Abstract
Purpose of Review
Genitourinary rhabdomyosarcoma (RMS) is a relatively uncommon pediatric urologic oncologic condition with significant implications for both short- and long-term bladder functions. It is important for both pediatric and adult urologists to be aware of the pathophysiology, treatment, and long-term prognosis of this tumor and its potential impact on bladder function in both pediatric and adult survivors.
Recent Findings
Abnormalities in bladder function may arise secondary to surgical or medical management. Typical presenting complaints include lower urinary tract symptoms, which may be more common in pediatric RMS survivors than in survivors of other childhood cancers. Post-treatment evaluation of urinary function should include a baseline urodynamics study and an assessment of symptom severity and bother using validated, age-appropriate questionnaires. Upper tract monitoring should include renal/bladder ultrasound and serum creatinine. Hemorrhagic cystitis, which often develops acutely in patients receiving alkylating agents, may also present as a late effect. Lastly, urinary diversion may be required in patients with bladder/prostate (BP)-RMS either due to initial or delayed cystectomy or due to radiation-related loss of bladder function. Recent data suggest that diversion can maintain excellent quality of life in these patients.
Summary
Both BP-RMS and its treatments have the potential for profound impacts on long-term bladder function. Most BP-RMS patients will survive beyond their initial diagnosis and treatment, and it is therefore important for both pediatric and adult urologists to be aware of the pathophysiology, treatment, and long-term prognosis of this tumor and its potential impact on bladder function in both pediatric and adult survivors.
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References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
•• Dasgupta R, Rodeberg DA. Update on rhabdomyosarcoma. Semin Pediatr Surg. 2012;21(1):68–78. Excellent update on rhabdomyosarcoma pathophysiology, treatment, and outcomes.
•• Ferrer FA, Isakoff M, Koyle MA. Bladder/prostate rhabdomyosarcoma: past, present and future. J Urol. 2006;176(4 Pt 1):1283–91. Excellent review of bladder management and outcomes specifically focusing on BP-RMS.
Joshi D, Anderson JR, Paidas C, Breneman J, Parham DM, Crist W. Age is an independent prognostic factor in rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children’s Oncology Group. Pediatr Blood Cancer. 2004;42(1):64–73.
Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, et al. Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol. 2001;19(12):3091–102.
Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol. 1995;13(3):610–30.
Meza JL, Anderson J, Pappo AS, Meyer WH, Children’s Oncology G. Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: the Children’s Oncology Group. J Clin Oncol. 2006;24(24):3844–51.
Harel M, Ferrer FA, Shapiro LH, Makari JH. Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma. Urol Oncol. 2016;34(2):103–15.
Hingorani P, Missiaglia E, Shipley J, Anderson JR, Triche TJ, Delorenzi M, et al. Clinical application of prognostic gene expression signature in fusion gene-negative rhabdomyosarcoma: a report from the Children’s Oncology Group. Clin Cancer Res. 2015;21(20):4733–9.
Malempati S, Hawkins DS. Rhabdomyosarcoma: review of the Children’s Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer. 2012;59(1):5–10.
Ruymann FB, Maddux HR, Ragab A, Soule EH, Palmer N, Beltangady M, et al. Congenital anomalies associated with rhabdomyosarcoma: an autopsy study of 115 cases. A report from the Intergroup Rhabdomyosarcoma Study Committee (representing the Children’s Cancer Study Group, the Pediatric Oncology Group, the United Kingdom Children’s Cancer Study Group, and the Pediatric Intergroup Statistical Center). Med Pediatr Oncol. 1988;16(1):33–9.
Yang P, Grufferman S, Khoury MJ, Schwartz AG, Kowalski J, Ruymann FB, et al. Association of childhood rhabdomyosarcoma with neurofibromatosis type I and birth defects. Genet Epidemiol. 1995;12(5):467–74.
Leuschner I, Harms D, Mattke A, Koscielniak E, Treuner J. Rhabdomyosarcoma of the urinary bladder and vagina: a clinicopathologic study with emphasis on recurrent disease: a report from the Kiel Pediatric Tumor Registry and the German CWS Study. Am J Surg Pathol. 2001;25(7):856–64.
Wolden SL, Lyden ER, Arndt CA, Hawkins DS, Anderson JR, Rodeberg DA, et al. Local control for intermediate-risk rhabdomyosarcoma: results from D9803 according to histology, group, site, and size: a report from the Children’s Oncology Group. Int J Radiat Oncol Biol Phys. 2015;93(5):1071–6.
•• 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(6 Pt 1):2396–403. This is the first major multi-institutional group study focusing on bladder outcomes and organ preservation in BP-RMS.
Dorr W, Beck-Bornholdt HP. Radiation-induced impairment of urinary bladder function in mice: fine structure of the acute response and consequences on late effects. Radiat Res. 1999;151(4):461–7.
Hays DM, Raney RB Jr, Lawrence W Jr, Soule EH, Gehan EA, Tefft M. Bladder and prostatic tumors in the intergroup rhabdomyosarcoma study (IRS-I): results of therapy. Cancer. 1982;50(8):1472–82.
Lerner SP, Hayani A, O'Hollaren P, Winkel C, Ohori M, Harberg FJ, et al. The role of surgery in the management of pediatric pelvic rhabdomyosarcoma. J Urol. 1995;154(2 Pt 1):540–5.
Merguerian PA, Agarwal S, Greenberg M, Bagli DJ, Khoury AE, McLorie GA. Outcome analysis of rhabdomyosarcoma of the lower urinary tract. J Urol. 1998;160(3 Pt 2):1191–4 discussion 1216.
Meir DB, Inoue M, Gur U, Livne PM, Yaniv Y, Tiedeman K, et al. Urinary diversion in children with pelvic tumors. J Pediatr Surg. 2004;39(12):1787–90.
Chui CH, Spunt SL, Liu T, Pappo AS, Davidoff AM, Rao BN, et al. Is reexcision in pediatric nonrhabdomyosarcoma soft tissue sarcoma necessary after an initial unplanned resection? J Pediatr Surg. 2002;37(10):1424–9.
Rodeberg DA, Wharam MD, Lyden ER, Stoner JA, Brown K, Wolden SL, et al. Delayed primary excision with subsequent modification of radiotherapy dose for intermediate-risk rhabdomyosarcoma: a report from the Children’s Oncology Group Soft Tissue Sarcoma Committee. Int J Cancer. 2015;137(1):204–11.
Rodeberg DA, Stoner JA, Hayes-Jordan A, Kao SC, Wolden SL, Qualman SJ, et al. Prognostic significance of tumor response at the end of therapy in group III rhabdomyosarcoma: a report from the Children’s Oncology Group. J Clin Oncol. 2009;27(22):3705–11.
Mosiello G, Gatti C, De Gennaro M, et al. Neurovesical dysfunction in children after treating pelvic neoplasms. BJU Int. 2003;92(3):289–92.
Ozkan KU, Bauer SB, Khoshbin S, Borer JG. Neurogenic bladder dysfunction after sacrococcygeal teratoma resection. J Urol. 2006;175(1):292–6 discussion 296.
Yeung CK, Ward HC, Ransley PG, Duffy PG, Pritchard J. Bladder and kidney function after cure of pelvic rhabdomyosarcoma in childhood. Br J Cancer. 1994;70(5):1000–3.
Stevens MCRA, Bouvet N, Ellershaw C, Flamant F, Habrand JL, Marsden HB, et al. Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: third study of the International Society of Paediatric Oncology--SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol. 2005;20(23):2618–28.
Angelini L, Bisogno G, Alaggio R, et al. Prognostic factors in children undergoing salvage surgery for bladder/prostate rhabdomyosarcoma. J Pediatr Urol. 2016;12(4):265 e261–8.
Angelini L, Bisogno G, Esposito C, Castagnetti M. Appraisal of the role of radical prostatectomy for rhabdomyosarcoma in children: oncological and urological outcome. Ther Adv Urol. 2018;10(6):189–96.
Jiang R, Kelly MS, Routh JC. Assessment of pediatric bowel and bladder dysfunction: a critical appraisal of the literature. J Pediatr Urol. 2018;14(6):494–501.
Bauer SB, Hallett M, Khoshbin S, et al. Predictive value of urodynamic evaluation in newborns with myelodysplasia. JAMA. 1984;252(5):650–2.
Houle AM, Gilmour RF, Churchill BM, Gaumond M, Bissonnette B. What volume can a child normally store in the bladder at a safe pressure? J Urol. 1993;149(3):561–4.
McGuire EJ, Woodside JR, Borden TA, Weiss RM. Prognostic value of urodynamic testing in myelodysplastic patients. J Urol. 1981;126(2):205–9.
•• Ritchey M, Ferrer F, Shearer P, Spunt SL. Late effects on the urinary bladder in patients treated for cancer in childhood: a report from the Children’s Oncology Group. Pediatr Blood Cancer. 2009;52(4):439–46. Large multi-institutional group study focusing on bladder effects from RMS treatments in the contemporary era.
Dorr W, Bentzen SM. Late functional response of mouse urinary bladder to fractionated X-irradiation. Int J Radiat Biol. 1999;75(10):1307–15.
Heyn R, Raney RB Jr, Hays DM, Tefft M, Gehan E, Webber B, et al. Late effects of therapy in patients with paratesticular rhabdomyosarcoma. Intergroup Rhabdomyosarcoma Study Committee. J Clin Oncol. 1992;10(4):614–23.
Mangar SA, Foo K, Norman A, Khoo V, Shahidi M, Dearnaley DP, et al. Evaluating the effect of reducing the high-dose volume on the toxicity of radiotherapy in the treatment of bladder cancer. Clin Oncol (R Coll Radiol). 2006;18(6):466–73.
Jerkins GR, Noe HN, Hill D. Treatment of complications of cyclophosphamide cystitis. J Urol. 1988;139(5):923–5.
Filipas D, Fisch M, Stein R, Gutjahr P, Hohenfellner R, Thuroff JW. Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. BJU Int. 2004;93(1):125–9.
Wang HH, Zhang T, Shanahan M, Retik AB, Lee RS. Long-term urologic outcomes in genitourinary rhabdomyosarcoma: a single-center 48-year experience. Paper presented at: American Urological Association Annual Meeting, 2019; Chicago, IL.
Duel BP, Hendren WH, Bauer SB, Mandell J, Colodny A, Peters CA, et al. Reconstructive options in genitourinary rhabdomyosarcoma. J Urol. 1996;156(5):1798–804.
van Hemelrijck M, Thorstenson A, Smith P, Adolfsson J, Akre O. Risk of in-hospital complications after radical cystectomy for urinary bladder carcinoma: population-based follow-up study of 7608 patients. BJU Int. 2013;112(8):1113–20.
• Castagnetti M, Angelini L, Alaggio R, Scarzello G, Bisogno G, Rigamonti W. Oncologic outcome and urinary function after radical cystectomy for rhabdomyosarcoma in children: role of the orthotopic ileal neobladder based on 15-year experience at a single center. J Urol. 2014;191(6):1850–5. Modern-era outcomes of a large series of patients treated with cystectomy and urinary diversion.
Austen M, Kalble T. Secondary malignancies in different forms of urinary diversion using isolated gut. J Urol. 2004;172(3):831–8.
Higuchi TT, Fox JA, Husmann DA. Annual endoscopy and urine cytology for the surveillance of bladder tumors after enterocystoplasty for congenital bladder anomalies. J Urol. 2011;186(5):1791–5.
Higuchi TT, Granberg CF, Fox JA, Husmann DA. Augmentation cystoplasty and risk of neoplasia: fact, fiction and controversy. J Urol. 2010;184(6):2492–6.
Kokorowski PJ, Routh JC, Borer JG, Estrada CR, Bauer SB, Nelson CP. Screening for malignancy after augmentation cystoplasty in children with spina bifida: a decision analysis. J Urol. 2011;186(4):1437–43.
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Granberg, C., Routh, J.C. Outcomes of Bladder Preservation Following Treatment for Rhabdomyosarcoma. Curr Bladder Dysfunct Rep 14, 214–221 (2019). https://doi.org/10.1007/s11884-019-00524-9
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DOI: https://doi.org/10.1007/s11884-019-00524-9