Intraureteral Wilms tumor.
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Diagnostic Imaging↗
Biomedical subjects
S J Shochat
Publications and source records attributed to S J Shochat.
3D gadolinium-enhanced MRA: evaluation of hepatic vasculature in children with hepatoblastoma.
We used contrast-enhanced three-dimensional magnetic resonance angiography (3D MRA) modified for pediatric use to evaluate the hepatic vasculature prior to partial hepatectomy in five consecutive children with hepatoblastoma. Modifications included non-breath-hold technique in four of the five children who were sedated. The single breath-hold technique was performed in only one awake child. Scan delay times were based on contrast infusion time rather than total infusion time. The hepatic artery, portal vein, and inferior vena cava were identified in all patients. MRA findings were confirmed by conventional angiography in one patient and by surgery in all. Contrast-enhanced 3D MRA is a useful and rapid technique prior to partial hepatectomy in patients with hepatoblastoma.
Child↗
Nonrhabdomyosarcoma soft tissue sarcomas in children: is age at diagnosis an important variable?
PURPOSE: The associations between age at diagnosis, tumor characteristics, and outcome in children diagnosed with nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) were studied. METHODS: Retrospective review was conducted of 192 children from 1962 through 1996. Patients were divided into groups: birth to 1 year (n = 13), 1 to 5 years (n = 26), 5 to 10 years (n = 49), 10 to 15 years (n = 55), and older than 15 years (n = 49) of age at diagnosis. Characteristics including IRS group, histological grade and pattern, tumor size, and invasiveness were investigated. Survival rate was estimated by age group. The median follow-up was 8.8 years (range, 2 to 28 years). RESULTS: There were 81 group I patients, 40 group II, 41 group III, and 30 group IV. A significant difference of IRS groups among the age groups was seen (P = .034). There were no IRS group IV patients less than 1 year of age; 50% of IRS group IV patients were older than 15 years. A significant difference in the distribution of histological grade among the age groups (P = .032) was seen. Ten of 13 (77%) children less than 1 year of age had low-grade tumors, whereas 42%, 45%, 60%, and 37% of patients aged 1 to 5, 5 to 10, 10 to 15, and older than 15 years, respectively, had low-grade tumors. Patients older than 15 years had the highest incidence of invasive tumors (59%). Histological pattern also varied with age. The most prevalent histology in the less-than-1-year age group was infantile fibrosarcoma. No predominant histology was seen in the 1- to 5-year age group. Malignant fibrous histiocytoma was the most frequent histological subtype in children between 5 and 10 years of age. In the 10- to 15-year age group and children older than 15 years the malignant peripheral nerve sheath tumor and synovial sarcoma were the most prevalent subtypes. Without adjusting for any other factors, age group was prognostic of survival (P = .007). Patients less than 1 year at diagnosis had the best outcome, with a 5-year survival rate of 92% +/- 9%. Five-year estimates were lowest for patients older than 15 years (49% +/- 7%). CONCLUSIONS: Significant differences in IRS group, histological grade, and histological subtype were observed in different age groups. Infants with NRSTS were more likely to have low grade, less invasive, and lower stage tumors. These characteristics may account for their improved prognosis.
Adolescent↗
Enhanced neuroblastoma transduction for an improved antitumor vaccine.
BACKGROUND: A recent clinical trial of an antineuroblastoma vaccine used adenovirus serotype 5 (Ad5) vectors to transduce autologous tumor cells with the gene encoding IL-2. A method to improve transduction efficiency was sought to enable the use of lower viral titers, especially when in situ adenoviral-mediated tumor cell transduction is considered. MATERIALS AND METHODS: A chimeric adenoviral delivery vector was utilized in which the fiber head from adenovirus serotype 3 was incorporated into the backbone of Ad5. Since the fiber head protein is responsible for viral attachment to target cells, a different spectrum and range of infectivity might result. Both the chimeric (Av9LacZ4) and Ad5 (Av1LacZ4) vectors were constructed to carry a beta-galactosidase transgene. The relative transduction efficiency of these two vectors was then evaluated in five tumor-derived short-term neuroblastoma cultures and four established neuroblastoma cell lines. Enzyme activity was assessed using three different methods: in situ staining, flow cytometric analysis, and a quantitative assay. RESULTS: A significant improvement in transduction efficiency of the short-term neuroblastoma cultures with the new chimeric adenovector was demonstrated. A similar improvement in transduction efficiency was not observed in the established cell lines, suggesting that the cell surface receptor for the Ad 3 serotype had been lost in vitro. Increased transduction of tumor cells with N-myc amplification was also observed. CONCLUSIONS: The newly constructed chimeric adenoviral vector transduces short-term neuroblastoma cultures more efficiently than the standard Ad5 vector. This vector will permit the use of lower viral titers and may be useful in other adenoviral-based gene-therapy protocols. Increased transgene expression in N-myc-amplified cells offers possible selectivity for in situ gene delivery.
Adenoviruses, Human↗
Neuroblastoma regression and immunity induced by transgenic expression of interleukin-12.
PURPOSE: Interleukin-12 (IL-12) is a cytokine with potent antitumor effects. The authors sought to assess its capacity to increase tumor immunogenicity when expressed by tumor cells in a murine model of neuroblastoma. METHODS: Syngeneic A/J mice were inoculated subcutaneously with 2 x 10(6) cells from a murine neuroblastoma-derived cell line (neuro-2a). In situ transduction of the neuroblastoma cells was achieved by intratumoral injection of an adenoviral vector encoding both subunits of the murine IL-12 heterodimer. Growth of the IL-12 gene-modified tumor cells was compared with untreated neuro-2a cells. Tumor immunity was assessed by rechallenging mice that had rejected their tumor with unmodified neuroblastoma cells. The contribution of cytotoxic T lymphocytes (CTLs) was evaluated through cytotoxicity assays. RESULTS: Eighteen (72%) of 25 tumor-bearing mice treated with the mlL-12 adenoviral vector exhibited tumor regression, with 12 mice (48%) completely rejecting their tumors over 2 to 3 weeks. None of the mice that had rejected their tumor and were rechallenged with unmodified neuro-2a cells subsequently developed new tumors. Pooled splenocytes from mice rejecting their tumors showed significant tumor killing (>20% cytolysis) in vitro in 51Cr release assays. CONCLUSIONS: Adenoviral-mediated IL-12 expression by tumor cells in a murine neuroblastoma model produced a significant antitumor response. Most treated tumors demonstrated at least transient regression, whereas many completely regressed. Cured mice exhibited protective immunity and CTL activity against the tumor. These data confirm the immunomodulatory efficacy of IL-12 as part of a vaccine-based antineuroblastoma strategy.
Adenoviridae↗
Comparison between single-dose and divided-dose administration of dactinomycin and doxorubicin for patients with Wilms' tumor: a report from the National Wilms' Tumor Study Group.
PURPOSE: The National Wilms' Tumor Study (NWTS)-4 was designed to evaluate the efficacy, toxicity, and cost of administration of different regimens for the treatment of Wilms' tumor (WT). PATIENTS AND METHODS: Between August 6, 1986 and September 1, 1994, 1,687 previously untreated children less than 16 years of age with stages I to II/favorable histology (FH) or stage I/anaplastic histology WT (low-risk [LR] group) or stages III to IV/FH WT or stages I to IV/clear cell sarcoma of the kidney (high-risk [HR] group) were randomized to treatment that included vincristine and either divided-dose (standard [STD]) courses (5 days) or single-dose (pulse-intensive [PI]) treatment with dactinomycin. HR patients also received either STD courses (3 days) or PI treatment with doxorubicin. RESULTS: The 2-year relapse-free survival (RFS) rates for LR patients were 91.3% for 544 randomized to treatment with PI and 91.4% for 556 randomized to treatment with STD chemotherapy (P = .988). The 2-year RFS rates for HR patients were 87.3% for 299 randomized to treatment with PI and 90.0% for 288 randomized to treatment with STD chemotherapy (P = .865). CONCLUSION: We conclude that patients treated with PI combination chemotherapy for LR or HR WT or clear cell sarcoma of the kidney have equivalent 2-year RFS to those treated with STD regimens. PI drug administration is recommended as the new standard based on demonstrated efficacy, greater administered dose-intensity, less severe hematologic toxicity, and the requirement for fewer physician and hospital encounters.
Adolescent↗
Treatment of metastatic osteosarcoma at diagnosis: a Pediatric Oncology Group Study.
PURPOSE: To estimate the duration of survival (S) of patients with metastatic osteosarcoma (MOS) at diagnosis treated with a multiagent, ifosfamide-containing chemotherapeutic and surgical regimen and to evaluate the toxicity of this regimen. PATIENTS AND METHODS: Thirty patients aged younger than 30 years received two courses of ifosfamide followed by surgery on the primary tumor and metastatic sites. Patients then received a postsurgical multiagent chemotherapeutic regimen that consisted of high-dose methotrexate (HDMTX), ifosfamide, doxorubicin, and cisplatin. RESULTS: The 5-year event-free survival (EFS) rate was 46.7% (95% confidence interval [CI]; 28.5 to 64.9) and 5-year S rate was 53.3% (95% CI; 35.1 to 71.5). Three patients with bone metastases and one patient with lymph node metastases died. Twenty-six patients presented with pulmonary metastatic nodules only. Eight of these patients had at least eight nodules at diagnosis and had an estimated 5-year EFS rate of 25.0% compared with 66.7% for the 18 patients with less than eight nodules (P=.06). Fourteen patients presented with bilateral lung metastases and had a 5-year EFS rate of 35.7% compared with the 12 patients who presented with unilateral involvement and had a 5-year EFS rate of 75.0% (P=.03). The hematopoietic toxicity experienced by the patients during the entire regimen was relatively mild. Seven patients had renal toxicity characterized by hypophosphatemia and/or hypokalemia. CONCLUSION: This ifosfamide-containing regimen is tolerable and effective in the treatment of patients with osteosarcoma (OS) who present with lung metastases. However, better regimens are required for this group of patients.
Adult↗
Endodermal sinus tumor in children.
Malignant germ cell tumors account for about 3% of neoplasms in children, and endodermal sinus tumor (EST) is the most common histological subtype. The authors reviewed 22 years' experience (at their institution) in the management of 37 patients with this tumor. Fifteen of them (41%) had a sacrococcygeal primary, 10 had a testicular tumor (27%), 6 had an ovarian tumor (16%), 3 had a vaginal tumor (8%) and 3 had tumors at other sites (8%). Seven (19%) patients presented with metastatic disease, primarily pulmonary. The serum alpha-fetoprotein (AFP) level was elevated in all cases tested. The initial chemotherapy regimen included vincristine, actinomycin, cyclophosphamide (VAC), and Adriamycin (6 patients), but since 1985 the regimen has been changed to include cisplatin or Carboplatin, etoposide, and bleomycin (21 patients). Eight patients with testicular tumors initially were treated with surgical excision alone. Computed tomography results were a poor predictor of recurrence, but AFP surveillance was extremely sensitive. No second-look operation detected residual tumor in the absence of AFP elevation. Initial relapse for all patients tended to occur early (within 2 years), locally, and often with pulmonary metastases. Although historically the prognosis for patients with EST has been poor, the overall 2-year survival rate in this series was 70%. The best prognosis was among the children who had a testicular primary tumor (survival rate, 100%). The 2-year survival rate for patients with ovarian tumors was 67%; for those with sacrococcygeal primaries it was 60%. These results suggest that the prognosis for children with EST has improved significantly over the past decade. Contributing factors include therapy based on cisplatin, etoposide, and bleomycin, and relapse surveillance with serial AFP determinations. Second-look procedures should be reserved for patients who have an increasing level of serum AFP, suspicious computed tomography findings, and no obvious evidence of metastatic disease.
Adolescent↗
Severe head injury caused by airbag deployment.
Passenger-side airbags are present in over 21 million automobiles and will be required on all passenger vehicles by the model year 1999. Although airbags are effective supplemental restraint systems and have saved over 1,500 lives, airbag-related injuries have been reported. The National Highway Traffic Safety Administration recently reported 15 child deaths caused by airbag deployment. All of these children were believed to be improperly restrained in the front passenger position of a car equipped with a passenger-side airbag. This case represents the first serious injury caused by airbag deployment in a child that was properly positioned in an approved child restraint.
Accidents, Traffic↗
Treatment of osteosarcoma with ifosfamide: comparison of response in pediatric patients with recurrent disease versus patients previously untreated: a Pediatric Oncology Group study.
This study was designed to test if the activity of a phase II agent, ifosfamide, would have been underestimated if it was tested exclusively in a population of children and young adults with recurrent osteosarcoma. The response rate to ifosfamide was compared in patients younger than 30 years of age with previously untreated osteosarcoma with metastases at diagnosis and/or unresectable primary tumors (stratum 1) with that of patients with recurrent osteosarcoma following adjuvant chemotherapy who were not previously exposed to ifosfamide (stratum 2). Evaluation of response was conducted 3 weeks after two courses of ifosfamide (2400 mg/m2 x 5 days) were administered 3 weeks apart. Nine of 33 (27%) evaluable patients in stratum 1 responded (1 complete and 8 partial responses) to ifosfamide. Among 30 evaluable patients in stratum 2, only 3 (10%) responded (1 complete and 2 partial responses; P = .04) Both groups of patients received equal doses of ifosfamide and experienced comparable toxicities. Results from this study suggest that the activity of new agents will be underestimated if tested in a population of heavily pretreated patients with recurrent disease. When possible, new chemotherapeutic agents should be tested in patients with a poor prognosis who have not been exposed to chemotherapy.
Adolescent↗
Lobar lung transplantation as a treatment for congenital diaphragmatic hernia.
The mortality rate for infants severely affected with congenital diaphragmatic hernia (CDH) remains high despite significant advances in surgical and neonatal intensive care including delayed repair and extracorporeal membrane oxygenation (ECMO). Because of the increasingly successful experience with single-lung transplantation in adults; this approach has been suggested as a potential treatment for CDH infants with unsalvageable pulmonary hypoplasia. The authors report on a newborn female infant who was the product of a pregnancy complicated by polyhydramnios. At birth, she was found to have a right-sided CDH and initially was treated with preoperative ECMO, followed by delayed surgical repair. Despite the CDH repair and apparent resolution of pulmonary hypertension, the infant's condition deteriorated gradually after decannulation, and escalating ventilator settings were required as well as neuromuscular paralysis and pressor support because of progressive hypoxemia and hypercarbia. A lung transplant was performed 8 days after decannulation, using the right lung obtained from a 6-week-old donor. The right middle lobe was excised because of the size discrepancy between the donor and recipient. After transplantation, the patient was found to have duodenal stenosis and gastroesophageal reflux, which required duodenoduodenostomy and fundoplication. The patient was discharged from the hospital 90 days posttransplantation, at 3 1/2 months of age. Currently she is 24 months old and doing well except for poor growth. This case shows the feasibility of single-lung transplantation for infants with CDH, and the potential use of ECMO as a temporary bridge to transplantation. Lobar lung transplantation allowed for less stringent size constraints for the donor lung.
Extracorporeal Membrane Oxygenation↗
Early hernia repair in the premature infant: long-term follow-up.
The incidence of inguinal hernia and incarceration is high among premature infants. Optimal timing, anesthetic technique, and long-term results of hernia repair in hospitalized premature infants remain undefined. The authors reviewed the records of 52 consecutively treated premature infants who underwent bilateral inguinal herniorrhaphy under general anesthesia before discharge from the intensive care nursery. There were no significant differences in gestational age, birth weight, age and weight at time of surgery, or presence of preoperative apnea or bradycardia in between infants extubated within 24 hours and those intubated for more than 24 hours. Twenty-four infants (46%) were available for follow-up of 24 months or more (mean follow-up period, 57 months). One recurrence was identified, representing 4% of the long-term follow-up group and 2% of the initial population. Two patients had asymmetric testicular volumes suggestive of unilateral atrophy. The short- and long-term results suggest that repair under general anesthesia can be safely performed before discharge from the intensive care nursery.
Female↗
Peritoneal drainage as primary management of perforated NEC in the very low birth weight infant.
Advances in perinatal and neonatal care in the past decade have produced a change in the population of infants with perforated necrotizing enterocolitis (NEC) treated at our institution: the majority are now of very low birth weight (VLBW, < 1,000 g). Peritoneal drainage has been reported as an initial resuscitative procedure for unstable infants who have complicated NEC. Initial success with peritoneal drainage prompted us to adopt an aggressive approach to its use in this patient population. Since 1987, peritoneal drainage has been the primary treatment for most infants weighing less than 1,500 g who have perforation, and for unstable infants weighing more than 1,500 g. Perforation was documented by pneumoperitoneum or aspiration of meconium by paracentesis. Intestinal resection was performed in most infants weighing more than 1,500 g and in those for whom drainage was ineffective. Twenty-nine infants with low or VLBW (mean gestational age, 27 weeks; mean birth weight, 994 g) were treated with one or two drains in the right lower quadrant. Broad spectrum antibiotics were continued until all drains were removed, usually within 10 to 14 days. Nasogastric suction was continued until patency of the gastrointestinal (GI) tract was confirmed by a nonionic upper GI series. Six (21%) infants died, although one of the deaths occurred 5 months after drainage; the patient had chronic lung disease and an intact GI tract. Seventeen of the 23 (74%) survivors required no further operative procedure, and 6 (26%) required laparotomy and resection because drainage had been ineffective. Peritoneal drainage provided definitive treatment in 18 of 29 (62%) infants in this series.(ABSTRACT TRUNCATED AT 250 WORDS)
Drainage↗
Preoperative therapy for intracaval and atrial extension of Wilms tumor.
BACKGROUND: Primary surgical management of Wilms tumor with intravascular tumor extension is associated with significant morbidity. Retrospective analysis of a group of Wilms tumor patients with intravascular tumor extension treated with preoperative therapy was performed to evaluate the feasibility of this approach. METHODS: Thirty children with caval (15 patients) or atrial (15 patients) extension of Wilms tumor who received preoperative therapy were reviewed. Patients were classified according to the stage of the disease as follows: (1) Stage III, 7 patients; (2) Stage IV, 18 patients; and (3) Stage V, 5 patients. The histologic type was favorable in 20 patients, anaplastic Wilms tumor in 7, and unknown in 3. All of the children received preoperative chemotherapy, ranging from 1-16 weeks with an average of 7.2 weeks. RESULTS: One child died of progressive disease and another from toxicity before surgery. In 23 patients, a decrease in the size of the intravascular extension was noted preoperatively, without tumor embolization in any case. There was complete resolution of tumor thrombus in seven patients. Two-year survival for the entire group is 70%, with a median follow-up period of 21 months. CONCLUSIONS: The use of preoperative therapy is appropriate in selected patients with extensive intravascular tumor involvement. Surgical management was facilitated in the majority of cases due to shrinkage of the primary tumor and reduction in the size of the tumor thrombus.
Antineoplastic Combined Chemotherapy Protocols↗
Thoracic neuroblastoma: a Pediatric Oncology Group study.
Ninety-six patients with thoracic neuroblastoma were studied in a prospective fashion. Median age at presentation was 0.9 years. Forty-eight percent of the patients presented with stage A disease, 20% stage B, 13% stage C, 17% stage D, and 2% stage DS. Seventy-five patients have been followed for greater than 4 years. A posterior mediastinal mass was diagnosed on incidental chest roentgenograms performed for nontumor-related symptoms in 49% of the cases. Sixteen percent of the cases presented with neurological symptoms and 14% of the patients presented with acute respiratory distress. Urinary catecholamines were elevated in 76% of the cases. Complete surgical resection was carried out in 47% of the cases, while incomplete resection or biopsy was performed in 45%. No operation was performed in 3 patients. Minor surgical complications occurred in 20% of the patients, and 3% of the patients had significant perioperative complications. One patient died as a complication of therapy. Overall actuarial survival was 88% at 4 years. This study confirms the favorable outcome in children with mediastinal neuroblastoma. The basic biology of thoracic neuroblastomas seems to differ from that of other sites in that the majority of patients present at a younger age with localized disease or regional lymph node metastases, and have an improved survival even after correcting for age and stage. While complete excision is recommended, if possible, radical surgical procedures are not indicated since an excellent prognosis is associated with combined modality therapy.
Age Factors↗
Wilms' tumor: diagnosis and treatment in the 1990s.
The present therapy of Wilms' tumor is a classic example of the modern multidisciplinary treatment of childhood malignancies, and the organization of the National Wilms' Tumor Studies (NWTS) has served as a prototype for other collaborative studies of childhood tumors. More than 80% of all children with Wilms' tumor can now be expected to have a long term relapse free survival. The NWTS is in large part responsible for this excellent outlook and continues its emphasis on decreasing the morbidity of therapy without sacrificing relapse free survival by tailoring therapy based on histology and accurate surgical staging. The pathological classification of Wilms' and its effect on therapy and prognosis is now being used as a guide in the study of other childhood malignancies. The surgeon continues to play a key role in the multimodality management of the child with a Wilms' tumor since subsequent therapy will be determined by the extent of the tumor at the time of operation. The identification of histological type, the presence or absence of residual tumor within the abdomen following resection, and the status of lymph node metastases are of utmost importance. Advances in cytogenetics and refinement of histopathologic staging will allow the pediatric surgical oncologist, pediatric oncologist, and pediatric radiation therapist to continue to modify therapy for specific patient groups in the future.
Adolescent↗
Incidental appendectomy during nephrectomy for Wilms' tumor.
Incidental appendectomy is frequently performed during nephrectomy for Wilms' tumor. The rationale for this approach is that the onset of appendicitis during a period of neutropenia after chemotherapy may cause increased morbidity. Additionally, children receiving chemotherapy or radiation therapy, or both, may experience abdominal pain, producing a diagnostic dilemma if the appendix is still present. We reviewed the charts of 1,910 children enrolled in the Third National Wilms' Tumor Study who underwent primary nephrectomy. Four hundred and fifty-five patients (23.8 percent) had appendectomy at the time of nephrectomy. An inversion appendectomy was performed upon 188 patients, and the appendix was excised in 267. Infectious complications and postoperative intestinal obstructions occurred with equal frequency in children who did or did not undergo appendectomy. The median follow-up period for the entire group of patients was 5.1 years. Only three of the 1,455 children (0.2 percent) who did not have an incidental appendectomy had appendicitis at two, seven and ten months after nephrectomy. None of the three children experienced any perioperative morbidity after appendectomy. These data suggest that incidental removal of the appendix is not essential for children undergoing removal of a Wilms' tumor.
Appendectomy↗
Superior mesenteric artery injury during nephrectomy for Wilms' tumor.
Iatrogenic injury to the aorta or its major branches during nephrectomy for Wilms' tumor in children is rarely reported but may be more common than is currently acknowledged. We identified four patients with ligation of the superior mesenteric artery (SMA) that occurred during nephrectomy for nephroblastoma and another child in whom SMA thrombosis developed postoperatively. All of the tumors were on the left side. Interruption of the SMA was recognized intraoperatively in all four children, and primary repair was done. In three patients, appearance of the bowel remained normal before repair of the injury. Three of the arteries were repaired by primary reanastomosis, and one was joined with an interpositioned hypogastric artery graft. None of these patients had gastrointestinal complications postoperatively. In the fifth patient, SMA thrombosis developed after repair of an aortic tear during nephrectomy. This patient required subsequent small bowel resection for bowel infarction and died in the perioperative period. Every surgeon treating children with Wilms' tumor should be aware of the possibly distorted vascular anatomy and take precautions to avoid such a significant injury. Attempts at early ligation of the vessels may not be justified until the renal vasculature is clearly identified.
Child, Preschool↗