[Guidelines from the National Federation of Cancer Prevention Centers (FNCLOC) on trials of drug prevention of breast cancer by tamoxifen ].
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Biomedical subjects
Publications and source records attributed to T Philip.
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BACKGROUND: Desmoplastic small round cell tumors (DSRCT) have been only recently identified. METHODS: The authors report DSRCT in two pediatric patients (an 8-year-old boy and 12-year-old boy). In both patients, the initial diagnosis was rhabdomyosarcoma. The resistance to standard chemotherapy and radiation therapy prompted the authors to review the initial biopsy specimens and perform complementary immunophenotypic characterization. RESULTS: These analyses revealed that the tumor cells were strongly positive for keratin epithelial marker antigen, desmin, vimentin, neurospecific enolase, and S100 protein, corresponding to pleomorphic differentiation, characteristic of DSRCT: CONCLUSIONS: The authors suggest that extensive immunohistologic characterization be performed in all cases of small round cell tumors of the abdomen so that the diagnosis of DSRCT is not overlooked. These rare tumors are refractory to chemotherapy, and initial aggressive surgery is warranted.
Primary osteosarcoma of the pelvic bones represents less than 10% of all osteosarcoma. Despite mutilating surgical procedures or high-dose radiotherapy and neoadjuvant chemotherapy, local failures and distant metastases remain common. The 2-year actuarial survival rate is less than 50% and the disease-free survival is under 20% at 2 years. We report 4 cases of pelvic osteosarcoma treated by a combination of chemotherapy, photon beam and neutron beam radiotherapy. After a median follow-up of 24 months, all 4 patients are alive and disease free.
Nine children with soft tissue sarcomas, five of them rhabdomyosarcomas with initial metastatic disease, (one patient, partial response, one patient), refractory primary, (two patients, relapse, five patients) were treated with a combination of high-dose VP16 (100 mg/m2 daily for 5 days) and cisplatin (40 mg/m2 daily for 5 days). The response rate was five out of nine or 55% (+/- 32%) (two complete and three partial, responses). Three of the five responders had rhabdomyosarcomas. The duration of response was 4-58 months (median 11 months). The toxicity was mainly hematological. Thus, the high-dose VP16-cisplatin association warrants further evaluation in soft tissue sarcoma in children.
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A double-institution phase II study was performed in patients with metastatic renal cell carcinoma treated subcutaneously (s.c.) with interleukin 2 (IL-2) and alpha-interferon (INF-alpha). Thirty-eight patients were treated over a course of 7 weeks. Initially (day 1 + 2) patients received s.c. IL-2 at 18 x 10(6) IU m-2. During the following 6 weeks, patients received s.c. IL-2 at 3.6 x 10(6) IU m-2 for 5 days per week and s.c. INF-alpha at 5 x 10(6) for 3 days per week. Thirty-eight patients were evaluated for response. An objective response was seen in seven patients (18.4 +/- 12.3%), with one complete response and six partial responses. Median duration of response was 6.7 months. Toxicity could be evaluated in 38 patients and was limited. Mild to moderate toxicity included fever (97%), fatigue or malaise (76%), nausea or vomiting (50%), anorexia (32%), hypotension (26%), neurological disturbances (26%) and hypercreatininaemia (39%). In addition, four grade IV haematological toxicities were noted. No cardiac side-effects were seen. IL-2 and INF-alpha given by this schedule can be safely administered in an outpatient setting. The objective response rate was similar to our previous treatments with high-dose IL-2 given as a continuous infusion.
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PURPOSE: CNS involvement of non-Hodgkin's lymphoma (NHL) has always been considered a poor prognostic factor in relation to survival with conventional therapy. However, its effect on the outcome of autologous bone marrow transplantation (ABMT) has not been assessed. We examined this using data from the European Bone Marrow Transplant (EBMT) Lymphoma Registry. PATIENTS AND METHODS: One thousand four hundred sixty-four patients with NHL have been reported to the EBMT registry, of whom 62 had CNS involvement. Patients were divided into those who were clear of CNS disease at the time of ABMT and those who were not. Response, complications, and outcome to ABMT were analyzed, as were details of CNS diagnosis, treatment, and prophylaxis. RESULTS: Status at transplant was the only factor that significantly affected outcome of ABMT on univariate analysis (P = .03). The progression-free survival (PFS) rate of the group that had no CNS involvement at ABMT was 42% at 5 years, compared with 27% in a group of stage IV NHL patients without CNS disease (matched for status at transplant and histology). There were four of 45 (8.9%) toxic deaths. The PFS rate of the group that had CNS involvement at ABMT was 9% at a median follow-up time of 71 months, which was significantly different (P = .001) from that of the other group. There were five of 17 (29.4%) toxic deaths (P = .043). Patients who had CNS involvement at diagnosis, as compared with relapse, and those treated with both intrathecal chemotherapy and irradiation had a better outcome. CONCLUSION: The presence of CNS disease before ABMT but not present at transplant does not adversely affect the outcome of ABMT. In contrast, patients with CNS involvement at the time of ABMT have a poor prognosis, although a small number survive in complete remission (CR).
The results of high-dose therapy and autologous BMT for patients with intermediate/high grade NHL were analysed in 82 patients aged > or = 55 years, identified from the EBMT lymphoma database. These were compared with the results for 82 patients aged < 55 years who were matched on the basis of disease status at transplantation, presence of bone marrow or CNS involvement and closest date of transplantation. The 5 year actuarial progression-free survival (PFS) for patients aged < 55 years was 33% compared with 37% for the > or = 55 year group (p = 0.08). Corresponding figures for overall survival (OS) were 39% and 38%, respectively (p = 0.19). No difference in outcome was observed according to histological subtype. Although the number of patients receiving total body irradiation (TBI) is small, a significantly lower PFS was observed in patients > or = 55 years receiving TBI-based high-dose regimens compared with younger patients. This difference was due to a higher toxic death rate in the older patient group. In this retrospective matched analysis, age > or = 55 years was not associated with lower PFS or OS following high-dose therapy and autologous BMT. The increased toxic death rate in patients receiving TBI suggests that this should be avoided in older patients, who should receive chemotherapy only high-dose regimens.
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