Amylase Levels Are Useful for Diagnosing Omphalomesenteric Cysts: A Case Report.

Omphalomesenteric cysts are an exceedingly rare type of embryologic remnant of the omphalomesenteric duct. Owing to its rarity and unspecific imaging findings, it is occasionally difficult to diagnose preoperatively. Herein, we report the case of a 15-month-old female with an omphalomesenteric cyst that presented as a painful abdominal mass. Imaging showed a 4 cm cystic lesion just beneath the umbilicus, which also contained a 1 cm enhanced lesion. On the immediate right side of this cyst, a 7 cm hematoma was found within the abdominal wall. Aspiration revealed bloody fluid with an amylase level of 38,250 U/L. She was then diagnosed with an omphalomesenteric cyst, with aberrant pancreas and associated hematoma of the abdominal wall. These findings were confirmed with laparotomy and subsequent pathological examinations. The high level of amylase in the cyst led us to speculate the existence of ectopic pancreatic tissue. Thus, amylase measurement may be considered for the diagnosis of an omphalomesenteric cyst.

Genetic background-dependent abnormalities of the enteric nervous system and intestinal function in Kif26a-deficient mice.

The Kif26a protein-coding gene has been identified as a negative regulator of the GDNF-Ret signaling pathway in enteric neurons. The aim of this study was to investigate the influence of genetic background on the phenotype of Kif26a-deficient (KO, -/-) mice. KO mice with both C57BL/6 and BALB/c genetic backgrounds were established. Survival rates and megacolon development were compared between these two strains of KO mice. Functional bowel assessments and enteric neuron histopathology were performed in the deficient mice. KO mice with the BALB/c genetic background survived more than 400 days without evidence of megacolon, while all C57BL/6 KO mice developed megacolon and died within 30 days. Local enteric neuron hyperplasia in the colon and functional bowel abnormalities were observed in BALB/c KO mice. These results indicated that megacolon and enteric neuron hyperplasia in KO mice are influenced by the genetic background. BALB/c KO mice may represent a viable model for functional gastrointestinal diseases such as chronic constipation, facilitating studies on the underlying mechanisms and providing a foundation for the development of treatments.

Infection dynamics of Toxoplasma gondii in gut-associated tissues after oral infection: The role of Peyer's patches.

Toxoplasma gondii is a common perorally transmitted parasite; however, its immunopathogenesis in gut-associated tissues remains unclear. Here, we compared disease manifestation in C57BL/6 immunocompetent wild type (WT) mice and immunocompromised interferon (IFN)-γ-deficient (GKO) mice after peroral infection (PI) with T. gondii cysts (Fukaya strain). Strong PI-induced Th1 cytokine expression was detected in WT mice. Moreover, bradyzoite-specific T.g.HSP30/bag1 mRNA was detected in the ileum parenchyma and Peyer's patches (PP), but not in the mesenteric lymph nodes, at 7 days post-infection in WT mice, and was significantly higher than that in GKO mice. Nested PCR showed that parasites existed in ileum parenchyma at days 1 and 1.5 post-PI in GKO and WT mice, respectively. In addition, quantitative competitive-PCR indicated that T. gondii first colonized the PP (day 3 post-PI), followed by the ileum parenchyma and mesenteric lymph nodes, spleen, and portal and aortic blood (day 7 post-PI). Although parasites were consistently more abundant in GKO mice, similar invasion and dissemination patterns were observed in the two hosts. Collectively, these data suggest that some zoites differentiate from tachyzoites to bradyzoites in the ileum and that T. gondii initially invades the ileum parenchyma, and then accumulates and proliferates in the PP before disseminating through the lymphatic systems of both GKO and WT hosts.

Significance and indications for reoperative portoenterostomy in biliary atresia in light of long-term outcome.

BACKGROUND: Among biliary atresia (BA) patients undergoing revision following failed portoenterostomy, long-term native liver survival (NLS), physical condition, and indications for revision were explored. METHODS: From 1977-2015, 33 of 95 BA patients (35%) at our institution underwent revision. Ten- and 20-year NLS rates (NLSRs) and biochemical statuses of 20-year-old native liver survivors were compared between post-portoenterostomy BA patients with and without history of revision. Factors associated with 10-year NLS following revision and optimal cut-offs for potential predictors were analyzed. RESULTS: Overall 10- and 20-year NLSRs were 57% and 54%, respectively. Ten- and 20-year NLSRs were 49% and 45% in the revision group and 63% and 61% in the non-revision group, respectively. Among 20-year-old native liver survivors, differences in admission rates between ages 10-20 years were not significant for cholangitis (revision, 46%; non-revision, 40%; P = 0.30) or portal hypertension (revision, 38%; non-revision, 15%; P = 0.21). Differences in hepatobiliary function-associated blood tests between these groups at 20 years old were only significant for aspartate aminotransferase (P = 0.02) and gamma-glutamyl transpeptidase (P = 0.047). Among potential predictors of 10-year NLS that we investigated, rate of change in total bilirubin (TB) over the first month post-portoenterostomy was best (P = 0.0019), and the receiver operating characteristic curve revealed an optimal cut-off for this first-month change in TB of -3.7 mg/dl (area under the curve, 0.85; sensitivity, 0.79; specificity, 0.83). CONCLUSIONS: In approximately half of the patients, revision provided 10- and 20-year NLS, and biochemical status at 20 years old was comparable across revision and non-revision patients. Rate of change in TB during the first month post-portoenterostomy offers a sensitive predictor of revision.

Prediction of respiratory collapse among pediatric patients with mediastinal tumors during induction of general anesthesia.

PURPOSE: Fatality resulting from respiratory collapse (RC) during general anesthesia (GA) induction in children with mediastinal tumors has been reported. We explored potentially useful parameters for predicting the risk of RC based on objective imaging results. METHODS: We retrospectively reviewed the records of 31 patients (median age: 4years; range: 4months-15years) with mediastinal tumors treated between 2000 and 2015. Comparing those with RC under GA induction to those without (RC group vs. non-RC group), we analyzed a variety of factors that might predict respiratory obstruction during GA induction, including our new parameter, the standardized tumor volume (STV), which is adapted from the formula for the volume of an ellipsoid. RESULTS: All eight patients in the RC group had large tumors in the anterior mediastinum, including lymphoma, teratoma, and germ cell tumor. The mean STV value of the RC group was significantly larger than that of the non-RC group (3.6±1.4 vs. 1.4±1.0, p=0.006). Using an STV cut-off value of 2.5, the sensitivity and specificity for predicting RC under GA induction were both 0.86, making STV more useful than previously reported risk factors. CONCLUSION: Anterior mediastinal tumors in children can often cause airway obstruction under GA induction. Measuring STV can help predict the respiratory risk during GA among pediatric patients with anterior mediastinal tumors. LEVEL OF EVIDENCE: III.

Usefulness of 99mTc-DTPA galactosyl human serum albumin liver scintigraphy for evaluating hepatic functional reserve after Kasai procedure.

BACKGROUND/PURPOSE: We investigated whether 99mTc-DTPA galactosyl human serum albumin (GSA) liver scintigraphy could help evaluate post-Kasai procedure hepatic functional reserve in order to inform therapeutic strategies, including liver transplantation. METHODS: GSA scintigraphy was performed post-Kasai procedure. Patients were classified as having favorable (native liver survival) or poor (liver transplantation or death) prognoses. The clearance index (HH15), receptor index (LHL15), and time at which cumulative liver radioactivity reached 85% of heart plus liver activity (T0.85) were compared to laboratory data and prognosis. RESULTS: GSA scintigraphy was performed 106 times on 35 patients. T0.85 correlated well with laboratory variables associated with liver function (platelet count, international normalized ratio of prothrombin time, serum albumin, total cholesterol, and cholinesterase). HH15 correlated moderately and LHL15, poorly. HH15 and T0.85 values were significantly higher in the poor-prognosis than in the favorable-prognosis group, and LHL15 was significantly lower. The T0.85 cutoff value (6.25min) corresponded with division into prognosis groups, and had high sensitivity (0.78) and specificity (0.83). CONCLUSIONS: Hepatic functional reserve post-Kasai procedure can be evaluated using the GSA scintigraphy T0.85 value. A value consistently >6.25min indicates the need for liver transplantation. TYPE OF STUDY: Diagnostic Test. LEVEL OF EVIDENCE: Level III.

Significance of imaging modalities for preoperative evaluation of the pancreaticobiliary system in surgery for pediatric choledochal cyst.

OBJECTIVE: Roles of magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), drip infusion cholangiography with computed tomography (DIC-CT), and intraoperative cholangiopancreatography (IOCP) in visualizing pancreaticobiliary anatomy for pediatric choledocal cyst (CC) operations were examined. METHODS: From 1980-2013, 117 pediatric CC patients (median age, 3 years) underwent hepaticojejunostomy at our institution, with imaging modalities of ERCP (n = 81 over 34 years), MRCP, DIC-CT, and IOCP (n = 45, 20, and 45 cases over the last 12 years). First, visualization rates for pancreaticobiliary maljunction (PBM), common bile duct (CBD), pancreatic duct (PD), and intrahepatic bile duct (IHBD) were investigated. Sensitivity, specificity, and accuracy for detecting hepatic duct stricture were then compared between MRCP and IOCP. RESULT: Visualization rates of PBM, CBD, PD, and IHBD were 57%, 100%, 64%, and 100% for MRCP; 82%, 77%, 95%, and 32% for ERCP; 25%, 75%, 21%, and 85% for DIC-CT; and 87%, 100%, 87%, and 100% for IOCP, respectively. Combination of MRCP and IOCP achieved rates of 89%, 100%, 91%, and 100%, respectively. Sensitivity, specificity, and accuracy for detecting stenosis were 86%, 68%, and 71% for MRCP, and 100%, 89%, and 91% for IOCP, respectively. CONCLUSIONS: Combining MRCP and IOCP can provide satisfactory pancreaticobiliary anatomical information for surgical planning for pediatric CC, with hepatic duct strictures evaluated more accurately by IOCP.

Ellis-van Creveld syndrome associated with chronic intestinal pseudo-obstruction.

Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive disorder characterized by hypoplastic nails, polydactyly, and achondroplasia. Patients usually exhibit normal cognitive function and no remarkable developmental delay. We herein present an unusual case of EVC syndrome. A Japanese 2-year-old boy was born at term, but immediately developed severe respiratory failure due to thorax deformity, postaxial polydactyly and nail hypoplasia. We identified a novel pattern of germinal compound heterozygous nonsense EVC2 mutations of c.1814C > A (p. S605X) and c.2653C > T (p. R885X), leading to the diagnosis of EVC syndrome. Interestingly, he also had severe developmental delay, and suddenly developed excessive abdominal distension at the age of 2. On surgery, extensive necrotic bowel with chronic intestinal pseudo-obstruction was noted. This is, to our knowledge, a most severe phenotype of EVC syndrome, illustrating that the specific pattern of EVC2 compound heterozygous mutations may cause severe developmental delay and intestinal malfunction.

Antibody-dependent cellular cytotoxicity toward neuroblastoma enhanced by activated invariant natural killer T cells.

Anti-ganglioside GD2 antibodies mainly work through antibody-dependent cellular cytotoxicity (ADCC) and have demonstrated clinical benefit for children with neuroblastoma. However, high-risk neuroblastoma still has a high recurrence rate. For further improvement in patient outcomes, ways to maximize the cytotoxic effects of anti-GD2 therapies with minimal toxicity are required. Activated invariant natural killer T (iNKT) cells enhance both innate and type I acquired anti-tumor immunity by producing several kinds of cytokines. In this report, we investigated the feasibility of combination therapy using iNKT cells and an anti-GD2 antibody. Although some of the expanded iNKT cells expressed natural killer (NK) cell markers, including FcγR, iNKT cells were not directly associated with ADCC. When co-cultured with activated iNKT cells, granzyme A, granzyme B and interferon gamma (IFNγ) production from NK cells were upregulated, and the cytotoxicity of NK cells treated with anti-GD2 antibodies was increased. Not only cytokines produced by activated iNKT cells, but also NK-NKT cell contact or NK cell-dendritic cell contact contributed to the increase in NK cell cytotoxicity and further IFNγ production by iNKT cells and NK cells. In conclusion, iNKT cell-based immunotherapy could be an appropriate candidate for anti-GD2 antibody therapy for neuroblastoma.

Coexistence of neuroblastoma and ganglioneuroma in a girl with a hemizygous deletion of chromosome 11q14.1-23.3.

Constitutional 11q interstitial deletion syndrome presents with congenital anomalies including microcephaly with craniostenosis, minor dysmorphic features, vitreoretinopathy, and renal anomalies. This syndrome is occasionally associated with neuroblastoma (NB) as a life-threatening complication, which is important for clinical care. Although the corresponding locus to NB has been predicted to exist in 11q22-23 by previous deletion studies related to NB, the causative haploinsufficient genes have not yet been identified. We herein reported for the first time the simultaneous coexistence of adrenal NB and abdominal prevertebral ganglioneuroma in a 6-year-old girl with a constitutional hemizygous 11q14.1-23.3 deletion. Of the 11 haploinsufficient genes predicted with an in silico database, we focused on NCAM1 and CADM1 as the genes accountable for NB and ganglioneuroma. The deletion range, especially the 11q22.3 involvement, needs to be determined in 11q deletion cases in order to predict susceptibility to peripheral nerve tumors involving NB and ganglioneuroma.

Risk Factors for Intestinal Obstruction After Ladd Procedure.

Intestinal obstruction is a common complication after Ladd procedure. Ninety-three cases who had undergone the Ladd procedure between 1977 and 2013 treated at our own institution were retrospectively reviewed to identify the causes and risk factors for intestinal obstruction. The Ladd procedure has been performed without any intestinal fixing. Of the 87 cases who survived to discharge, intestinal obstruction was observed in 22 (25.3%). Among the cases with intestinal obstruction, 13 (59.1%) showed intestinal ischemia at the initial operation; this incidence was notably high, although it is low when only those cases with another concurrent surgical digestive disease are considered. All cases of intestinal obstruction were caused not by recurrent volvulus, but by adhesion between the intestine and the mesentery. Intestinal fixing is not required to prevent recurrent volvulus, but it is important to achieve adequate widening of the mesenteric base. The risk of intestinal obstruction after the Ladd procedure, on the other hand, is high. Moreover, patients with intestinal ischemia have an increased risk of intestinal obstruction.

Evidence for viral infection as a causative factor of human biliary atresia.

OBJECTIVES: To explore the evidence for viral infections triggering human biliary atresia (BA) by reviewing archival original articles that analyzed human samples via polymerase chain reaction (PCR) experiments, considering the recent experimental trend of extensive use of rotaviral BA animal models. METHODS: A PubMed search retrieved original articles that reported the results of PCR experiments for detecting viral DNA or RNA in patient samples as proof of past infection. Search terms included the often-debated DNA or RNA viruses and BA. Special focus was directed toward PCR analyses that targeted reovirus and rotavirus, where PCR accuracy, specimen characteristics and their interpretations were compared. RESULTS: Nineteen studies were conducted on 16 different kinds of viruses using PCR, with 5 studies on reovirus, 3 on rotavirus, 10 on cytomegalovirus, 5 on Epstein-Barr virus, 4 on parvovirus B19, and so on. Among the papers suggesting a possible viral link to only BA, there was no study on reovirus, 1 on rotavirus, 3 on cytomegalovirus, 1 on EB virus, and 1 on papillomavirus. Of the 6 PCR studies on Reoviridae, 3 on reovirus and 2 on rotavirus were evaluated rigorously for experimental accuracy, including their sensitivity. Two research groups analyzed preoperative stool samples in addition to generic hepatobiliary tissue obtained at surgery. Sample collection timing varied widely, with storage period prior to PCR experimentation not revealed in most reports on Reoviridae. CONCLUSION: Although a considerable number of PCR studies have sought to clarify a viral role in the pathogenesis of BA using human samples, the findings have been contradictory and have not succeeded in achieving an obvious differentiation between causative and accidental infection of the focused virus. Reproducible and convincing evidence for a causative Reoviridae infection has been lacking based on objective data from highly sensitive PCR experiments. Even though the possibility remains of viral disappearance at the timing of collection, to avoid further ambiguous interpretations of PCR results, rigorous and meticulous collection of large numbers of specimens at carefully planned timing, along with a strictly adjusted and finely tuned PCR system, is strongly recommended for obtaining more reliable and consistent results.

Malignant rhabdoid tumor of the liver: a case report and literature review.

Malignant rhabdoid tumor (MRT) is a rare and aggressive malignancy associated with poor outcomes. MRT of the liver is even rarer, and little information has been described. We report the case of an 8-month-old boy with MRT of the liver. The tumor showed aggressive progression despite a multidisciplinary approach, and the patient died due to multiple organ failure 14 days after admission. Autopsy revealed the liver tumor and multiple metastases with negative immunohistochemistry for INI1/BAF47. A review of 53 cases of pediatric MRT of the liver is provided.

Endoscopic management for congenital esophageal stenosis: A systematic review.

Congenital esophageal stenosis (CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscular thickening, tracheobronchial remnants (TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography (EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.

Hybrid video-assisted thoracoscopic surgery lobectomy of fissureless congenital cystic adenomatoid malformation: a case report.

INTRODUCTION: Thoracoscopic lobectomy for congenital pulmonary airway malformation has been indicated from the neonatal period to adolescence. However, it is difficult to approach the pulmonary artery for lobectomy in congenital lung malformations with incomplete or absent interlobar fissures. Multidetector computed tomographic images and computed tomography pulmonary angiography gave us helpful information before the operation. We performed thoracoscopic lobectomy for congenital pulmonary airway malformations with absent interlobar fissures and adhesions in accordance with information from multidetector computed tomographic images. CASE PRESENTATION: A 14-year-old Japanese girl received a diagnosis of congenital pulmonary airway malformation when she presented with pneumonia. Using multidetector computed tomography and three-dimensional reconstruction provides meticulous characterization of the anatomy in pediatric patients. We confirmed that her left A4+5 artery arose from her left pulmonary artery medial to A6. Her left pulmonary artery was divided just proximal to the A6 origin before the lobes were separated safely. We took advantage of using a stapler to divide the fissureless thick parenchyma. Perioperative diagnosis was congenital cystic adenomatoid malformation. CONCLUSIONS: We used preoperative multidetector computed tomography to outline the bronchovascular anatomy and guide hybrid video-assisted thoracoscopic surgery for a congenital cystic adenomatoid malformation in a fissureless left lung.

Histological features of primary tumors after induction or high-dose chemotherapy in high-risk neuroblastoma.

PURPOSE: In the recent years in Japan, an increasing number of patients with neuroblastoma (NB) are being treated by the "delayed local treatment (DL)" policy, undergoing surgery after the completion of high-dose chemotherapy with hematopoietic stem cell rescue (HDC). We reviewed the histopathological findings of second-look operations, including those of patients treated with DL. PATIENTS: From 1998 to 2013, 26 patients with high-risk NB underwent radical operation following chemotherapy. Surgery was performed after induction chemotherapy in 17 cases (standard; STD), whereas 9 cases completed induction chemotherapy and HDC before undergoing tumor resection (DL). The amount of necrosis and the degree of differentiation within the post-treatment tumor were assessed. RESULTS: Eighty-eight percent of the tumors showed necrosis in more than 1/3 of the specimen. Two DL cases showed complete disappearance of viable tumor cells. Amount of necrosis did not affect the prognosis of the patient. Tumors with immature, poorly differentiated phenotypes showed an extremely aggressive thereafter. Though not statistically proven, (123)I-MIBG (metaiodobenzylguanidine) uptake may be correlated with the amount of viable cells remaining within the tumor, but not with the degree of differentiation. CONCLUSIONS: Our results support the previous reports advocating that tumors that sustain unfavorable histology after chemotherapy behave aggressively thereafter.

Role of pediatric endoscopic retrograde cholangiopancreatography in an era stressing less-invasive imaging modalities.

BACKGROUND: The significance of pediatric endoscopic retrograde cholangiopancreatography (ERCP) according to age or disease variation is inconclusive. This study aimed to evaluate the usefulness of pediatric ERCP in diagnosing or treating small children with pancreaticobiliary disorders, including choledochal cyst (CC) and biliary atresia (BA). METHODS: From 1980 to 2011, 235 ERCPs were performed in 220 pediatric patients (median age, 2 years) at our institution. Underlying pathology was CC in 92 patients (3 years), BA in 62 patients (55 days), and others in 66 patients. Success and complication rates, and ERCP findings were retrospectively analyzed. RESULTS: The overall success rate was 96%. Rates for individual pathologies were 99% for CC, 92% for BA, and 96% for others. The success rate was 100% among children >3 years, but 92% when limited to infants. Post-ERCP hyperamylasemia and duodenal perforation occurred in 9% and 0.4% of cases, respectively. Regarding preoperative evaluation of the pancreaticobiliary system in CC, ERCP clearly delineated pancreaticobiliary maljunction (79%) and the pancreatic duct (94%), whereas it visualized the common bile duct and intrahepatic bile duct at relatively low rates (77% and 33%, respectively). ERCP successfully identified 16 cases (18%) with non-BA of 90 patients with suspected BA. Moreover, ERCP demonstrated only pancreatic duct in 70% of all BA. CONCLUSIONS: ERCP among children including infants with CC and BA can be performed with fairly satisfactory results. Although pediatric ERCP can also help describe the pancreaticobiliary system in detail, its indication should be deliberately considered when anatomical information from less-invasive imaging modalities is insufficient.

Urinary amylase/urinary creatinine ratio (uAm/uCr)--a less-invasive parameter for management of hyperamylasemia.

BACKGROUND: The serum level of amylase (sAm) is commonly used as a biochemical marker for diagnosis and management of pancreatic disorders. However, the use of the urine level of amylase (uAm) is limited in practice, because the diagnostic ability of uAm is inferior to that of sAm. In the present study, the possible concordance of uAm-rerated parameters with sAm was investigated, and evaluate the usefulness of uAm for management of hyperamylasemia. METHODS: From June 1995 to October 2009, 804 samples of both urine and blood were collected from 128 patients in order to measure the serum level of amylase (sAm) and the urine level of amylase (uAm) and creatinine (uCr). Concordance of parameters using uAm compared to sAm was assessed. Parameters used were uAm, amylase creatinine clearance ratio (ACCR), and the ratio of uAm to uCr (uAm/uCr). RESULTS: uAm/uCr had the best correlation with sAm (r = 0.779, p < 0.001) compared to uAm (r = 0.620, p < 0.001) and to ACCR (r = 0.374, p < 0.001), when sAm was over the standard level. The area under the receiver operating characteristic curve of uAm/uCr (0.884) was significantly higher than that of uAm (0.766) and of ACCR (0.666) (p < 0.001 for each). The cutoff value of uAm/uCr was 569.8, with a sensitivity of 81.0% and a specificity of 83.1%. CONCLUSIONS: The uAm/uCr ratio correlated with sAm, and may be an alternative to sAm for prediction of hyperamylasemia. Use of urine samples results in a decreased need for blood sampling, which is especially beneficial in pediatric patients.

Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas.

BACKGROUND: Soft tissue sarcomas (STS) of pelvic origin in boys often involve the urogenital organs. The optimal extensiveness of radical surgery has long been an issue of discussion, since exenterative surgeries result in severe urogenital adverse effects. We conducted a retrospective review of patients with pelvic STS treated in two regional center hospitals and assessed the radicality of surgery and the functional outcome of the bladder. PATIENTS: Medical records and surgical reports of nine cases (embryonal rhabdomyosarcoma 6, malignant triton tumor 2, suspected rhabdomyosarcoma 1) treated within 1997-2012 were reviewed. Site of origin was prostate in seven, retroperitoneal in two. Average follow-up period was 96 months. TREATMENT AND OUTCOME: All cases were subjected to neoadjuvant chemotherapy. Response was PR in four, SD in two, and PD in two. Radical surgery resulted in gross total resection in eight, and partial resection in one. Three underwent cystoprostatectomy, two cases underwent prostatectomy, and bladder-preserving tumor resection was carried out in four cases. At the last follow-up, three retained a functional bladder. Two required augmentation cystoplasty with intestinal conduits. CONCLUSIONS: The majority of the on-going clinical trials recommend conservative surgery for bladder/prostate rhabdomyosarcoma, and to preserve the bladder function particularly in chemosensitive tumors. Some other groups, however, advocate the importance of radical surgery to prevent local relapse. These reports include heterogenous group of patients in the cohort, and therefore it is difficult to draw a conclusion of which approach truly contributes to the survival of the patients better. Future studies should evaluate bladder and sexual function objectively to establish reliable evidence regarding the benefit and adverse effects of different surgical approaches. These data would be informative to optimize the treatment balance for children with pelvic rhabdomyosarcomas.