摘要:

AbstractThe association between aniridia and Wilms' tumor (WT) is well known. The availability of data on a large number of WT patients entered on the first and second National Wilms' Tumor Studies provided the opportunity to review which examinations had been most effective in detecting the presence of WT in patients being followed with aniridia. The records of 1287 patients were available and 13 or 1% had known aniridia. The details of the prediagnostic studies were available in 9/13 patients. Those nine patients had been examined regularly by IVP at intervals of 3–12 months prior to the discovery of the WT, but in only one was the unsuspected WT detected by these means. In the remaining eight patients, the tumor was discovered by palpation in six and suspected because of hematuria in two. It is concluded that repeated IVP's are not a satisfactory method to diagnose a WT at an early stage and newer radiodiagnostic studies, such as ultrasonography, will probably prove more effectiv

ISSN:0098-1532    

DOI:10.1002/mpo.2950110202

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractTwenty‐two patients with newly diagnosed acute myelocytic leukemia (AML) and white cell counts (WBC) greater than 100,000/μl received one, two, or three leukaphereses prior to induction chemotherapy with cytosine arabinoside (Ara‐C) and daunorubicin (DNR). Fifteen of 22 (68%) achieved a complete remission. Greater than a 30% decrease in initial WBC was found to be an important predictor of response. Fifteen of 17 patients (88%) with greater than 30% reduction of initial WBC achieved remission, while none of 5 patients with less than a 30% reduction responded (P = 0.

ISSN:0098-1532    

DOI:10.1002/mpo.2950110203

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractSeventy‐four children ranging in age from 6 months to 17.5 years with acute lymphoblastic leukemia newly diagnosed between 1976 and 1979 were entered on a study incorporating intermittent chemotherapy with or without the addition of bacillus Calmette‐Guérin (BCG). The chemotherapy program consisted of induction with vincristine, clexamethasone, and intrathecal methotrexate, intensification with adriamycin and asparaginase, central nervous system treatment with cranial irradiation and intrathecal methotrexate, and continuation treatment with 5‐day courses of combination chemotherapy administered every three weeks. The first phase of continuation therapy incorporated vincristine, adriamycin, 6‐mercaptopurine, and dexamethasone. In the second phase, oral methotrexate was substituted for the adriamycin in non‐T‐cell patients; in T‐cell patients, cytosine arabinoside or cyclophosphamide and methotrexate in alternating cycles were substituted for the adriamycin and asparaginase was added. Total duration of therapy was approximately 2.5 years. Connaught BCG was administered by Heaf gun on days 8 and 15 of each 3‐week cycle for the first 8 months of treatment in approximately one‐third of the patients. Actuarial disease‐free survival with a median follow‐up of 59 months shows no difference in outcome between the BCG and non‐BCG poor‐risk patients. However, there is an improvement in disease‐free survival of BCG‐treated good‐ and average‐risk girls (P = 0.04). While patients were actively receiving BCG there was also a trend toward the development of fewer significant infections than when patients were not receiving BCG (P = 0.85). Toxicities from BCG administration included satellite rashes, local tenderness, lymphadenopathy, secondary infection, and residual scars. Overall disease‐free survival by actuarial analysis is 60% at 6 years; for patients with disease‐tree survival of girls with good‐ and average‐risk prognostic features and also may have decreased the susceptibility to infection while it was being administered. However, the benefit does not appear sufficient to warrant its routine use, especi

ISSN:0098-1532    

DOI:10.1002/mpo.2950110204

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractData from four pediatric hospitals concerning 20 children treated for renal cell carcinoma (RCC) from 1964‐1978 were reviewed. Median age of the patients (pts) was 11.8 years (range, 14 months‐19 years). Twelve were male and eight female; 17 were white and three black. Most patients presented with pain and hematuria with or without a palpable mass. An intrarenal tumor was detected at IV urography (17 pts), arteriography (2 pts), or at surgery (1 pt). Treatment consisted of nephrectomy in 15 pts, renal biopsy (4 pts), or no surgery (1 pt), followed by chemotherapy (5 pts), radiation therapy (1 pt), or both (7 pts). Ten pts died of distant metastases at a median of one year (range, 0.2 to two years) after diagnosis. The other 10 pts (50%) survive free of relapse at a median of 4 years (range, two to ten years) from diagnosis. Proportions surviving free of recurrent disease two or more years by National Wilms' Tumor Study (NWTS) Group were 5/5 in Group 1, 3/7 in Group 11, 1/3 in Group III, and 1/5 in Group IV; by age at diagnosis, 6/6 in those under 11 years old and 4/14 in those 11 or older; and by type of surgery, 10/15 who had nephrectomy and 0/5 with limited or no surgery. The data indicate that radiation and chemotherapy had only minor if any influences on relapse‐free survival. We conclude that (1) RCC in children is similar to its counterpart in adults; (2) RCC has a worse prognosis than Wilms' tumor except for the earliest stage; (3) nephrectomy alone is adequate treatment for Group I RCC, and (4) young age (<11 years old) may be prognostically favo

ISSN:0098-1532    

DOI:10.1002/mpo.2950110205

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractThe pathologic materials and clinical courses of 36 children aged 1 month‐22 years, with histiocytosis × (H‐X) seen at the Philadelphia Children's Cancer Research Center from 1970 to 1979 were reviewed. The pathologic subtype of H‐X was favorable (type II) in 31 patients, unfavorable (type I) in one patient, and unclassified in four patients whose specimens were limited to a skin biopsy. Sixteen patients had localized H‐X involving bone (14 patients), soft tissue (1 patient), or skin only (1 patient); all are alive and well after treatment with surgery alone (12 patients), radiation therapy (RT) (3 patients), or observation (1 patient); only 1 of the 16 developed recurrent H‐X. The other 20 patients presented with multifocal H‐X involving the skeleton alone (3 patients); the skeleton and soft tissues other than liver (7 patients); soft tissue exclusive of the liver (3 patients); soft tissue including the liver (4 patients); or soft tissues, skeleton, and liver or multiple drugs ± RT (15 patients). Seven of the 20 patients are alive and well without recurrence at a median of 4 years after diagnosis. Nine of the 20 patients, including 3 with liver dysfunction, responded completely to initial therapy but developed recurrence; each was retreated with drugs and is alive and well at a median of 4 years. The remaining 4 patients had widespread disease with dysfunction of the liver and/or hematopoietic system at diagnosis, failed to respond, and died. We conclude that (1) patients with multiple bony lesions with or without associated soft tissue disease or skin involvement have a favorable outlook and do not require systemic chemotherapy; (2) systemic treatment also is unnecessary for patients with localized H‐X since recurrence is rare; (3) drugs can benefit patients with multifocal H‐X, although the optimal duration of therapy is unclear; and (4) favorable response to treatment indicates high probability of disease‐free survival. However, organ dysfunction at diagnosis is ominous: four of seven patients with liver dysfunction are dead, as are all three patients who prsented with peripheral blo

ISSN:0098-1532    

DOI:10.1002/mpo.2950110206

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractThe distribution of T‐, B‐ and null‐lymphocytes was studied in the peripheral blood of 38 adult patients with acute nonlymphocytic leukemia (ANLL) and 15 with acute lymphocytic leukemia (ALL) at first diagnosis, during induction treatment, and in remission. Thirteen ANLL and 9 ALL patients were followed until relapse and during reinduction therapy. T‐ and B‐cells were detected by specific membrane marker. The pre‐ and posttreatment pattern of lymphocyte subpopulations was analyzed to determine their prognostic significance for remission incidence, remission duration, and survival. It was observed that in both types of leukemia, T‐cells are more affected by the leukemic process and cytostatic drugs than B‐cells. Nonresponding patients possibly have a reduced potential for recruiting precursor T‐ and B‐cells. At first diagnosis, no significant correlation was found between pre‐ or posttreatment variables and prognosis. At relapse, ANLL patients had a longer second remission when a high proportion of B‐cells was found; ALL patients with a high lymphocyte count before and after treatment, expe

ISSN:0098-1532    

DOI:10.1002/mpo.2950110207

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractA woman with pancreatic cancer developed acute interstitial infiltrates while receiving modest doses of two potential pulmonary toxins, mitomycin‐C and chlorozotocin. Chemotherapy pneumonitis, confirmed by lung biopsy, responded dramatically to prednisone with negligible sequelae. Comparison with the doses in other reported cases suggested additive or synergistic toxicity between these two alkylating agent

ISSN:0098-1532    

DOI:10.1002/mpo.2950110208

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractWe report a 15‐month‐old boy with acute myelomonocytic leukemia [French‐American‐British classification M4 (FAB‐M4)] whose leukemic cells demonstrated a translocation 46,XY, t(9;11)(p24;q12) that has not been described p

ISSN:0098-1532    

DOI:10.1002/mpo.2950110209

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractPneumothorax is an uncommon complication of radiation therapy to the chest. The proposed pathogenesis is radiation‐induced fibrosis promoting subpleural bleb formation that ruptures resulting in pneumothorax. We report on two young patients with primary sarcomas without pulmonary metastases who developed spontaneous pneumothorax after irradiation. Neither patient had antecedent radiographic evidence of pulmonary fibrosi

ISSN:0098-1532    

DOI:10.1002/mpo.2950110210

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY

摘要:

AbstractA comparison of radiographic and99mTechnetium methyldiphosphonate scintigraphic evaluations for extent of skeletal disease was made retrospectively in 17 pediatric patients with Group III and IV rhabdomyosarcoma. Thirteen children had evidence of skeletal metastases. Of these, four exhibited multiple sites of blastic as well as lytic skeletal involvement on x‐rays that were not detected by bone scans: two at the time of initial diagnosis, and two at relapse in children with prior radiotherapy to involved bones. In three additional patients a greater extent of bony disease was evident on x‐rays than on bone scan. Neither the pathology of the tumor, lytic versus blastic quality of the bone lesion, nor lesion size per se was found to correlate with the failure to detect them on the bone scan. Although bone scans have greater sensitivity than x‐rays for the detection of metastatic bone involvement in several adult and pediatric neoplasms, in the case of rhabdomyosarcoma, the radiographic exam appears to be a more sensitive indicator in some patients and should be considered an essential study in the evaluation of skeletal involvement by this

ISSN:0098-1532    

DOI:10.1002/mpo.2950110211

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1983

数据来源: WILEY