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Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare soft tissue tumor of childhood usually found in the extremities. The authors present the case of a 17-year-old girl who presented with right flank pain and hematuria and during operation was found to have a right ureteral mass. The histopathologic, immunohistochemical, ultrastructural, and cytogenetic characteristics of the excised mass were consistent with extraosseous ES/PNET. This is the first known reported case of extraosseous ES/ PNET of the ureter. The pathologic features and clinical management of this case, as well as a review of the literature, are presented.

Background/purpose: Young patients with differentiated thyroid cancer typically present with regional lymph node involvement (60% to 80%), and 10% to 20% have distant metastases. This study characterizes the clinical presentation, treatment, and outcome in patients with differentiated thyroid cancer who were less than 21 years of age at diagnosis and who presented with distant parenchymal metastases., Methods: A retrospective, multi-institutional data base that included 327 patients in this age group with differentiated thyroid carcinoma was searched for patients who presented with distant metastases, and 83 cases (25%) were found. The median time to first disease progression was 2.4 years (range, 0.1 to 12.4 years) and the overall median follow-up was 10.9 years (range, 1.0 to 42.1 years)., Results: The median age at diagnosis was 14.6 years (range, 6.6 to 20.8 years); 69% were girls and 92% were white. In 12%, there was a history of prior head and neck irradiation, and 10% of these patients had a family history of carcinoma. Preoperative needle biopsies were performed in 25%. Regional lymph nodes were positive in 90%, and extrathyroidal extension occurred in 48%. The site of distant metastases included the lungs in all patients. Total thyroidectomy, subtotal thyroidectomy, lobectomy, and nodule excision was done in 66%, 24%, 3%, and 8% of patients, respectively. There was no residual cervical disease after surgery in 75%, whereas 14% had microscopic and 11% had gross residual. Histopathologic subtypes included papillary-follicular (48%), papillary (42%), and follicular (10%). The median tumor size was 3.0 cm (range, 0.4 to 11.0 cm). In this group, 100% of patients received adjuvant iodine 131I therapy, and the overall survival rate at 10 years was 100%. The progression-free survival rate was 76% at 5 years and 66% at 10 years from diagnosis., Conclusions: A significant number of young patients with thyroid cancer present with distant metastases and will require radioiodine therapy. This should be considered when planning the surgical approach because total or subtotal thyroidectomy facilitates 131I imaging and treatment. Although about one third of these patients will experience relapse or disease progression, the overall mortality rate is low.

A 4-year-old boy presented with a duodenal hematoma and was admitted for conservative management including nasogastric tube drainage and parenteral nutrition. Within 2 days, the child became fungemic and went on to require urgent laparotomy. This previously undescribed life-threatening complication of duodenal hematoma is discussed in the context of standard treatment of this injury.

Background/purpose: Neuroblastoma is the most solid common extracranial malignancy in childhood. Despite multimodality treatment, high-risk disease continues to carry a poor prognosis. Glucocorticoids have been shown previously to induce differentiation in murine neuroblastoma cell lines, but no such effect has been documented in human neuroblastoma cells. Glucocorticoids are known to be active in the differentiation process of the neural crest. These studies describe the effects of dexamethasone on 6 human neuroblastoma cell lines., Methods: Dexamethasone was added to cultured neuroblastoma cell lines (LA1-5S, LA1-15N, BE[2]S, BE[2]N, SH-EP-1, SH-SY5Y) maintained in media supplemented with either normal serum or charcoal-depleted serum. Proliferation assays were performed, and flow cytometry was used to assess alterations in cell cycle. Cells were closely monitored for morphological changes with serial phase-contrast microscopy. Immunohistochemistry (3F8, NF-1, TRK-A) of cultured cells was used to evaluate differentiation. Glucocorticoid receptor levels was assessed using immunoblotting., Results: Dexamethasone decreased the rate of cellular proliferation in both standard and charcoal-depleted conditions. Flow cytometry showed a G1 accumulation. Increased expression of the differentiation-associated antigens was found in cells cultured in charcoal-depleted media, and a further augmentation was seen with the addition of dexamethasone. In standard media, dexamethasone had no detectable effect on the expression of these antigens. Glucocorticoid receptor expression was found to be comparable in all cell lines., Conclusions: Human neuroblastoma cells are sensitive to the differentiating effects of dexamethasone in an environment of charcoal-depleted serum. This phenomenon may be caused by the existence of growth and mitogenic factors in serum that are inhibiting differentiation.

Background/purpose: Extended left hepatectomy, also referred to as left hepatic trisegmentectomy, in which segments II, III, IV, V, and VIII are excised, is rarely performed in children. Experience with 7 such resections is reported to describe the anatomy, technique, indications, and outcomes of the operation., Methods: The medical records of all pediatric patients treated at our institution over the last 15 years who underwent extended left hepatectomy were reviewed. Demographic information as well as operative, pathological, and follow-up data were analyzed., Results: Seven patients underwent extended left hepatectomy over this period. There were 5 boys and 2 girls ranging in age between 4 months and 9 years with a median age of 3.1 years. Follow-up ranged from 8 months to 5 years with a median of 3.5 years. Diagnoses included hepatoblastoma (HB, n = 3), focal nodular hyperplasia (FNH, n = 1), leiomyosarcoma (LMS, n = 1), hepatocellularcarcinoma (HCC, n = 1), and metastatic neuroblastoma (NB, n = 1). All surgical margins were grossly negative. Median operative blood loss was 13 mL/kg (range, 5 to 32 mL/kg), and mean hospital stay was 9 days (range, 7 to 12 days). No major intra- or postoperative complications were encountered, and there was no perioperative mortality. The 3 HB patients, 1 FNH patient, 1 LMS patient, and 1 NB patient are without evidence of disease, whereas the 1 child with HCC died of recurrent and distant disease. The 6 surviving children have normal hepatic function., Conclusion: Although technically challenging and rarely performed, extended resection of the left hepatic lobe is feasible in children and can yield curative results with minimal morbidity.

Background/purpose: Although there has been a precedent of testicular-sparing surgery in some centers, the authors find it is still not general practice among pediatric surgeons. To address this and emphasize the role of testicular-sparing surgery in children, four patients with testicular masses are presented who underwent this procedure., Methods: Four patients who underwent testicular-sparing surgery between the years 1993 and 1998 were reviewed. Demographic data, histopathology, and follow-up data were obtained from office charts. The period of follow-up ranged from 1 to 5 years., Results: Four patients whose ages at diagnosis were 1, 2, 4, and 17 years presented with unilateral testicular masses. The alpha-fetoprotein and beta-human chorionic gonadotropin levels were within normal limits. Testicular ultrasonography was carried out on all patients, and groin exploration with spermatic cord isolation was performed in each case. After enucleation, frozen sections to confirm benignity was carried out before repair of the testis. Follow-up of 6 months to 5 years has shown no recurrence, and on examination, testicular volume is normal in all cases., Conclusions: Testicular-sparing surgery preserves testicular volume, which is important for both cosmetic and functional purposes. It is a viable and useful method in the management of benign testicular tumors in children.

Background/purpose: Mediastinal masses commonly are referred to the pediatric surgeon and can be difficult diagnostic problems. Various techniques have been used to perform biopsy in the mediastinum, but there are few reports of mediastinoscopy or Chamberlain procedure in children in the literature. The authors reviewed their experience with these techniques in a pediatric oncology population., Methods: The medical records of all patients on the pediatric surgical service between 1987 and 1997, inclusive, who underwent mediastinoscopy or Chamberlain procedure were reviewed. Demographic data, diagnostic accuracy, complications, operating time, and blood loss were recorded., Results: Sixteen consecutive patients underwent 13 Chamberlain procedures and six mediastinoscopies over the above period. Diagnostic accuracy was found to be 95% overall (100% for Chamberlain procedure, 83% for mediastinoscopy). Five complications occurred in the Chamberlain group and none in the mediastinoscopy group. No complication required thoracotomy or sternotomy. Among patients whose sole reason for admission was diagnosis of a mediastinal mass, the mean hospital stay was 1.7+/-0.8 days (n = 7) for those who underwent Chamberlain procedure and 1.4+/-0.9 days (n = 4) for those who underwent mediastinoscopy. One mediastinoscopy was performed as an ambulatory procedure., Conclusion: Mediastinoscopy and the Chamberlain procedure are effective and safe techniques for biopsy of mediastinal masses in this age group.

Background/purpose: Bronchogenic cysts and esophageal duplications are usually considered as separate foregut malformations. Yet, both are thought to arise from the same embryological event, division of the embryonic foregut, and they share common histological characteristics, often making their clinical differentiation difficult., Methods: A retrospective review of the cases of 68 children treated at a single institution between 1937 and 1995 was performed. Thirty children were girls (44%) and 38 were boys (56%). Ages ranged from newborn to 24 years. Complete records were available in all children. Fourteen of these 68 children were asymptomatic., Results: Respiratory (54%) or gastrointestinal (13%) symptoms were the most frequent presenting problems. The majority of children were treated by resection of the cyst (52 of 68; 76%), while 9 of 68 (13%) required lobectomy for intraparenchymal lesions. Three children underwent marsupialization, with all of these children requiring additional surgery for recurrent disease. Five children (5 of 68; 7%) had multiple cysts. The mortality rate from this series was 10% (7 of 68). Two deaths were caused by perioperative exsanguination, one related to bleeding from a cyst lined with gastric mucosa with subsequent ulceration and hemorrhage into the esophagus. Two deaths occurred secondary to septic complications, one from an esophageal leak and the other from an intraparenchymal abscess. Two deaths were caused by respiratory failure; one was unrelated (SIDS). The majority of cysts found on histological review were lined by respiratory epithelium or bronchial glands (51 of 68; 75%). Gastrointestinal epithelium was present in cysts of nine children, only two of which were clinically diagnosed as esophageal duplications. Twenty-one cases (21 of 68; 31%) were classified as esophageal duplications based on the intramural location of the cyst, yet 15 of 21 (71%) contained respiratory epithelium, substantiating the hypothesis of the common origin of these lesions., Conclusions: The histological similarity and anatomic proximity of the "bronchogenic cysts" and the intramural "esophageal duplications" supports their common origin. The possible complications of bleeding, ulceration, infection, and obstruction of the esophagus or airway, should generally lead to prompt resection.

In human neuroblastoma cell cultures, two phenotypes with differing biological properties have been described: neuroblastic (N) and substrate-adherent (Schwannian or S). In nude mice, N cells are rapidly growing, clonigenic in soft agar, and tumorigenic, whereas S cells are not. The difference in malignant properties between these two cell types and their ability to interconvert in vitro may have clinical relevance. In an attempt to identify genes that may be important in the phenotypic differences and the interconversion, the authors analyzed five representative N and three S cell lines for differential expression of six genes relevant to uncontrolled growth. Beta2-microglobulin was the only gene measured that showed differential expression between the N and S cell lines. This finding supports the theory that beta2-microglobulin and class I MHC expression are markers for differentiation as well as the use of beta2-microglobulin as a differentiation marker in neuroblastoma. The data also suggest that a disregulation of the beta2-microglobulin gene may be partly responsible for the tumorigenicity of the N phenotype.

To identify prognostic factors for renal carcinoma in young patients, a retrospective analysis was performed of 22 patients (< or = 21 years of age) with histologically verified renal cell carcinoma. Demographic, staging, and treatment variables were collected in a database, and their effect on survival was determined using Kaplan-Meier probability distribution. The median age was 15.5 years (range, 3 to 21 years), and the male:female ratio was 13:9. Only three patients were black. Histopathologic examination showed 15 clear cell tumors, 4 mixed cell type, 2 papillary, and one well-differentiated adenocarcinoma. The median size of the primary tumor was 10 cm (range, 5 to 20). There were seven patients with stage I tumors, one with stage II, and 14 with stage IV. Complete resection of the primary tumor was accomplished in 12 patients. The overall 5-year survival rate was 30% (confidence interval, 20% to 40%). The 5-year survival rate was better for patients who had complete resection of the primary tumor (60% v 10%). Unresectability was associated with involvement of nodes and/or occurrence of metastases, thus an independent effect of complete resection on survival could not be demonstrated. The data showed that age, tumor size, location, and histology were not predictors of outcome; tumor stage and complete surgical resection were the only meaningful prognostic factors. The presentation of renal cell carcinoma as a localized or systemic disease may reflect a twofold biological behavior. In the first group, the disease is curable with resection, in the second, it is unaffected by surgery or adjuvant therapy. In light of the very low incidence of this renal malignancy in childhood, prospective multicenter studies will be required to improve the poor therapeutic results.

Purpose: The treatment of pericardial effusion resulting in cardiac tamponade has undergone an evolution in recent years, with the use of less invasive drainage methods in selected cases. To determine optimal therapy for pediatric oncology patients with pericardial effusion and tamponade, the authors reviewed their institutional experience with percutaneous catheter drainage., Methods: Patient records and operative reports were reviewed, and nine patients were identified who met clinical and echocardiographic criteria of cardiac tamponade and were treated with percutaneous pericardial catheter drainage., Results: The median age at time of diagnosis was 14 years (range, 5 months to 19 years), and the male:female ratio was 7:3. Underlying malignancies included acute myeloblastic leukemia in three, acute lymphoblastic leukemia in one, and Hodgkin's disease, B-cell lymphoma, medulloblastoma, desmoplastic small round cell tumor, and rhabdomyosarcoma in one each. EIght patients (89%) were receiving granulocyte colony-stimulating factor (GCSF) during the period when tamponade developed. All patients had a large or moderate-to-large pericardial effusion and right ventricular collapse with hemodynamic compromise on echocardiography, and two patients (22%) also had pericardial thickening. In nine patients, percutaneous catheter drainage was performed intraoperatively and under fluoroscopic or echocardiographic guidance. A median of 300 mL (range, 82 to 500 mL) of fluid was removed from the pericardial sac during the initial drainage, and cytology was positive in one (6%). Complete echocardiographic resolution was observed in eight patients (89%); a small posterior component persisted in one patient but was not significant hemodynamically. The catheters remained in place for a median of 5 days (range, 1 to 35 days) while repeat aspirations were performed. Tamponade resolved in all patients, and one died of overwhelming systemic sepsis. The survival period was 10 to 22 months, and tamponade or the drainage procedure did not contribute to death. Four patients remain alive after 4 month to 7 years of follow-up., Conclusion: Cardiac tamponade was effectively treated in all patients and did not recur with percutaneous catheter drainage alone. THere was no evidence of pericardial loculation or infection despite pancytopenia being prevalent with underlying illness and chemotherapy. Percutaneous catheter drainage is an effective treatment for pediatric oncology patients with pericardial tamponade. Because of its simplicity in comparison to move invasive techniques, initial treatment with percutaneous drainage should be considered in this patient population.

Hepatoblastoma is the most common pediatric liver cancer, and complete resection is required for long-term survival. There are few reported hepatoblastoma cell lines available for study. In order to develop in vitro and animal models, the authors isolated an additional cell line (HB1) from a human hepatoblastoma and here report its characteristics in culture. Cells were analyzed for growth rate, clonigenicity, and tumorigenicity, using electron microscopy. Immunocytochemistry and Northern analysis for the expression of specific surface markers and genes of interest were also performed. HB1 cells required fetal calf serum but grew well in culture (population doubling time of 2 days) and had an undifferentiated appearance under electron microscopy. Epidermal growth factor (EGF) and hydrocortisone stimulated growth with or without serum, but not autocrine growth stimulation via the epidermal growth factor receptor was detected. Proteins produced by HB1 cells under normal culture conditions included alpha-fetoprotein, cytokeratins 8 and 18, and lactate dehydrogenase (with an isozyme subunit composition similar to that of liver). HB1 cells showed chronic, high expression of c-myc and Ha-ras oncogenes but no N-myc and apparently normal RB gene expression. This cell line shows characteristics in culture consistent with malignant, hepatic epithelial cells and is apparently EGF dependent. It may provide a model system for the future development of alternative therapies.

The importance of gross total resection of the primary tumor in stage 4 neuroblastoma is controversial. The purpose of this study was to assess the impact of gross total resection of the primary tumor on clinical outcome in patients with stage 4 neuroblastoma diagnosed at more than 1 year of age. The authors retrospectively analyzed 70 newly diagnosed cases treated with one of five regimens of increasing dose-intensity. Outcome variables included survival time from diagnosis, and time to local recurrence or local tumor progression. Patient variables analyzed for impact on survival included age, anatomic location of the primary tumor, radiation treatment of the primary site, complete resection at diagnosis, gross total resection (GTR) at any time in the course of therapy, and treatment protocol dose-intensity. GTR was accomplished in seven patients at the time of diagnosis and in 32 patients after chemotherapy. The likelihood of complete gross resection after chemotherapy increased with greater protocol intensity. The only patient variables that correlated with improved survival were GTR (P = .03) and chemotherapy protocol (P = .01). GTR was also associated with improved local control. Although an independent effect of GTR on survival was not demonstrable because complete resection after chemotherapy correlated strongly with increasing protocol intensity, its association with improved overall survival was striking. These results support a continued role for GTR in high-risk neuroblastoma, along with intensive chemotherapy.

Background: The incidence of Hickman catheter sepsis is 10% to 40%, with resultant catheter loss in one third of infections. Urokinase causes dissolution of colonized intracatheter fibrin thrombi and may improve salvage., Study Aims: To evaluate the efficacy of 12-hour-interval slow-push urokinase infusion in addition to standard antibiotic therapy in the treatment of catheter sepsis in a pediatric oncology population., Methods: A two-arm randomized double-blind trial was undertaken, with catheter salvage rate as the end point. Patients with Hickman catheter sepsis were randomized after culture data confirmed the diagnosis. The study drug was administered by a slow intravenous push and given at 12-hour intervals for a total of four doses. The catheters were aspirated after 1 hour., Results and Conclusions: The trial was stopped after 41 patients were entered into the study; 18 patients received a placebo, and 23 received the urokinase. In the placebo group, six catheters were lost; in the urokinase group, eight were lost. The rate of bacterial clearance was equivalent for both. After administration of the study drug, each group had three episodes of fever and chills; two of these resulted in hypotension (one in each group). The authors conclude that slow-push urokinase infusion during established Hickman catheter sepsis does not result in improved catheter salvage or bacterial clearance. Slow intravenous push infusions in this setting may provoke hemodynamic instability even after initiation of antibiotics.

To identify significant predictors of device-related infections, we performed a prospective, nonrandomized analysis of our experience with vascular access devices over a 2-year period in a pediatric oncology population. Variables analyzed included: (1) age at placement, (2) sex, (3) underlying disease, (4) type of device used (catheter v port), and (5) total white blood cell count at placement. Quantitative microbiologic criteria were used for diagnosis of bacteremia while clinical and microbiologic criteria were used in diagnosis of tunnel/port/site infections. During the study period a total of 351 devices, comprising 78,159 days in situ, were placed and data for univariate and multivariate analysis were available on 271 (77%). The mean age at placement was 7.2 +/- 4.7 years for catheters and 9.5 +/- 4.8 years for implantable devices (P less than or equal to .01). Significant predictors of device-related infections in univariate analysis were type of device (P less than or equal to .0001) and age (P less than or equal to .002). External catheters and age less than or equal to 7 years were associated with increased risk of infection. Underlying disease had a marginal effect on the infection rate (P = .08). In multivariate analysis, device type (P less than or equal to .0001) and age (P less than or equal to .002) continued to affect infections, whereas underlying disease demonstrated only a borderline effect (P = .14). We conclude that device type and age significantly affect the rate of device-related infections. These data support increased use of implantable devices in pediatric oncology patients.

In order to examine surgical factors predictive of fatal outcome in patients presenting with histologically verified rhabdomyosarcoma of the urinary bladder, we performed a retrospective analysis of cases presenting between the years 1970 and 1985 and treated by protocol. Twenty-five patients were identified and data were complete for univariate and multivariate analysis on all. Staging was done according to the criteria of the International Union Against Cancer (TNM). Median age at presentation was 14.7 years and 10 patients were younger than 10 years. Median follow-up was 4.8 years overall and 8.4 years in survivors. Four patients presented with involvement of regional lymph nodes and three with distant metastases. Complete surgical resection, defined as negative microscopic margins, was accomplished by total cystectomy in 14 patients, and partial cystectomy in two. In this group cystectomy was performed prior to chemotherapy and radiation in five and after in 10 (persistent disease). Three salvage cystectomies were performed in patients who recurred after initial complete responses to chemotherapy and radiation therapy. Thirteen patients received a median of 3,000 cGy (range, 1,800 to 5,000 cGy) of external beam pelvic irradiation, and two received brachytherapy. All patients received multiple agent chemotherapy according to either the T2 or T6 protocol. There are 11 disease-free survivors (44%) and 10 of these have been followed for more than 6 years. One patient is alive with disease 6.5 years after diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Recent experience with two cases of rectal duplication, which had been misdiagnosed as hemorrhoids, or fistula-in-ano with resultant delay in diagnosis, prompted us to review our prior experience with 11 of these unusual cases. Age at presentation ranged from newborn to 18 years (mean, 17 months). The most common presenting sign was a perianal or anal fistula, observed in five children. Two children presenting with fistulae had concomitant infection in the duplication. Other presenting signs included obstruction or prolapse caused by the rectal mass in three patients, rectal bleeding in three, and urinary retention in one. Some children presented with more than one finding. No associated spinal or vertebral anomalies were observed. Total excision was performed using a transanal approach in eight patients, postanal (transcoccygeal) in two, and posterior sagittal in one. Postoperative continence was normal in all patients. These cases illustrate that rectal duplications can be confused with other types of anorectal pathology including hemorrhoids, fistula-in-ano, and perirectal abscess. Total excision performed using a posterior sagittal, transanal, or transcoccygeal approach is curative.