ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Childhood endodermal sinus tumors (CEST) are a distinct category of germ cell tumors that involve the testis and extragonadal sites of young children. Recurrent deletions of 1p and 6q have been reported by classic cytogenetic analysis of a small number of cases. Comparative genomic hybridization, a technique that screens the entire genome for genetic abnormalities, is applied to additionally define the genetic changes present in CESTs. Sixteen frozen CESTs (10 testicular, 6 extragonadal) obtained from Pediatric Oncology Group-affiliated institutions or from the Cooperative Human Tissue Network were analyzed. The most common changes were gain of 20q (10 tumors), 1q (6 tumors), 11q and 22 (4 tumors each), and loss of 6q (8 tumors with common deleted region of 6q24-qter), 16q (4 tumors), and 1p (4 tumors). Localized regions of gain were identified at 8q24 (2 tumors both showingc-mycamplification by fluorescence in situ hybridization). Gain of 12p, characteristic of adolescent germ cell tumor, was present in one testicular tumor. Comparative genomic hybridization was useful in defining genetic differences between adult and childhood tumors, in determining the common regions deleted on chromosome 6, and in identifying other involved loci to be correlated with clinical parameters in future studies.

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

The presence of well-differentiated rhabdomyoblasts at the end of therapy for rhabdomyosarcoma has been noted. This study was undertaken to investigate the therapeutic implications of the presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma. Six patients with pelvic rhabdomyosarcoma (bladder-prostate, 4; vulvovaginal, 2) with disease diagnosed between the years 1974 and 1992 were sequentially investigated by cystoscopic or vaginoscopic examination and biopsy during and after completing therapy. All six patients received treatment according to prevailing therapeutic protocols. Biopsy material from all six patients at the end of therapy documented the presence of well-differentiated rhabdomyoblasts. Repeated biopsies demonstrated the presence of rhabdomyoblasts; however, they appeared to decrease in number with time. Mitotic activity was not observed in the biopsy materials obtained. All six patients are alive without evidence of disease from 37 to 233 months after therapy ended. The presence of well-differentiated rhabdomyoblasts at the end of therapy for pelvic rhabdomyosarcoma is a common finding. The biologic nature of these well-differentiated rhabdomyoblasts is not completely known, but they do not appear to connote the persistent presence of malignant disease and are not an indication for the continuation of therapy.

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Purpose:The aim of this study was to assess renal tubular toxicity (RTT) of ifosfamide-containing regimens (ICR) in patients with newly diagnosed sarcomas at St. Jude Children's Research Hospital.Methods:The authors reviewed the records of 199 patients receiving ICR at St. Jude between June 1986 and December 31, 1994 for evidence of RTT. Their median age was 13.3 years (range 1.2-24.8); 150 patients were white and 112 were male patients. Diagnoses included osteosarcoma (n = 82), Ewing sarcoma (n = 82), rhabdomyosarcoma (n = 28), and a group of other tumors (n = 7).Results:The authors estimated the proportion of patients with severe RTT during the first five cycles of ICR and within 1 year after therapy for three groups of patients receiving ifosfamide (IFOS, n = 110), ifosfamide/cisplatin (IFOS/CDDP, n = 51), and ifosfamide/carboplatin (IFOS/CARBO, n = 38). The IFOS/CDDP patients received three cycles of IFOS before receiving CDDP and received only 200 mg/m2by cycle 5, whereas the IFOS/CARBO patients received both agents simultaneously. The authors compared the probability of severe RTT among treatment groups using a generalized linear model for the first five cycles of ICR, as well as the probability of severe RTT within 1 year after therapy among treatment groups for patients receiving all prescribed IFOS using an exact chi-square test with pairwise comparisons when the three-wayPvalue was less than 0.10. The proportion of patients with severe RTT during the first three cycles of ICR was significantly greater in the IFOS/CARBO group than in the other two. Although the proportion of patients with severe RTT in the IFOS/CDDP group increased during cycles 4/5, the proportion of patients with severe RTT remained significantly greater in the IFOS/CARBO group. Within 1 year after therapy, the proportion of patients with severe RTT differed among the three groups, and pairwise comparisons revealed a significant difference between the IFOS and the IFOS/CDDP group. Severe RTT developed in four IFOS/CDDP patients more than 1 year after therapy, suggesting a long-term effect of CDDP on tubular function.Conclusions:Chemotherapy regimens including IFOS/CARBO produce severe acute RTT more frequently than regimens including IFOS or IFOS/CDDP. Patients receiving IFOS/CDDP appear at risk for delayed RTT. Long-term follow-up of these patients is essential to assess whether the number of patients receiving IFOS/CDDP with severe RTT continues to increase over time and to evaluate the long-term significance of these abnormalities.

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Purpose:The activity of etoposide (VP-16) has been demonstrated to be schedule-dependent. Several studies have been conducted on the efficacy and safety of different schedules of VP-16 both in adults and in children, but the optimal schedule has not been determined.Methods:In the current study, the feasibility and effectiveness of prolonged oral VP-16 in children with high-risk malignancies were evaluated. Between April 1995 and February 1999, 15 pretreated patients with high-risk tumors received oral VP-16. The schedule of therapy was oral VP-16 50 mg/m2/day for 10 consecutive days and 1-week interval between cycles. Therapy was stopped after 1 year of treatment or at time of progressive disease or possible surgery. All patients had received parenteral VP-16 in their earlier chemotherapy.Results:Twelve patients were evaluable for tumor response. After 2 to 4 months of treatment, one patient had complete remission (CR), two had partial response (PR), two had minor response (MR), two had mixed response (MxR), three had stable disease (SD), and two had progressive disease (PD). A useful palliative effect was noted in patients with stable disease. In three patients, oral VP-16 was administered for maintenance therapy. After an average follow-up of 27.5 months (range, 7-41 months), five patients are alive without disease (in three, total surgery was performed after VP-16 therapy) and three patients are alive with disease. Six patients died of progressive disease, and one died of promyelocytic leukemia. One patient had Grade 3-4 thrombocytopenia; in the remaining patients, no acute toxicity was observed during treatment.Conclusions:This schedule of oral VP-16 produced CRs, PRs, and MRs in medulloblastoma, neuroblastoma, teratocarcinoma, and ependymoma. Stable disease was observed in three patients, one with an Askin tumor, one with medulloblastoma, and one with hepatoblastoma. Given the possible leukemogenic risk, this schedule should be used as a palliative form of therapy or in patients with poor prognosis.

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

Purpose:To determine the effect of standard antiemetic treatment in children receiving various combination chemotherapy regimens.Methods:A validated nausea/vomiting survey was administered to 78 patients receiving 13 different emetogenic chemotherapy regimens. Patients received antiemetic prophylaxis with ondansetron (0.3 mg/kg/day) alone for moderately emetogenic chemotherapy regimens or ondansetron (0.45 mg/kg/day) and methylprednisolone (4 mg/kg/day) for severely emetogenic chemotherapy regimens. A total of 324 different courses of chemotherapy were surveyed. Nausea and vomiting severity, duration, number of emetic episodes, appetite, daily activity interference, and rates of both complete and good antiemetic protection were determined for each chemotherapy protocol. Differences between genders and ages were analyzed.Results:Chemotherapy combinations containing platinum compounds were found to be highly emetogenic and nauseating despite antiemetic therapy with ondansetron plus a corticosteroid. In addition, complete antiemetic protection for the combination of vincristine, cyclophosphamide, and dactinomycin was poor. For most of the severely emetogenic chemotherapy protocols, patients experienced good protection from nausea and vomiting less than 60% of the time, despite the use of ondansetron plus methylprednisolone. Significant differences were found in rates of residual nausea and vomiting and failure of antiemetic protection among the severely emetogenic chemotherapy protocols despite identical antiemetic therapy. Good protection rates were higher for moderately emetogenic chemotherapy treated with ondansetron alone.Conclusions:The currently recommended prophylactic therapy for pediatric patients receiving severely emetogenic chemotherapy fails to provide protection for many patients receiving commonly administered chemotherapy regimens and for most pediatric patients receiving platinum-containing chemotherapy combinations. New and refined antiemetic strategies are needed to improve efficacy in the pediatric population.

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID

摘要:

An extra Y chromosome(s) is occasionally found in patients with various hematologic neoplasias; however, an association with hereditary blood diseases is unknown. In a child with β-thalassemia who received a transplant with sex-mismatched umbilical cord blood and neonatal blood, fluorescent in situ hybridization (FISH) with probes to X and Y chromosomes revealed an extra Y signal in 19.3% of the hepatocytes and in 38.9% of the pretransplant peripheral blood cells, yielding YY/Y ratios of 0.24 and 0.64, respectively. In granulocyte colony-stimulating factor-mobilized autologous peripheral blood stem cells, the FISH ratio of interphase XYY cells to XY cells was 1.64, and there were 3.4 times more G-banded XYY metaphases than normal metaphases. Both FISH and polymerase chain reaction persistently demonstrated posttransplant mixed chimerism. There was a greater proportion of residual autologous XYY cells than XY cells with a mean posttransplant YY/Y ratio of 1.56 (n = 13) (1 SD = 0.33; range, 1.10 to 2.17). In vitro clonogenic assays yielded a normal number of colony-forming units but no growth in cultures without growth factor supplement. This study suggests that hematopoietic stem cells with an extra Y chromosome may upregulate cell growth in response to cytokine stimulation. A posttransplant preponderance of XYY cells might be attributable to an extra Y chromosome in hematopoietic stem cells withstanding the myeloablative conditioning regimen.

ISSN:1077-4114    

出版商:OVID    

年代:2000

数据来源: OVID