Use of ultrasound for the palpable undescended testis: a wasteful practice.

Background: Many primary care physicians order an ultrasound (US) before referral to specialist care for suspected undescended testis; however, the value of this practice is questionable. Objective: To determine the proportion of boys referred for suspected undescended testis who had accompanying US, the cost of this practice and the accuracy of US for testis localization when compared with physical examination by a pediatric urologist. Methods: This was a retrospective chart review at a pediatric urology service, including all patients referred for suspected undescended testis from 2008 to 2012. We determined the cost of US ordered, and calculated Cohen's kappa, sensitivity and specificity, and positive and negative predictive value. Results: We identified 894 eligible patients; 32% (289/894) were accompanied by US. In 77% (223/289), the urologist was able to palpate the testis: 51% (147/289) had a normal/retractile testis and 26% (76/289) had a palpable undescended testis. At a cost of 71.10 CAD per US, 20 547.90 CAD was expended on this practice. Of the 223 patients with palpable testes, we were able to gather detailed US and physical examination results for 214 patients. Cohen's kappa was 0.06 (95% CI -0.005, 0.11; P = 0.10). US had 92.8% sensitivity (95% CI 84.1, 96.9%) and 15.2% specificity (95% CI 10.2, 21.9%) to detect an undescended testis. The positive predictive value was 34.2% (95% CI 27.8, 41.3%), while the negative predictive value was 81.5% (95% CI 63.3, 91.8%). Conclusions: Referral of patients for suspected undescended testis should not be accompanied by an US study as US is not useful in these cases.

Practical approach to evaluating testicular status in infants and children.

OBJECTIVE: To review the differences between normal, retractile, ectopic, ascended, and undescended testes and to describe the optimal way to perform a testicular examination to distinguish one from the other, as well as to demonstrate that ultrasound imaging is not necessary and to clarify when to consider specialist referral. SOURCES OF INFORMATION: This paper is based on selected findings from a MEDLINE search on undescended testes and orchiopexy referrals, and on our experience at the Urology Clinic at the Children's Hospital of Eastern Ontario in Ottawa, including review of referrals to our clinic for undescended testes and the resultant findings of normal variants versus surgical cases. The MeSH headings used in our MEDLINE search included undescended testicle, retractile testicle, ectopic testicle, ascended testicle, referral and consultation, and orchiopexy. MAIN MESSAGE: An undescended testis is defined as the true absence of one testis (or both testes) from normal scrotal position. Ectopic and ascended testes will likewise be absent from the scrotum, the latter having been present at one point in development. Differentiating among testicular examination findings is important, as descended and retractile testes are managed conservatively, while prompt surgical intervention should be offered for ascended, ectopic, and undescended testes. Uncertainty surrounding the diagnosis of an undescended testis causes anxiety, might lead to unwarranted imaging, and might increase the wait list for specialty assessment. For this reason, avoidance of ultrasound in the evaluation of undescended testes was included in the recent Choosing Wisely Canada campaign. We seek to clarify the physical examination findings in the evaluation of possible undescended testes, the suggested referral parameters, and the subsequent management. CONCLUSION: Undescended testes and their variants are common. As decision for referral is based on the primary care physician's physical examination findings, we clarify distinguishing between normal and abnormal findings on testicular examination to aid in appropriate referral for subspecialist evaluation. Consultation, if needed, should be sought at 6 months' corrected gestational age, or at detection if later than 6 months, without delay for ultrasound imaging, as surgical management is recommended for those patients with undescended, ectopic, or ascended testes.

Analysis of the clinical significance and cost associated with the routine pathological analysis of pediatric inguinal hernia sacs.

PURPOSE: Pediatric inguinal and scrotal surgeries for inguinal hernia, cryptorchidism and hydrocele are common and usually involve the excision of a hernia sac. Groups at many centers send hernia sacs for pathological analysis to identify occult disease as well as structures that may have been erroneously resected. We hypothesized that, since the incidence of significant findings is low and the associated health care costs are significant, the routine pathological analysis of inguinal hernia sacs is unnecessary. MATERIALS AND METHODS: After receiving institutional review board approval we retrospectively reviewed pathology reports at our institution of patients who underwent surgery with an inguinal hernia sac sent for pathological analysis from January 2000 to September 2009. The primary outcome was to determine the incidence of clinically significant structures in hernia sac specimens. The secondary outcome was to evaluate the costs associated with analyzing these specimens. RESULTS: A total of 2,287 boys and 441 girls underwent some form of inguinal or scrotal surgery during the study. In the 2,287 boys a total of 2,657 hernia sac specimens were analyzed, of which 2 (0.08%) contained clusters of epididymal-like tubules. Most unexpected findings were likely clinically insignificant, including mesothelial proliferation in 5.6% of cases, genital duct remnants in 0.8%, lipoma in 0.23% and adrenocortical rests in 0.04%. The average cost of analyzing hernia sac specimens at our institution was approximately $7,100 Canadian annually. CONCLUSIONS: Routine analysis of inguinal hernia sacs is unnecessary and costly, and should be reserved for cases in which resection of important structures such as the vas deferens is suspected.

The futility of continued surveillance of epididymal cysts - A study of the prevalence and clinico-demographics in pre- vs. post-pubertal boys.

INTRODUCTION: The first description of epididymal cysts in children appears from a 1976 case study. Since then, there have been a total of 24 indexed publications relating to pediatric epididymal cysts. Risk factors that may exist for children presenting with epididymal cyst remain unknown, as has the best method of management. And there have not been any studies looking at the cost implications of this diagnosis. The aim of this study was to assess the incidence, clinico-demographics, outcomes, and costs of epididymal cysts in pre-pubertal boys compared with a post-pubertal epididymal cyst cohort, and to assess whether this cohort requires continued surveillance METHODS:: Our institutional ultrasound (US) database was searched for all scrotal US. From these, a filtered, institution review board-approved search was performed for any reports containing the word "cyst." These were then cross-referenced with a retrospective chart review (October 2006 to September 2017). Clinico-demographics, cyst characteristics, and outcomes were analysed for both pre- and post-pubertal boys using descriptive and non- parametric statistical methods RESULTS:: Of 4508 boys undergoing scrotal US during the study period, 191 were indicated to contain cysts. This was manually reduced to 109 scans (2.4%) that met inclusion criteria (85 pre-pubertal; 24 post-pubertal). Thirty-one scans were ordered by urology, including all those with abnormal testicular echotexture (n=5). The average age of the post-pubertal cohort was 15.8 years, compared with 3.8 years in the pre-pubertal cohort. Most (70.5%) epididymal cysts were incidental. There was no difference between the pre- and post-pubertal cohorts in terms of presence of hydrocoeles (p=0.9), symptoms (p=0.9), ordering service (p=0.61), rate of resolution (4.2% vs. 8.2%; p=0.68), or length of followup (4 vs. 4.5 years; p=0.44). Pre- pubertal cysts were significantly smaller in size (3.35 vs. 14.52 mm; p=0.025) and more likely to trigger repeat scanning (67 vs. 10; p=0.008). There were no operative interventions and no subsequent clinical deterioration occurred with observation. At a cost of $71.10 CAD per US, $15 002.10 CAD was expended on epididymal cyst surveillance in direct cost to the healthcare system. CONCLUSIONS: Epididymal cysts are comparable in both pre- and post-pubertal boys and can be safely managed non-operatively without the use of continued US surveillance or urological referral. The higher than expected rate of detection may be a result of the improved ultra-resolution of modern scanners. These children should not require continued followup with repeat surveillance imaging solely for epididymal cysts and could be managed in the primary care setting as part of routine clinical examination.

Pediatric cystogram: Are we considering age-adjusted bladder capacity?

INTRODUCTION: There are a small number of reports in the literature describing bladder rupture during cystograms in children. We hypothesized that children undergoing cystograms may have their bladder overfilled during the test. We aimed to evaluate the current practice when performing cystograms in our institution, contrasting actual volume used to fill the bladder to the age-adjusted bladder capacity. METHODS: We performed a retrospective review of all voiding cystourethrograms(VCUG) and radionuclide cystograms (RNC) performed at the Children's Hospital of Eastern Ontario from 2006-2013. Formulas used to estimate age-adjusted bladder capacity: 1) infants <1 year: Capacity (mL) = (2.5 × age [months]) + 38 (Holmdahl et al, 1996); and 2) older children >1 year: Capacity (mL) = (2 + age [years]) × 30 (Koff et al, 1983). RESULTS: A total of 2411 cystograms were done (1387 VCUG; 1024 RNC) in 817 infants and 1594 children older than one year - 1113 boys and 1298 girls. Generally, bladders tended to be overfilled with 32% more volume in mL than the expected age-adjusted bladder capacity - VCUG 151 (122) mL vs 120 (97) mL (p< 0.001) and RNC 191 (97) mL vs. 151(74) mL (p<0.001). Most importantly, infants had a striking overfilling rate of 64% and 38 % more volume than their expected age-adjusted bladder capacity for VCUG and RNC, respectively (p<0.001). CONCLUSION: Bladders are filled above the estimated age-adjusted capacity in mL at the following rates: 32% in the whole group and 64% in infants undergoing VCUG. It raises concern of possible bladder rupture in this age group. Furthermore, this may lead to overgrading and overdiagnosing of vesicoureteric reflux, as well as overestimation of post-void residual. Attention should be paid to filling to age-adjusted bladder capacity and allowing the child adequate time to void during performance of the cystogram.

Pathologic and physiologic phimosis: approach to the phimotic foreskin.

OBJECTIVE: To review the differences between physiologic and pathologic phimosis, review proper foreskin care, and discuss when it is appropriate to seek consultation regarding a phimotic foreskin. SOURCES OF INFORMATION: This paper is based on selected findings from a MEDLINE search for literature on phimosis and circumcision referrals and on our experience at the Children's Hospital of Eastern Ontario Urology Clinic. MeSH headings used in our MEDLINE search included "phimosis," "referral and consultation," and "circumcision." Most of the available articles about phimosis and foreskin referrals were retrospective reviews and cohort studies (levels II and III evidence). MAIN MESSAGE: Phimosis is defined as the inability to retract the foreskin. Differentiating between physiologic and pathologic phimosis is important, as the former is managed conservatively and the latter requires surgical intervention. Great anxiety exists among patients and parents regarding non-retractile foreskins. Most phimosis referrals seen in pediatric urology clinics are normal physiologically phimotic foreskins. Referrals of patients with physiologic phimosis to urology clinics can create anxiety about the need for surgery among patients and parents, while unnecessarily expanding the waiting list for specialty assessment. Uncircumcised penises require no special care. With normal washing, using soap and water, and gentle retraction during urination and bathing, most foreskins will become retractile over time. CONCLUSION: Physiologic phimosis is often seen by family physicians. These patients and their parents require reassurance of normalcy and reinforcement of proper preputial hygiene. Consultation should be sought when evidence of pathologic phimosis is present, as this requires surgical management.

Infant nephrolithiasis and nephrocalcinosis: Natural history and predictors of surgical intervention.

INTRODUCTION: Renal stone disease diagnosed in the first year of life is relatively uncommon. While risk factors such as low birth weight, furosemide exposure, and metabolic disorders are well established, there exists little information regarding resolution rates and need for surgical intervention. Our study objective was to evaluate urolithiasis and renal calcification resolution rates, time to resolution, and need for surgical intervention in children diagnosed in their first year of life. MATERIAL AND METHODS: REB approved retrospective chart review of children younger than 12 months of age (corrected for prematurity) diagnosed with nephrolithiasis and/or nephrocalcinosis in a tertiary pediatric hospital between April 2000 and August 2015 with a minimum 1-year follow-up period. Exact logistic regression was performed to assess the relationship between size of the largest stone (on either side) and the need for surgical intervention. Kaplan-Meier curves were constructed to examine time to stone resolution among those not requiring surgical intervention. RESULTS: 62 patients (61% male) were diagnosed with stones or nephrocalcinosis by ultrasound at a median age of 2.9 months. Of these, 37% had been admitted to the NICU because of prematurity, low birth weight or comorbidities. A total of 45 patients were found to have stones (Table); 35 of these had a stone at initial ultrasound and 10 initially diagnosed as nephrocalcinosis were later confirmed to have a stone. 67% of all stones were asymptomatic on presentation. Metabolic anomalies were present in 56% (35/62), and 16% (10/62) required medical treatment. Seven patients ultimately required surgical intervention. Stone size was found to predict the eventual need for surgical intervention (OR 3.52, 95% CI 1.47-12.78) for each 0.1 mm increase in diameter). Among patients not requiring surgical intervention (n = 38), the estimated median time to spontaneous resolution of urolithiasis was 1.1 years (95% CI 0.89-1.53, range 2 months-6 years) and 1.2 years for nephrocalcinosis (95% CI 0.59-2.13). CONCLUSIONS: Spontaneous resolution was a common outcome for newborns and infants diagnosed with urolithiasis in the first year of life, but high variability in time-to-resolution was observed. Only a small proportion who had confirmed stones on ultrasound required surgical intervention (15%), and large stone size was a predictive factor for surgery.

Urothelial carcinoma of the bladder in a pediatric patient.

Urothelial carcinoma of the bladder in children and adolescents is rare. The World Health Organization database has recorded approximately 80 patients under age 16 that have been diagnosed with papillary bladder tumour since 1968.1 We are reporting on our case of urothelial carcinoma diagnosed in a 14-year-old male who presented with painless gross hematuria.

Selective use of laparoscopy in nonpalpable undescended testes.

INTRODUCTION: Approximately, 20% of undescended testes (UDT) are nonpalpable. Surgical management of the nonpalpable testis comprises laparoscopy. The aim of this study was to determine if ultrasonography can be used as a preoperative tool to localize the nonpalpable inguinal testis, eliminating the need for laparoscopy. METHODS: We identified 46 patients diagnosed with nonpalpable UDT between 2007 and 2012 who underwent an inguino-scrotal ultrasound preoperatively. We analyzed correlations between radiological and surgical findings. RESULTS: A total of 46 patients (53 UDT), median age 14 months (quartile 1(st): 7; 3(rd): 80) were included. Ultrasound localized the testis as intracanalicular in 24/53 (45.2%), intraabdominal in 10/53 (18.8%), scrotal in 1/53 (1.8%), and could not localize 18/53 (33.9%) testes. In 35/53 (66%) testes, the ultrasound location correlated with the surgical findings (P < 0.001). Ultrasound detection showed 96% sensitivity and 56% specificity for intracanalicular testes. CONCLUSION: The use of preoperative ultrasound in this series was helpful in identifying the location of nonpalpable testes in children. In particular, the ultrasound finding of an intracanalicular testis may preclude the need for laparoscopy.

Phimosis--a diagnostic dilemma?

INTRODUCTION: Phimosis is defined as the inability to retract the foreskin. Differentiating between physiological phimosis and pathological phimosis is important, as the former is managed conservatively and the latter requires surgical intervention. Referrals of patients with physiological phimosis to urology clinics may create anxiety regarding the need for surgery amongst patients and parents, while unnecessarily expanding the waiting list for specialty assessment. OBJECTIVES: To determine the ability of referring physicians to differentiate physiological from pathological phimosis, and to see whether there is any difference in this ability between generalists versus specialists. MATERIALS AND METHODS: A retrospective chart review of 284 consecutive referrals for phimosis to the Children's Hospital of Eastern Ontario (CHEO) Urology Clinic during November 2000 - April 2003 was conducted. Referral sources included family physicians (FP), pediatricians (PD), emergency physicians (ER), and other subspecialists (SS). Data for this study were obtained from the original referral letters and cross-referenced with the impressions of the pediatric urologist following the initial patient encounter. The accuracy in diagnosing phimosis was evaluated among the various types of referring physicians. RESULTS: A total of 284 phimosis referrals were reviewed of patients ranging from 2 months to 16 years of age (mean = 6.6 years). The referral sources consisted of 222-GP, 33-PD, 23-ER, and 6-SS. The majority of referred cases were diagnosed by the attending pediatric urologist as physiological phimosis across all referral sources, with the exception of subspecialists (FP = 75.2%, PD = 81.8%, ER = 56.5%, SS = 33.3%). Second to this was the diagnosis of pathological phimosis across all referral sources except SS (FP = 14.9%, PD = 12%, ER = 34.8%, SS = 50%). Overall, the circumcision rate for the 284 phimosis referrals reviewed was 14.4%. CONCLUSIONS: Our findings reveal that many physicians continue to face difficulties in distinguishing physiological phimosis from the pathological. As a result, many unnecessary referrals are made for phimosis . We suggest the implementation of improved educational measures regarding preputial pathophysiology in the medical curriculum. Such measures would serve two purposes: first, to reduce the number of unnecessary specialty referrals and secondly, to aid primary care physicians in recognizing the presence of physiological phimosis so that patients and families may be reassured of normalcy.

Topical steroid therapy for phimosis.

INTRODUCTION: Circumcision has been the traditional treatment for phimosis. Recent reports of medical management of phimosis with topical steroids quote success rates of 67%-95%. We present our results with topical steroid therapy for treatment of phimosis. MATERIALS AND METHODS: Retrospective review of 69 boys between the age of 3 and 13 years (average 7.4) who were referred to Urology clinic over a one year period (August 1999-2000) with phimosis. Boys without a distal preputial ring were excluded. A course of triamcinolone cream was prescribed and parents/patients were instructed to apply it to the preputial outlet twice daily for one month. Six weeks after treatment initiation patients were reassessed. Results were classified as follows: Full retraction (FR)-entire glans and coronal margin seen, Moderate retraction (MR)-proximal glans seen, residual preputial adhesions, Partial retraction (PR)-distal glans and meatus seen, and Failure (F)-no change. FR, MR, and PR were considered successful outcomes. Statistical analysis was performed with Splus software using the prop.test and ordinal logistic regression procedures. RESULTS: Follow-up ranged from 1-12 months, and 8 patients were lost to follow-up. Of the remaining 61 boys, 82% (12 FR, 26 MR, 12 PR) were successfully managed with topical steroid (95% confidence interval: 69%-90%). Scarring on examination was observed to negatively impact outcome (92% versus 67%) and was statistically significant using ordinal logistic regression (Chi square test=4.48, p-value=0.034). Two boys with severe balanitis xerotica obliterans (BXO) and two boys with buried penis and penoscrotal webbing failed treatment and required surgery. There was an association noted between older age and poorer outcome but this was not statistically significant. There were no local or systemic side effects noted. CONCLUSIONS: Local application of steroid cream to the phimotic foreskin may allow some degree of retraction and avert the need for circumcision. Although the length of follow-up is insufficient to decree ultimate success, topical steroid appears to be a safe and effective treatment for boys over three years of age with evidence of a tight preputial ring. Boys with severe BXO or buried penis and penoscrotal webbing should be considered primarily for surgery.

Incidentally detected nephrogenic rests in the setting of congenital obstructive uropathy.

PURPOSE: Nephrogenic rests (NR) are clusters of cells similar to renal blastema. NR are frequently seen in kidneys with Wilms' tumor (WT) and are seen with higher frequency in nephrectomy specimens from obstructed and/or multicystic dysplastic kidneys (MCDK) compared to autopsy series of normal kidneys. The significance of NR and their role in tumorigenesis is largely unknown. We report the findings of two cases with NR associated with ureteral ectopy/obstruction and review the relevant literature. MATERIALS AND METHODS: Two cases of upper pole heminephrectomy associated with ectopic upper pole ureter and resultant hydronephrosis were found to have nephrogenic rests present on pathologic examination. A literature search was done to review recent developments in the understanding of NR and their significance, primarily to guide patient recommendations regarding follow-up. RESULTS: Recent developments in the understanding of NR include the description of intralobar versus perilobar nephrogenic rests and prognostic considerations associated with each. However, the implications of finding nephrogenic rests in upper pole hemi-nephrectomy specimens associated with ureteral ectopy is not well delineated. CONCLUSIONS: The role of NR in tumorigenesis is still poorly understood. Because of the still undefined relationship with WT we recommend patients with incidentally detected NR be followed with serial abdominal ultrasounds for the first 5 years of life.

Posterior urethral valves in Eastern Ontario - a 30 year perspective.

INTRODUCTION: Posterior urethral valves (PUV) are the most common cause of male pediatric obstructive uropathy. Recent advancements in antenatal ultrasound and endoscopy have altered the presentation and management of PUV. Herein we describe the presentation, management and outcome of PUV patients in Eastern Ontario/Western Quebec over the last 3 decades. A comparison analysis of those cases identified pre and post widespread utilization of antenatal ultrasound diagnosis was performed to discern the clinical evolution of PUV with respect to long-term outcome. METHODS: Retrospective systematic chart review of all PUV cases diagnosed and treated at the Children's Hospital of Eastern Ontario over the last 3 decades. Charts were reviewed for initial presentation, method of diagnosis, radiological and clinical findings at diagnosis, initial management, and long-term clinical outcome. The evolution of PUV was interpreted by dividing the cohort into two groups chronologically delineated by the first case detected by antenatal ultrasound in the mid-1980s. These pre- and post- antenatal ultrasound eras were compared with respect to the parameters outlined above. RESULTS: Fifty-three cases were reviewed - 21 prior to widespread antenatal ultrasound screening in the mid-1980s and 32 after. There were 13/53 cases (32%) discovered by prenatal ultrasound evidence of hydronephrosis, none prior to 1985. VCUG confirmed the diagnosis in all cases. Mean age at presentation in the remaining post-natally diagnosed patients was 33 months. Of the cases diagnosed post-natally, ultrasound investigation complemented VCUG findings in 19/40 cases (47%), whereas IVP was utilized in 14/40 (35%). IVP has not been utilized for this purpose since 1987. Overall, 26/53 cases (49%) had documented VUR - 16/26 (62%) bilateral; 42/53 (79%) had hydronephrosis on ultrasound - 37/42 (88%) bilateral; 26/53 (49%) had radiological evidence of renal parenchymal damage at diagnosis; 41/53 (77%) cases had a thickened bladder wall on ultrasound at diagnosis, and 23/53 (43%) had at least one bladder diverticulum. Techniques of initial management comprised: valve ablation 32/53, vesicostomy 11/53, and high diversion 10/53. Clinically significant bladder dysfunction was found in 31% of cases, ranging from bladder instability to myogenic failure. Globally impaired renal function, as determined by significantly elevated serum creatinine levels, reduced GFR, or both, was found in 12/53 (23%). 6/53 (11%) progressed to ESRD, of which 4 received transplants. Two patients died - one from complications related to renal failure. Of the six cases of myogenic bladder failure identified, three (50%) had concurrently significant renal impairment. Average length of follow-up was 8.3 years, varying between 1 month and 18 years. CONCLUSIONS: The presentation of PUV is variable, and currently antenatal detection is the most common mode. Despite this, it still does not make up the majority of diagnoses. Complete radiological work up should include abdominal and pelvic U/S in conjunction with VCUG. Concurrent VUR in 50% of boys mandates suppressive antibiotic use. Primary valve ablation remains the gold standard for treatment of PUV, with vesicostomy reserved for selected cases. Long-term bladder and renal dysfunction is common in this population, and mandates long-term urological and nephrological follow-up.

Wilms' tumor at the Children's Hospital of Eastern Ontario: 1990-2001.

BACKGROUND: Wilms' Tumor is the most common malignant neoplasm of the urinary tract in children. Since 1969, the National Wilms' Tumor Study Group (NWTSG) has contributed to improving the clinical management and outcome of children affected by Wilms' Tumor. We have managed our patients according to NWTSG protocols and report our results herein. METHODS: Retrospective chart review of consecutive patients presenting at the Children's Hospital of Eastern Ontario (CHEO) with a diagnosis of Wilms' Tumor between April 1990 and March 2001. RESULTS: Forty patients with Wilms' Tumor (18 M/22 F) were diagnosed at CHEO during this interval. Mean age at diagnosis was 28.5 months. The most common presenting feature was a palpable abdominal mass in 85%. An overgrowth syndrome was noted in 10%. Metastatic disease was present at diagnosis in 20%, with the lungs (75%) the most common site of involvement. Distribution of clinical stage: I = 40%, II = 20%, III = 20%, IV = 15%, V = 5%. Favorable histology (FH) was diagnosed in 82.5%, anaplasia in 12.5% and clear cell sarcoma (CCSK) in 5%. Nephrogenic rests were present in 43% (perilobar (PL) = 20%, intralobar (IL) = 18%, PL + IL = 5%). Recurrence of disease occurred in 10%, with lungs (100%) the most frequent site of relapse. Survival was significantly greater in stage I disease and in those patients with FH. Patient 4-year survival data: stage I (FH), 100%; stage II (FH), 80%; stage III (FH), 100%, stage IV (FH), 67%; stages I-IV (CCSK), 100%; stage V, 0%. Overall 4-year survival rates of patients with FH were 92% and of all patients in the study was 86%. CONCLUSIONS: The presentation of Wilms' Tumor at our institution mirrors that described in the literature. Importantly, by following NWTSG protocols we have achieved an outcome of overall 4-year patient survival comparable to the "gold standard". This demonstrates the utility of the NWTSG protocols as regards patient management of this relatively uncommon disease.