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Biomedical subjects

E Bouffet

Publications and source records attributed to E Bouffet.

At least 109 records · Page 6Linked to original sources
PubMed1991

Burkitt's lymphoma: a model for clinical oncology.

Burkitt's lymphoma, a pathological entity initially described in Africa, is the most common childhood lymphoma in western countries and represents approximately 5% of all adults lymphomas. This high grade small non-cleaved diffuse lymphoma is a model with which to study the relations between cancer and viruses, the chromosomes and the genes. Burkitt's lymphoma is also a model for clinical research which allows evaluation of the dose effect concept with chemotherapy and the role of megatherapy with autologous bone marrow rescue.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1991

Expression of P-glycoprotein restricted to normal cells in neuroblastoma biopsies.

Immunohistological detection of P-glycoprotein (P-gp) with monoclonal antibody C219 was performed on serial sections of 37 neuroblastoma specimens representative of the different forms of the disease, from stage 1 ganglioneuroma to stage 4 neuroblastoma. Malignant cells, irrespective of their degree of maturation varying from neuroblasts to ganglion cells, were negative on all specimens. The expression of P-glycoprotein was detected in nine specimens, but it was restricted to normal cells within the tumour. In four specimens, C219 reacted with normal infiltrating cells in the stroma (i.e. monocytes, histiocytes or fibroblasts) representing 5 to 10% of the total population within the section; in three specimens, the residual adrenal gland was strongly positive, and in two ganglioneuromas, a weak reactivity of C219 was observed on a few satellite cells and schwann cells. Three of 15 biopsies obtained at diagnosis contained normal P-gp positive cells: two were classified as stage 1 ganglioneuromas; one was a typical stage 4 composite tumours with positive histiocytes and fibroblasts in the well-differentiated counterpart. Six of 22 biopsies obtained after patients had received our current protocol of chemotherapy contained normal P-gp positive cells: five were partially differentiated and necrotic under the effect of chemotherapy; only one positive specimen was classified as undifferentiated neuroblastoma. Among negative specimens from previously treated patients, one was obtained from a patient in relapse after high-dose chemotherapy and ABMT, two were obtained from patients who had not responded to induction therapy, and six from patients in partial remission after induction therapy. The clinical evolution was very similar in both groups of patients with P-gp negative or positive biopsies. These findings suggest that the quantitative assessment of MDR RNA by northern blotting on fresh homogenates is likely to overestimate its expression on neuroblastoma cells, and that the mechanism of chemoresistance in widespread neuroblastoma is less likely to be associated with P-gp expression.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1991

Improved survival at 2 and 5 years in the LMCE1 unselected group of 72 children with stage IV neuroblastoma older than 1 year of age at diagnosis: is cure possible in a small subgroup?

The objectives of this study were to determine (1) the role of selection before bone marrow transplantation (BMT), (2) the role of vincristine, melphalan, and total body irradiation (TBI) as consolidation of induction therapy for stage IV over 12 months at diagnosis, and (3) the role of immunomagnetic purging in metastatic neuroblastoma. Among 72 consecutive unselected patients, 10 were not grafted (four died at induction: two in complete remission [CR], two in partial remission [PR]); three had bone marrow progression before harvest; one had uncontrolled progression; and two had parental refusal). Sixty-two patients were grafted (23 in CR/very good PR [VGPR] and 39 in PR). Among the 62, 33 were consolidated with at least 90% excision of their initial tumor excised (53.2%), 15 with catecholamine secretions (24.2%), 22 with minor bone marrow involvement (35.5%), and 31 with positive bone scan (50%). Median observation time is 59 months. Progression-free survival (PFS) for the 10 excluded patients was 20% at 2 years and 0% at 4 years. PFS for the grafted population (n = 62) is 40% at 2 years, 20% at 4 years, and 13% at 7 years. No difference was observed between patients grafted in CR/VGPR or in PR. However, a group of 19 children was grafted resulting in complete normalization of metastasis (regardless of primary-site tumor status). In this group, PFS at 59 months was 38% with no relapses up to 7 years post-BMT. A group of 31 patients with no bone involvement at BMT was also identified. PFS at 5 years is 30% compared with 12% for bone-positive patients at BMT. Moreover, the 11 children presenting at diagnosis with no bone involvement (Evans stage IVS or stage C Memphis) and consolidated with BMT had PFS at 5 years of 50% with no late relapses. A subgroup of stage IV neuroblastoma patients older than 1 year of age at diagnosis may be curable with this therapeutic approach, and the use of multivariate analyses to search for prognostic factors is warranted in currently existing international registries.

Age Factors↗
PubMed1991

Interleukin-2 and lymphokine-activated killer cells in 15 children with advanced metastatic neuroblastoma.

A phase II trial using interleukin-2 (IL2) and lymphokine-activated killer (LAK) cells was carried out in an attempt to treat children with end-stage neuroblastoma. Fifteen patients (median age, 7 years) were enrolled in the study. Twelve were in relapse after massive chemotherapy and autologous bone marrow transplantation (ABMT), and three had a primary refractory disease after conventional chemotherapy. IL2 was administered as an 18 x 10(6) IU/m2/d continuous infusion. One course consisted of a double 5-day treatment period separated by a 6-day break. Cytapheresis to harvest LAK progenitor cells was performed during the rest period. After a 4-day in vitro culture, LAK cells were reinjected during the second cycle of therapy. A phenotypic and functional analysis of immunologic parameters was conducted along with the therapeutic protocol. Toxicity was significant with two toxic deaths (cardiotoxicity and respiratory distress). The reinfusion of large amounts of LAK cells was clearly involved in one case, but this particularly severe toxicity has to be related to the patient's status (ie, heavy pretreatment). No significant clinical response was seen. The immunologic monitoring showed phenotypic and functional modifications in these patients before initiation of treatment and an unexpected absence of evolution of these parameters during IL2 therapy. Although the origin of these immune dysfunctions is not clear, they could be involved in the failure of IL2 therapy. Future studies of IL2 therapy in neuroblastoma should be undertaken earlier in the course of the disease.

Antibodies, Monoclonal↗
PubMed1991

[Neuropsychological sequelae in children treated for brain tumor].

Treatment modalities of childhood brain tumors are associated with adverse neuropsychologic sequelae that may be manifested years after therapy. Based on recent published studies, the authors review the etiological factors, the symptoms and the evolutive features of these disorders and their management.

Brain Neoplasms↗
PubMed1991

[Contribution of imaging in the diagnosis and follow-up of brain tumors].

With the use of modern radiological imaging (US, CT-scan, MRI), the most important informations to recognize a tumor remain the morphological features (localization, size, extension, number of lesions...). The precision of these informations is constantly improved (use of contrast medium, various slices angulations...) and in this field, MRI is the most precise examination. Moreover, these less aggressive investigations allow a repeated and more efficient therapeutic follow-up. In contrast with initial expectations, these radiological investigations do not always allow a formal histological characterization of the different tumor types of the central nervous system, even if some intratumoral components are recognized (fat, calcifications, liquids...).

Brain Neoplasms↗
PubMed1991

[Medulloblastoma].

Medulloblastoma is a frequent brain tumor in childhood. Recent advances in histogenesis, management of early and late side-effects, and prognosis are discussed in this review.

Cerebellar Neoplasms↗
PubMed1991

[Hemispheric tumors].

Despite great histological polymorphism, clinical features of supratentorial brain tumors are mainly limited to neurological deficit, and focal epilepsy and/or symptoms secondary to raised intracranial pressure. The treatment relies on surgery and radiotherapy; chemotherapy has not yet demonstrated its efficacy. Failures are explained by the limitation of neurosurgery, and by the toxicity of the radiotherapy that precludes the use of high dosages. Some good vital results are hampered by neuropsychic sequellae.

Brain Neoplasms↗
PubMed1990

Interleukin 2 immunotherapy in children with neuroblastoma after high-dose chemotherapy and autologous bone marrow transplantation.

Four children with persistent neuroblastoma after marrow ablative chemoradiotherapy and autologous bone marrow transplantation received continuous infusion of recombinant interleukin 2, 75 to 120 days after the graft. Recombinant interleukin 2 therapy did not induce any major or nonreversible toxicity, hematological toxicity in particular. One patient entered complete remission for 9 months and a second patient had a long-lasting normalization of urinary catecholamine metabolites with more than 50% regression of bone marrow metastases (8 months). In three children, recombinant interleukin 2 and a second patient entered complete remission for 9 months therapy was followed by major increase and activation of circulating natural killer cells which amounted to 80% of the circulating mononuclear cells.

Bone Marrow Transplantation↗
PubMed1990

Autologous bone marrow transplantation in pediatric solid tumors.

During the last 5 years massive chemotherapy and autologous bone marrow transplantation have been increasingly explored in the treatment of pediatric solid tumors, mainly for neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, Wilms' tumor, germ cell tumors, osteosarcoma, and retinoblastoma. Although the disease course could be changed successfully in most instances, the long-term survival has not yet been improved much over the best available conventional treatments. Despite this, in responding relapsed patients this approach seems promising and may represent the only chance of cure. New and better induction regimens are needed.

Adolescent↗
PubMed1990

[Massive bone grafts in children (apropos of 10 cases)].

We studied 10 massive - 70 degrees C frozen dried allografts (7 terminal intermediate) in children between the ages of 9 and 16 with malignant tumors (8 primary osteogenic sarcomas and 2 Ewing sarcomas). A good response to chemotherapy was observed. The average follow-up is 2 years (7-36 months). Results showed 4 mechanical complications (2 plate failures, 2 graft fractures) and no deaths. The surgical procedure and postoperative management are discussed and compared to prosthetic reconstruction.

Adolescent↗
PubMed1990

[Role of massive chemotherapy and bone marrow graft in the treatment of non-Hodgkin's lymphoma].

Intensive chemotherapy and high dose chemotherapy with bone marrow transplantation are 2 complementary therapeutic procedures for non Hodgkin's lymphomas. In children, indications for BMT in NHL are limited to patients who fail to achieve complete remission and for patients in sensitive relapse. In adults, the role of BMT is also recognized for patients who fail to achieve remission with initial therapy. The advantage of either conventional regimens or massive therapy with BMT for patients with poor prognostic criteria is still under evaluation (LNH 87 protocol). The PARMA protocol tests, in a multicenter randomized study, the value of BMT for patients in sensitive relapse. Results of BMT for patients with refractory NHL and resistant relapse, remain poor. The role of BMT in low grade lymphomas is debatable. The trials for evaluation of massive chemotherapy and BMT in NHL will contribute to defining the potential role of this therapy in France: a potential 1,000 cases per year may be treated by this method.

Adult↗