Urinary Tract Management after Radical Cystectomy for Rhabdomyosarcoma in ChildrendWhat is the Best Option? MANAGEMENT of the urinary tract in children with rhabdomyosarcoma (RMS) continues to be a significant challenge. Due to advancements in chemotherapy and radiation, the majority of patients can be treated adequately with salvage of the native bladder. However, there is still a subset of patients who require cystectomy. In these patients traditional management has moved from incontinent diversion with an ileal or colonic conduit to continent diversion with a heterotopic intestinal catheterizable reservoir. More recently, preliminary experience is beginning to build with the use of an orthotopic neobladder. This evolution of urinary tract management in children with RMS is not dissimilar to that which occurred decades ago in adults who required cystectomy for invasive bladder cancer. While ileal neobladder reconstruction has become the gold standard for adults at many institutions,1 it is not clear at this time whether the pediatric experience should follow the same course. In this issue of The Journal Castagnetti et al (page 1850) present their 15-year experience with the use of ileal neobladder in children with RMS.2 The rationale for its use was based on the positive experience in adults at their institution with the same type of ileal neobladder known as the Padua ileal bladder (VIP).3 The initial use of the VIP in 4 children with RMS was first reported by this group in 2006.4 In this study the authors report on the use of the VIP in 8 patients with extended followup. Of the 8 patients 6 underwent VIP primarily at the time of the cystectomy while the remaining 2 underwent VIP in a delayed fashion. Of the 6 patients treated with primary VIP 4 were able to undergo long-term assessment. Of these 4 patients all demonstrated the ability to void volitionally with good efficacy. In contrast, the 2 patients who underwent delayed VIP could not void volitionally, and experienced upper tract changes and the subsequent need for clean intermittent catheterization. Given the small number of patients in this series it was not possible to definitively identify positive predictive factors to determine the subset of patients in whom VIP performs favorably.
0022-5347/14/1916-1650/0 THE JOURNAL OF UROLOGY® © 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
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The 2 main factors that need to be taken into account when considering whether to offer a neobladder are cancer control and long-term urological outcomes. Certainly cancer control, especially in the pediatric setting, takes precedence. The creation of an orthotopic neobladder requires preservation of the membranous urethra. Of the 4 patients in this series who eventually died of local recurrence and/or metastatic disease, all had negative margins and were free of urethral involvement at the initial operation. Thus, it does not appear that the absence of a positive urethral margin at the initial resection adequately predicts disease-free survival. Therefore, urethral margin status cannot be used as the ultimate factor in determining candidates for preservation of the membranous urethra and creation of a neobladder. Although the numbers are small, this series supports previous data in which it appears that the risk of local recurrence is decreased and prognosis is improved when disease is limited to the bladder without prostatic urethral involvement and when the original histology is botryoid.5 However, the preoperative determination of prostatic urethral involvement is not always straightforward. Therefore, it would make sense in terms of cancer control to divert the urinary tract initially and then wait to perform a neobladder in disease-free survivors. However, the 2 patients in this series who were treated in this fashion fared poorly and were not able to void volitionally. In many ways this defeats the purpose of having the undiversion and neobladder in the first place. In this series the long-term urological outcome of the patients who had the neobladder performed primarily and survived appears to be excellent. They had the ability to void with adequate continence and emptying of the neobladder, which parallels the adult experience.1 However, unlike in adults the long-term functionality of the reservoir will need to be maintained much longer in children. Whether the functionality of the neobladder (that is in part constructed from radiated bowel) will be maintained 30 to 40 years from now is certainly
http://dx.doi.org/10.1016/j.juro.2014.03.084 Vol. 191, 1650-1651, June 2014 Printed in U.S.A.
OPTIMIZING FUNCTION AFTER CYSTECTOMY FOR RHABDOMYOSARCOMA
in question. Ileal urinary reservoirs, whether heterotopic or orthotopic, are well-known to be prone to late complications that can be high grade in nature.6 Nevertheless, the encouraging results seen in this small subset of patients clearly demonstrate that it is possible to facilitate continence without a stoma as well as cancer-free survival in children with RMS that requires cystectomy. At present, the decision of whether a neobladder, heterotopic intestinal reservoir or intestinal conduit is most appropriate in these patients should not be in the hands of the surgeon. Rather, this decision is best left to the parents once they are educated about the present data suggesting that the desire for urinary tract reconstruction that facilitates volitional voiding can be fulfilled. However, this option comes at a potential cost of decreased cancer control. This leaves the parents with the difficult task of determining up-front whether their child will undergo neobladder surgery. This decision also comes when they do not have adequate information to determine
1651
the long-term prognosis. In addition, the family has to consider the complexities of the differences in postoperative recovery associated with the various surgical options. Depending on the parents’ choice, the consequences can be significant and unfortunate. Hopefully more widespread experience will identify a subset of patients who can have the best of both worlds, namely normal voiding and maximum cancer control. However, obtaining the data to make this determination will be difficult given the relative rarity of this condition compared to the adult experience with transitional cell carcinoma of the bladder. For now the best that we can do is tell the parents what we know and what we do not know. After that, it is their decision and our role is simply to support them in this difficult process. Earl Y. Cheng Division of Urology Lurie Children’s Hospital of Chicago and The Feinberg School of Medicine at Northwestern University Chicago, Illinois
REFERENCES 1. Lee RK, Abol-Enein H, Artibani W et al: Urinary diversion after radical cystectomy for bladder cancer: options, patient selection, and outcomes. BJU Int 2014; 113: 11. 2. Castagnetti M, Angelini L, Alaggio R et al: 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: 1850.
3. Pagano F, Artibani W, Ligato P et al: Vescica Ileale Padovana: a technique for total bladder replacement. Eur Urol 1990; 17: 149.
5. Filipas D, Fisch M, Stein R et al: Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. BJU Int 2004; 93: 125.
4. Rigamonti W, Iafrate M, Milani C et al: Orthotopic continent urinary diversion after radical cystectomy in pediatric patients with genitourinary rhabdomyosarcoma. J Urol 2006; 175: 1092.
6. Elshal AM, Abdelhalim A, Hafez AT et al: Ileal urinary reservoir in pediatric population: objective assessment of long-term sequelae with time-to-event analysis. Urology 2012; 79: 1126.
0022-5347/14/1916-1650/0 THE JOURNAL OF UROLOGY® © 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION
1650
j
www.jurology.com
AND
RESEARCH, INC.
The 2 main factors that need to be taken into account when considering whether to offer a neobladder are cancer control and long-term urological outcomes. Certainly cancer control, especially in the pediatric setting, takes precedence. The creation of an orthotopic neobladder requires preservation of the membranous urethra. Of the 4 patients in this series who eventually died of local recurrence and/or metastatic disease, all had negative margins and were free of urethral involvement at the initial operation. Thus, it does not appear that the absence of a positive urethral margin at the initial resection adequately predicts disease-free survival. Therefore, urethral margin status cannot be used as the ultimate factor in determining candidates for preservation of the membranous urethra and creation of a neobladder. Although the numbers are small, this series supports previous data in which it appears that the risk of local recurrence is decreased and prognosis is improved when disease is limited to the bladder without prostatic urethral involvement and when the original histology is botryoid.5 However, the preoperative determination of prostatic urethral involvement is not always straightforward. Therefore, it would make sense in terms of cancer control to divert the urinary tract initially and then wait to perform a neobladder in disease-free survivors. However, the 2 patients in this series who were treated in this fashion fared poorly and were not able to void volitionally. In many ways this defeats the purpose of having the undiversion and neobladder in the first place. In this series the long-term urological outcome of the patients who had the neobladder performed primarily and survived appears to be excellent. They had the ability to void with adequate continence and emptying of the neobladder, which parallels the adult experience.1 However, unlike in adults the long-term functionality of the reservoir will need to be maintained much longer in children. Whether the functionality of the neobladder (that is in part constructed from radiated bowel) will be maintained 30 to 40 years from now is certainly
http://dx.doi.org/10.1016/j.juro.2014.03.084 Vol. 191, 1650-1651, June 2014 Printed in U.S.A.
OPTIMIZING FUNCTION AFTER CYSTECTOMY FOR RHABDOMYOSARCOMA
in question. Ileal urinary reservoirs, whether heterotopic or orthotopic, are well-known to be prone to late complications that can be high grade in nature.6 Nevertheless, the encouraging results seen in this small subset of patients clearly demonstrate that it is possible to facilitate continence without a stoma as well as cancer-free survival in children with RMS that requires cystectomy. At present, the decision of whether a neobladder, heterotopic intestinal reservoir or intestinal conduit is most appropriate in these patients should not be in the hands of the surgeon. Rather, this decision is best left to the parents once they are educated about the present data suggesting that the desire for urinary tract reconstruction that facilitates volitional voiding can be fulfilled. However, this option comes at a potential cost of decreased cancer control. This leaves the parents with the difficult task of determining up-front whether their child will undergo neobladder surgery. This decision also comes when they do not have adequate information to determine
1651
the long-term prognosis. In addition, the family has to consider the complexities of the differences in postoperative recovery associated with the various surgical options. Depending on the parents’ choice, the consequences can be significant and unfortunate. Hopefully more widespread experience will identify a subset of patients who can have the best of both worlds, namely normal voiding and maximum cancer control. However, obtaining the data to make this determination will be difficult given the relative rarity of this condition compared to the adult experience with transitional cell carcinoma of the bladder. For now the best that we can do is tell the parents what we know and what we do not know. After that, it is their decision and our role is simply to support them in this difficult process. Earl Y. Cheng Division of Urology Lurie Children’s Hospital of Chicago and The Feinberg School of Medicine at Northwestern University Chicago, Illinois
REFERENCES 1. Lee RK, Abol-Enein H, Artibani W et al: Urinary diversion after radical cystectomy for bladder cancer: options, patient selection, and outcomes. BJU Int 2014; 113: 11. 2. Castagnetti M, Angelini L, Alaggio R et al: 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: 1850.
3. Pagano F, Artibani W, Ligato P et al: Vescica Ileale Padovana: a technique for total bladder replacement. Eur Urol 1990; 17: 149.
5. Filipas D, Fisch M, Stein R et al: Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery. BJU Int 2004; 93: 125.
4. Rigamonti W, Iafrate M, Milani C et al: Orthotopic continent urinary diversion after radical cystectomy in pediatric patients with genitourinary rhabdomyosarcoma. J Urol 2006; 175: 1092.
6. Elshal AM, Abdelhalim A, Hafez AT et al: Ileal urinary reservoir in pediatric population: objective assessment of long-term sequelae with time-to-event analysis. Urology 2012; 79: 1126.
