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Biomedical subjects

M Tefft

Publications and source records attributed to M Tefft.

At least 73 records · Page 4Linked to original sources

Primary chemotherapy in the treatment of children with bladder--prostate tumors in the Intergroup Rhabdomyosarcoma Study (IRS-II).

Twenty-nine children (24, male; 5, female) with non-disseminated rhabdomyosarcomas of the bladder or prostate were treated (1978-1980) by a primary chemotherapy regimen consisting of vincristine, actinomycin D, and cyclophosphamide ("Pulse" VAC), with or without local radiotherapy. During the initial 20 wk of chemotherapy, nine children achieved a Clinical Complete Response (CCR). Three of these are without evidence of disease (NED) and have functional bladders, two following partial cystectomy. Four who achieved a CCR subsequently relapsed or remained biopsy positive, but are at present NED following radiotherapy and anterior exenteration. Two patients who achieved CCR status relapsed and have died of disease. Twelve patients had a Clinical Partial Response (CPR) in less than 20 wk and two others in less than 40 wk. Seven of these are NED with intact bladders following chemotherapy-radiotherapy; and an additional patient is NED following partial cystectomy. Four patients in the CPR group have been treated by exenteration following failure to achieve complete response, and are NED. One patient has died, and one has progressive disease. Six patients had an inadequate response to chemotherapy (NR). Anterior exenteration was carried out in three, and two of these have survived. The overall results in these 29 patients are: (A) alive and disease-free with functional bladders, 11; (B) alive and disease-free following anterior exenteration, 10; and (C) dead or death from tumor anticipated, 8. The function of retained bladders (11) has been satisfactory.

Adolescent↗

Intergroup Hodgkin's disease in children study of stages I and II: a preliminary report.

The intergroup study of involved-field (IF) radiotherapy, IF radiotherapy plus MOPP chemotherapy, and extended-field (EF) radiotherapy for treatment of Hodgkin's disease in children has assessed 305 patients. Of these, 279 were "not ineligible" (no mediate cause for disqualification). Among 223 randomized patients, 144 were evaluable, 131 had documentation of complete or partial remission, 20 of the remitters relapsed, and two died. Among 62 nonrandomized patients with favorable presentations (unilateral upper neck, unilateral inguinal, or massive mediastinal disease), 29 had documented remission, two relapsed, and none died. Length of initial disease control (LIDC) was used to measure duration of response. LIDC was best in patients given IF plus MOPP, and 95% are disease free. EF was better than IF radiotherapy (P = 0.004). Of the disease characteristics prognostic for response (stage, histologic subtype, and presence of symptoms), only the last factor had a statistically significant effect on LIDC (P = 0.004). Ninety-six percent of the patients survive. Using criteria developed by the committee, 23% of the staging procedures reviewed were nonevaluable and 28% of the radiotherapy treatments were nonevaluable. The necessity for criteria for evaluation of staging and treatment is certain. Length of followup is too short for correlations of treatment with significant late effects and for relevant therapeutic recommendations.

Antineoplastic Agents↗

The treatment of Wilms' tumor: results of the Second National Wilms' Tumor Study.

One-hundred-eighty-eight children up to 16 years of age were randomized in the second National Wilms' Tumor Study (NWTS) with tumors that were confined to the kidney and that had been totally excised (Group I). Most fared well whether treated for six or for 15 months with both actinomycin D (AMD) and vincristine (VCR). No postoperative radiation therapy (RT) was given. The two-year relapse-free survival (RFS) and two-year survival rates were 88 and 95%, respectively. Two-hundred-sixty-eight randomized patients with more advanced local lesions (Groups II and III) and 57 with distant metastases (Group IV) had postoperative RT and were scheduled for 15 months treatment with either AMD and VCR (Reg. C) or AMD plus VCR plus Adriamycin (Reg. D). The 77% two-year RFS rate for Reg. D was significantly different from the 63% with Reg. C. As in the first NWTS, patients with tumors of unfavorable histology (UH) had a significantly worse prognosis than those with favorable histology (FH), as did those with positive nodes. Survival rates at two years were 54% for UH vs. 90% for FH, and 54% vs. 82% for those with and without lymph node involvement.

Adolescent↗

Radiotherapy and combination chemotherapy in advanced Ewing's Sarcoma-Intergroup study.

Forty-four patients with metastatic (36 patients) and advanced regional (eight patients) Ewing's sarcoma were treated on a protocol employing intensive combination chemotherapy and radiotherapy to all sites of overt disease (primary site and metastatic sites). Thirty-one patients achieved a complete remission, eight achieved a partial remission, and five patients did not respond to treatment. Of the 31 patients who achieved a complete remission (12 had metastases and six had regional spread), 18 remained disease-free with a median follow-up of 34 months, while recurrences occurred in nine patients. Four patients died of complications of treatment. Although late relapses can still be expected, it appears that intensive multimodal management in metastatic Ewing's sarcoma can produce prolonged disease-free survival and possibly cure in a significant percentage of patients.

Adolescent↗

Rhabdomyosarcoma of the female urogenital tract.

During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhabdomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, less than 4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.

Adolescent↗

Sarcomas of bone in childhood: pathologic aspects.

Some sarcomas of bone are not characteristically childhood tumors. Within the context of osteosarcomas, telangiectatic and parosteal (juxtacortical) variants deserve recognition. Among chondrosarcomas, which ordinarily are not tumors that occur in children, the mesenchymal variant should be recognized in children especially, this lesion may be extraosseous. Fibrous histiocytoma and its malignant variant have recently emerged as morphologically specific lesions, occasionally osseous, among other spindle cell tumors. Ewing's sarcoma, which is predominantly of osseous origin, consists of undifferentiated fragile cells. The elaborate spectrum of topographic, cytologic, nuclear, and other morphologic features that fall within the designation Ewing's sarcoma are described.

Adolescent↗

Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: an Intergroup Study.

A randomized study of 264 children and adults with previously untreated localized Ewing's sarcoma of bone was undertaken between 1973 and 1978 by 83 institutions of three national study groups: Children's Cancer Study Group, Southwest Oncology Group, and Cancer and Leukemia Group B. The Intergroup Study was designed to determine if the addition of adriamycin (ADR) or bilateral pulmonary radiotherapy (RT) to vincristine, dactinomycin, and cyclophosphamide (VAC therapy) would improve survival and reduce local recurrences and metastases. All patients received RT to the primary lesion, and the survival rate after 3 years was 65%. The most effective treatment regimen was VAC plus ADR; 74% of the patients were free of disease at 2 years. The lengths of disease-free status and survival of patients treated with VAC plus ADR or VAC plus RT did not differ. However, both regimens were significantly superior to treatment with VAC alone. The addition of ADR or bilateral pulmonary RT to VAC was highly advantageous to patients with nonpelvic primaries. Bone and lung were the major sites of distant relapse, but the addition of bilateral pulmonary RT showed no advantage over that of ADR in reducing the occurrence of lung metastases. These recent results should eliminate some of the pessimism that has accompanied a diagnosis of Ewing's sarcoma, although distant metastases continued to be a major reason for failure in the control of this tumor. Survival of these patients can be improved through well-controlled clinical trials designed to determine optimal adjuvant chemotherapy and treatment of the primary lesion.

Adolescent↗

Radiation therapy in the multimodal management of Ewing's sarcoma of bone: report of the Intergroup Ewing's Sarcoma Study.

This paper is a progress report on the role of radiation therapy (RT) in local tumor control and the decreased incidence of pulmonary metastasis in 251 patients entered in the Intergroup Ewing's Sarcoma Study. All were followed for more that 1 year, and their RT records were reviewed. Doses to the primary tumor in the range of 4,500--6,500 rad were administered over approximately 5 to 6 weeks in combination with 4 drugs, i.e., vincristine (VCR), dactinomycin (DAC), cyclophosphamide (CY), and adriamycin, or only the first 3. One group of patients received the 3 drugs and bilateral pulmonary irradiation (approximately 1,500 rad in 2 wk). Preliminary analysis showed a local primary tumor control of approximately 90%. Patients with lesions in the pelvis and humerus had local failure rates of 13% (7 of 54) and 21.4% (6 of 28), respectively. The treatment groups differed significantly in the incidence of pulmonary metastasis. Patients treated with the 4 drugs (regimen 1) had a 14% incidence, whereas 42% of those treated with only 3 drugs (regimen 2) developed pulmonary metastases. Of all patients treated with 3 drugs and pulmonary irradiation (regimen 3), 18% showed lung metastases. The study indicated that intensive chemotherapy and RT significantly improved the local control and survival of patients with localized Ewing's sarcoma. However, the high incidence of metastasis indicated the need for more effective systemic chemotherapy for further improvement of treatment results. More studies are needed so we can define the volume to be treated and the optimal dose of irradiation to determine a therapeutic strategy that will yield optimal survival and tumor control with the fewest sequelae.

Adolescent↗

Prognostic factors in children with Ewing's sarcoma.

Patient characteristics of 272 patients entered in a clinical trial conducted by the Pediatric Intergroup Ewing's Sarcoma Committee between June 1972 and November 1978 were examined for their relationship to prognosis. Prognosis was defined as disease-free survival time (time to local recurrence and/or metastatic disease) and overall survival time; all times were measured from the start of treatment. In a multivariate regression model, primary site of disease was the major variable that influenced prognosis, and patients with pelvic sites had the least favorable prognoses, followed by those with proximal and rib sites. The most favorable sites were distal and other. The median disease-free and survival times in weeks by primary site were, respectively: pelvis (69, 112), proximal (102, 141), rib (105, 109+), distal (226+, 240), and other (96+, 199+). Females had better prognoses than males; the median survival times were 197 and 147 weeks, respectively. An abnormal liver function as indicated by an abnormal serum glutamic-oxaloacetic transaminase value (greater than 45 IU) was a bad prognostic sign, although only 8 patients had this finding; their median survival time was 94 weeks. Patients who had resections had a slight advantage in survival compared with those having biopsies, though the difference favoring resection patients was not consistent for both sexes in any primary site. Individual characteristics of the patients that were of prognostic significance were: blood lymphocyte counts (high counts favorable), polymorphonuclear leukocyte counts (high counts unfavorable), and time from symptoms to diagnosis (times less than 1 mol favorable). Patients who received treatment 2 had significantly poorer prognoses than those given treatments 1 or 3. The median disease-free and survival times by treatment were (in wk): 1 (134, 198+), 2 (81, 120), and 3 (123, 182).

Adolescent↗

Multimodal therapy in metastatic Ewing's sarcoma: an Intergroup Study.

Multimodal therapy consisting of radiation therapy to all areas of gross disease and intensive combination chemotherapy was administered to 44 patients with Ewing's sarcoma. Seven of these patients had regional disease and the others had clinical evidence of distant metastases. The median duration of time on study for all patients was 75 weeks. A complete response occurred in 31 of the patients and 17 are currently free of disease. Four deaths resulted from complications of therapy; 2 were due to infection and 2 due to adriamycin-related cardiomyopathy.

Bone Neoplasms↗

Radiation therapy combined with systemic chemotherapy of rhabdomyosarcoma in children: local control in patients enrolled in the Intergroup Rhabdomyosarcoma Study.

The 524 children enrolled in the Intergroup Rhabdodomyosarcoma Study were the basis for our reviewing the effect on local tumor control by radiation therapy (RT) when combined with systemic chemotherapy. Of those who received RT, 291 were evaluated for dose and 317 for volume. No statistical differences of local control were observed at doses ranging between 3,500 and 6,000 rad, by extent of local disease, nor by adequacy of RT volume as long as the tumor mass and a 5-cm margin were in the RT portal.

Adolescent↗

Special considerations related to primary site in rhabdomyosarcoma: experience of the Intergroup Rhabdomyosarcoma Study, 1972--76.

Rhabdomyosarcomas (RMS) arising in the head and neck region, retroperitoneum, and perineum were considered together here because the usual surgical approach is incisional biopsy. Thus successful treatment of these neoplasms depends on effective radiation therapy and chemotherapy. From November 1972 through December 1976 the Intergroup Rhabdomyosarcoma Study accrued 127 patients with primary tumors in the head and neck, 34 with orbital tumors, 24 with sarcomas arising in the retroperitoneum-pelvis (no genitourinary), and 11 with perineal lesions. Results of treatment varied among these primary sites. Patients with orbital RMS had the best prognoses; 77% of them were free of disease, compared with a 51% disease-free rate in patients with nonorbital head and neck RMS. In this latter group, disease recurrence was evenly divided among local failure, distant metastases, and direct meningeal extension. Prognoses were similar for retroperitoneal tumors; 46% of such patients are currently free of disease. That the perineum was a rare site for RMS was fortunate because only 3 of 11 such patients (27%) are free of detectable disease now (January 1979). We concluded that the site of the primary tumor is an important prognostic variable in children with RMS.

Adolescent↗

Intergroup Ewing's Sarcoma Study: local control related to radiation dose, volume, and site of primary lesion in Ewing's sarcoma.

One hundred ninety-three patients with localized Ewing's sarcoma treated at participating instutitions of the Intergroup Ewing's Sarcoma Study form the basis for this report. All patients received radiation therapy to the primary lesion and were randomized to receive vincristine, actinomycin-D, and cyclophosphamide (VAC) plus adriamycin (Regimen I); VAC alone (Regimen II); or VAC and bilateral pulmonary irradiation (Regimen III). Local control was achieved in 96% of the patients in Regimen I, and 86% of the patients in both Regimens II and III. The median duration of follow up was 83 weeks and median survival time was 172 weeks. Incremental doses of irradiation did not result in significant changes in the rate of local control of primary lesions. The local control rate was the same (92%) for tumors treated by means of whole-bone irradiation or with at least 5 cm of free margin around the lesion. The local control rate decreased to 79% for lesions treated with less than 5-cm margin. Excellent control was obtained for lesions involving the skull or spine (100%), and distal bones (fibula, 96% and tibia, 91%). Less favorable control rates were noted for pelvic and humeral lesions (84% and 79%, respectively). Bilateral pulmonary irradiation for subclinical disease played a role in lowering the incidence of lung metastases from 38% to 20% for patients treated with VAC. Lung metastases were similarly decreased (10%) when adriamycin was added to VAC chemotherapy.

Bone Neoplasms↗