CWS-96. Pediatr Blood Cancer 2011; 56: 718-724.

5. Jenney M, Oberlin O, Audry G, et al. Conservative approach in localised rhabdomyosarcoma of the bladder and prostate: results from International Society of Paediatric Oncology (SIOP) studies: malignant mesenchymal tumour (MMT) 84, 89 and 95. Pediatr Blood Cancer 2014; 61: 217-222.

6. Alexander N, Lane S, Hitchcock R. What is the evidence for radical surgery in the management of localized embryonalbladder/prostate rhabdomyosarcoma?Pediatr Blood Cancer 2012; 58: 833-835.

7. Komasara L, Golebiewski A, Anzelewicz S, Czauderna P. A review on surgical techniques and organ sparing procedures in bladder/prostate rhabdomyosarcoma. Eur J Pediatr Surg 2014; 24: 467-473.

8. Arndt C, Rodeberg D, Breitfeld PP, Raney RB, Ullrich F, Donaldson S. Does bladder preservation (as a surgical principle) lead to retaining bladder function in bladder/prostate rhabdomyosarcoma? Results from intergroup rhabdomyosarcoma study iv. J Urol 2004; 171: 2396-2403.

9. Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thüroff JW. Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. BJU Int 2004; 93: 125-129.

10. Raney RB Jr, Crist WM, Maurer HM, Foulkes MA. Prognosis of children with soft tissue sarcoma who relapse after achieving a complete response. A report from the Intergroup Rhabdomyosarcoma Study I. Cancer 1983; 52: 44-50.

11. Hayes Jordan A, Doherty DK, West SD, et al. Outcome after surgical resection of recurrent rhabdomyosarcoma. J Pediatr Surg 2006; 41: 633-638.

12. Wu HY, Snyder HM 3rd, Womer RB. Genitourinary rhabdomyosarcoma: which treatment, how much, and when? J Pediatr Urol 2009; 5: 501-506.

13. Cecchetto G, Carretto E, Bisogno G, et al. Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee. Pediatr Blood Cancer 2008; 51: 593-597.

14. Kuru TH, Roethke M, Fenchel M, Hadaschik BA. How could imaging reduce therapy-associated morbidity in rhabdomyosarcoma of the bladder or prostate? Future Oncol 2013; 9: 1797-1800.

15. Rodeberg DA, Anderson JR, Amdt CA, et al. Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: combined results from the Chidren's Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. Int J Cancer 2011;128: 1232-1239.

16. Ferrer FA, Isakoff M, Koyle MA. Bladder/prostate rhabdomyosarcoma: past, presentand future. J Urol 2006; 176: 1283-1291.