ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

PurposeTo review the importance of prognostic factors in developing new protocols for children with rhabdomyosarcoma (RMS).Patients and MethodsFour studies conducted by the Intergroup Rhabdomyosarcoma Study (IRS) Group from 1972 through 1991.ResultsFavorable prognostic factors are: (1) undetectable distant metastases at diagnosis; (2) primary sites in the orbit and nonparameningeal head/neck and genitourinary nonbladder/prostate regions; (3) grossly complete surgical removal of localized tumor at the time of diagnosis; (4) embryonal/botryoid histology; (5) tumor size ≤5 cm; and (6) age younger than 10 years at diagnosis. The IRS-V protocols are risk-based and refine therapy by reducing exposure to cyclophosphamide and radiation therapy (XRT) in patients at low risk while adding new, active agents such as topotecan or irinotecan to the standard therapy of vincristine, actinomycin D, and cyclophosphamide (VAC) plus XRT for patients with unfavorable histology or advanced disease. Collection of biologic specimens from patients with newly diagnosed disease continues to identify other factors that may distinguish patients with favorable features from those who need more intensive therapy. A new protocol that takes into account their previous treatment is needed for patients with recurrent disease. This program (being planned) does not include bone marrow/stem cell reconstitution because this strategy has thus far failed to improve survival rates of patients with metastases at diagnosis.ConclusionBetter understanding of biologic differences and new, active agents are needed to improve outcome of patients with unfavorable features at presentation.

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

ObjectiveTo analyze retrospectively results of reverse transcription polymerase chain reaction (RT-PCR) testing and demographic information in ?patients with known or suspected Ewing sarcoma/primitive neuroectodermal tumor family of tumors referred to the National Cancer Institute and to describe factors influencing the determination of molecular marker status.Patients and MethodsTumor samples from 76 patients from February 1997 to December 1999 were analyzed. In all cases, the diagnosis of this family of tumors was confirmed by histopathologic review.ResultsIn 58 patients, the presence of a translocation associated with this family of tumors was confirmed using RT-PCR. Specifically, there were 45 Ewing sarcoma (EWS)-FLItype 1 translocations, fourEWS–FLItype 2 translocations, fiveEWS–ERGtranslocations, and four less commonEWS–FLIvariants. Of patients with a confirmed translocation, four were confirmed only after nested RT-PCR techniques were used. In five patients who initially underwent needle biopsy, the diagnosis was confirmed only after open biopsy or repeat needle biopsy was undertaken.Samples from 18 patients were translocation-negative. Of these, seven samples were deemed inadequate for RT-PCR testing as a result of inappropriate tissue handling or the presence of necrotic material. Five patients were found to have a different diagnosis after complete histopathologic and molecular characterization. Six samples remained, in which adequate tissue was obtained with no evidence of a characteristic translocation.ConclusionsIn apparently translocation-negative samples, close attention should be given to the possibility of an alternative diagnosis, the potential need for nested RT-PCR, and the possibility of an inadequate sample. Strong consideration should be given to the use of open biopsy as opposed to needle biopsy to avoid the need for repeat biopsies and the potential for inaccurate assessment of molecular marker status.

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID

摘要:

PurposeThis study was designed to estimate the partial and complete response rates (CR and PR) of two novel drug pairs (vincristine and melphalan vs. ifosfamide and etoposide) and to improve overall survival of previously untreated patients with metastatic rhabdomyosarcoma.Patients and MethodsOne hundred twenty-eight patients were randomly assigned to phase II window therapy consisting of vincristine and melphalan (VM-containing regimen) or ifosfamide and etoposide (IE-containing regimen). Brief window therapy (12 wks) was immediately followed-up by vincristine, dactinomycin, and cyclophosphamide (VAC), chemotherapy, surgery, and irradiation, with continuation of either VM or IE in patients with initial response. Major endpoints were initial CR and PR rates after the phase II window phase of therapy, failure-free survival (FFS), and survival.ResultsPatients who received the VM-containing regimen experienced significantly more anemia, neutropenia, thrombocytopenia, and had more cyclophosphamide dose reductions. The initial PR and CR rates were not significantly different for patients treated with either regimen (VM, 74%; IE, 79%;P= 0.428). However, FFS and overall survival (OS) at 3 years were significantly better with the IE-containing regimen (FFS: 33% vs. 19%;P= 0.043; OS: 55% vs. 27%;P= 0.012).ConclusionsAlthough the VM-containing regimen produced a high response rate, inclusion of melphalan appeared to limit the cyclophosphamide dose that could be administered, and ultimately, this regimen was associated with a significantly worse outcome than was the IE-containing regimen. Also, the IE-containing regimen was associated with a gratifyingly high survival rate at 3 years (55%), which is significantly higher than has been observed on any previous Intergroup Rhabdomyosarcoma Study Group regimen for similar patients. We believe that this promising outcome indicates that this drug pair merits further randomized testing in metastatic rhabdomyosarcoma.

ISSN:1077-4114    

出版商:OVID    

年代:2001

数据来源: OVID