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Biomedical subjects

E Bouffet

Publications and source records attributed to E Bouffet.

At least 73 records · Page 4Linked to original sources
PubMed1996

[Wilms tumor and Bloom syndrome].

UNLABELLED: Bloom syndrome is characterized by growth failure, skin anomalies with sun sensitivity, minor anatomic defects, excessive chromosomic fragility and usually severe immune deficiency. The chromosome fragility predisposes these children to the development of hematologic malignancies and solid tumors. CASE REPORT: Morgan, a 4-year-old boy with Bloom syndrome, developed a Wilms tumor. Chemotherapy was poorly tolerated. Two years later, the child died from an uncontrolled progressive disease. CONCLUSION: This is the fourth reported case of Wilms tumor occurring in a child with Bloom syndrome. This possibility requires repeated abdominal ultrasonography in such patients.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

[School at the end of life. What objectives what hope?].

BACKGROUND: School intervention programs for children and adolescents with cancer have been developed in France during the last years. Their primary objective is to improve the pschychosocial adaptation of these children to normal life through school integration. These programs are however inadequate for these children who will ultimately die from their disease and may require a specific school intervention. MATERIAL AND METHODS: School data from 30 children requiring palliative care were prospectively collected. School interest, its evolution and specific adaptations according to the child's status were analysed. RESULTS: Sixty per cent of the children showed a continuous desire for school education throughout their life's end. Reading, mathematics and computer work were the most favorite topics. Physical disability and fatigue may change their request during the evolution of the disease. School refusal occurred in 40% of the children and is mainly due to other extra-academic topics, or related to uncontrolled pain. CONCLUSION: Objectives of life's end school are different from educational programs proposed for children with cancer. They must be part of palliative care and have to be defined as a specific entity.

Adolescent↗
PubMed1996

[Pain in lumbar puncture. Results of a 2-year discussion at the French Society of Pediatric Oncology].

BACKGROUND: Lumbar puncture is a common procedure in pediatric onco-hematology. Repeated invasive painful procedures may contribute to increase distress displayed during medical treatment, and cause regression, depression and other psychological disorders. MATERIAL AND METHODS: A two-year workshop of the Société française d'oncologie pédiatrique was reviewed through a questionnaire assessing changes in technical management of lumbar puncture, local anesthesia, sedation and cognitive-behavioral interventions. RESULTS: Significant changes were found for pain assessment, local anesthesia procedures, and nitrous oxide administration. The extensive use of the Emla cream largely contributed to reduce pain. Anxiety however remained an unsolved problem, particularly among young children. CONCLUSION: Pain in pediatrics remains a major challenge. This workshop is a model of reflexion to achieve a better management of pain during invasive medical procedures.

Anesthetics, Local↗
PubMed1996

[Unusual relapse for neuroblastoma. Case of an intracranial relapse].

The usual sites of initial metastatic deposits in neuroblastoma are osteo-medullary. With modern therapies including megatherapy and hematopoietic rescue, prolonged survival is obtained. However, unusual metastatic sites are more and more often described during the prolonged evolution of these patients such as brain metastases. A case of isolated intracerebral metastatic relapse is reported here in a patient who had received 4 months before a megatherapy in first complete remission. Pathogeny and therapeutical implications are discussed.

Adrenal Gland Neoplasms↗
PubMed1995

The C5R protocol: a regimen of high-dose chemotherapy and radiotherapy in primary cerebral non-Hodgkin's lymphoma of patients with no known cause of immunosuppression.

In most reported series, less than 20% of patients with primary cerebral non-Hodgkin's lymphoma (PCL) and no known cause of immunodepression are alive and disease-free 5 years after the initial diagnosis. Whether chemotherapy improves the outcome of these patients remains unclear. We report a pilot study of a protocol (C5R) with 5 courses of chemotherapy followed by cranial radiotherapy in 25 adult patients with PCL and no known cause of immunodepression. The median age was 51 years (range, 16 to 70 years) and the median performance status was 2 (range, 1 to 4) in this series. Fourteen patients (56%) achieved a complete response and 4 (16%) achieved a partial response 1 month after the completion of the treatment. Four patients died in the first month of treatment because of progression (n = 1) or toxicity (n = 3). In 3 patients, the treatment could not be performed because of patient refusal (n = 1) or severe infections (n = 2). Myelosuppression was the most frequent side effect; febrile neutropenia occurred in 96%, 89%, 69%, and 74% of the patients after the second, third, fourth, and fifth courses of chemotherapy, respectively. Grade 4 thrombocytopenia occurred in 20% of the patients. With a median follow-up of 24 months, the projected survival of the group at 2 and 5 years is 70% and 56%, respectively. The 4 early deaths occurred in the subgroup of 6 patients greater than 60 years of age with an international prognostic index (IPI) greater than 3. In the 19 remaining patients (76% of this series) less than 61 years of age or with an IPI less than 4, the projected overall survival at 2 and 5 years is 88% and 70%, respectively. The C5R protocol is a highly efficient regimen in nonimmunosuppressed patients with PCL less than 61 years of age or with an IPI less than 4.

Adolescent↗
PubMed1995

Renal function following unilateral nephrectomy for neuroblastoma and Wilms' tumour.

To estimate the side effects of chemotherapy and the influence of age at the time of nephrectomy on renal function, we investigated renal function in 34 uninephrectomised children with neuroblastoma (NB) or Wilms' tumour (WT). The results were compared with 6 controls who underwent nephrectomy for non-malignant disease. Study of renal function was primarily based on the clearance of inulin and para-aminohippuric acid (Cin and CPAH, ml/min per 1.73 m2). No significant differences in Cin/CPAH (mean +/- SD) were found between the NB group (90 +/- 24/421 +/- 95), WT group (85 +/- 17/386 +/- 104) and the controls (93 +/- 13/430 +/- 61). Children with NB and WT were divided into two subgroups according to the theoretical nephrotoxic risk. There was no significant difference in renal function between NB or between WT subgroups. Cumulative cisplatin doses in children with NB did not affect renal function significantly. The age at time of unilateral nephrectomy (< or = 2 years vs. > 2 years) was not associated with a higher risk of renal damage in WT children, whereas in NB children the filtration fraction (Cin:CPAH) was higher in younger children (mean +/- SD: 0.243 +/- 0.023 vs. 0.191 +/- 0.041). In conclusion, uninephrectomised children with NB are supposed to have a higher risk of drug-induced renal impairment compared with those with WT. Our data do not confirm this hypothesis, since renal function was comparable to controls in both groups, except in younger patients with NB who show a high filtration fraction. Since the survival of children with NB has improved, a longer follow-up of their renal function in needed.

Age Factors↗
PubMed1995

Non-metastatic childhood ependymomas.

PURPOSE: Intracranial ependymomas of childhood are relatively infrequent. There are significant disagreements concerning optimal postoperative treatment as well as the patterns of relapse following treatment. The purpose of this retrospective study was the analysis of the recurrence pattern and therefore the implication on the extent of the radiotherapy fields. Data from 37 patients referred within 19 years were used for this study. PATIENTS AND METHODS: From April 1975 to July 1993, 37 children aged 1-14 years were referred for postoperative treatment of an intracranial ependymoma. Twenty-eight children received postoperative radiation therapy and 26 patients received chemotherapy. The median follow-up is 6 years (range 2 months to 19 years). RESULTS: Overall survival and event free survival at 5 and 10 years were 40%. Eighteen children relapsed. Relapses occurred from 1.5 months to 3.6 years post treatment. Relapses were distant in four cases and local in 14. Age, sex, extent of primary resection, chemotherapy and type of radiation therapy did not influence the outcome. Children with poorly differentiated tumors who did not receive postoperative radiation therapy had a higher relapse rate but this difference is not statistically significant. CONCLUSIONS: Despite doses of radiation > or = 50 Gy the majority of recurrences were local. Our results, despite the small number of patients are in accordance to those previously published, suggest that prophylactic craniospinal irradiation is superfluous. Better means of achieved local control are required, such as three-dimensional conformal radiation therapy with dose-escalation study or hyperfractionation regimen.

Adolescent↗
PubMed1995

Etoposide and carboplatin in extraocular retinoblastoma: a study by the Société Française d'Oncologie Pédiatrique.

PURPOSE: A phase II study of etoposide (VP-16) and carboplatin was performed in patients with extraocular retinoblastoma to evaluate the response rate with this drug combination. PATIENTS AND METHODS: Twenty patients with extraocular retinoblastoma, age 9 to 120 months, were included in a cooperative multicenter phase II study of the Société Francçaise d'Oncologie Pédiatrique (SFOP). The schedule consisted of consecutive 5-day treatment with VP-16 100 mg/m2/d and carboplatin 160 mg/m2/d. RESULTS: The response rate for the 20 patients was 85%; there were nine complete responses and eight partial responses. Hematologic toxicity was the only serious observed toxicity and was always manageable. CONCLUSION: This combination of VP-16 and carboplatin is highly effective in extraocular retinoblastoma. The high response rate is encouraging for further evaluation of this drug combination in adjuvant chemotherapy when necessary after enucleation or in neoadjuvant chemotherapy for intraocular tumors.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1995

Preirradiation chemotherapy including "eight drugs in 1 day" regimen and high-dose methotrexate in childhood medulloblastoma: results of the M7 French Cooperative Study.

The aim of this study was to evaluate the feasibility and efficacy of a protocol that includes "sandwich" chemotherapy, that is, chemotherapy alternated with radiotherapy, and reduced doses of supratentorial irradiation in children with medulloblastoma. Between March 1985 and September 1988, 70 successive children with newly diagnosed medulloblastoma from eight centers were treated in this prospective nonrandomized study. Patients were assigned to two risk groups. Group A included patients with macroscopically complete or subtotal excision, no brainstem involvement, no atypical cells in the cerebrospinal fluid, normal myelography, and who were more than 2 years of age. Group B patients encompassed those who did not fit the criteria for Group A. Two children were excluded from analysis after histological review confirmed ependymoma. Thus, a population of 68 children was selected, with 31 in Group A and 37 in Group B. Treatment consisted of two courses of the "eight drugs in 1 day" ("8/1") regimen followed by two courses of high-dose methotrexate (12 g/m2). Radiotherapy was begun during the 7th week after surgery in Group A and during the 5th week in Group B. In patients older than 2 years, the median radiation dose to the posterior fossa, the spinal axis, and the brain was 54 Gy, 36 Gy, and 27 Gy, respectively. Group B patients received postirradiation chemotherapy with four 8/1 courses monthly. The median time from surgery to radiation therapy was 50 days (range 21 to 141 days). One fatality due to chicken pox on Day 102 and one World Health Organization Grade IV infection occurred. The estimated 5- and 7-year disease-free survival (DFS) rates were 62% and 59%, respectively. These were 74% and 62% in Group A and 57% and 57% in Group B. Patient age, extent of resection, and radiation dose to the whole brain had no prognostic value. Patients with metastasis had a nonsignificant trend for a worse prognosis than patients with nonmetastatic disease (7-year DFS 45% vs. 68%, p = 0.11). In Group B, the 7-year DFS rates for children who received more or less than 30 Gy to the brain were 69% and 52% respectively (p = 0.15). There were recurrences in the posterior fossa (37%), spine (20%), and brain (20%). After a review of radiotherapeutic treatments, only one supratentorial failure could be blamed on reduction of the supratentorial radiation dose. This "sandwich" chemotherapy appeared to be feasible and did not show adverse survival data when compared to other series.

Adolescent↗
PubMed1995

[Radiotherapy and Ewing tumor in children].

Despite high local control rate in Ewing's sarcoma the exact indication of radiation therapy is still controversial as well as the choice of the target volume and the optimal dose of radiation. The importance of the quality of radiotherapy has been stressed in recently published data and has shown a significant impact on long term local control with adequate radiation therapy. The dramatic improvement of precision allowed by the conformal therapy and three dimensional dosimetry allow to expect a decrease of late effects expected for second malignancies. However, late sequellae and radio-induced osteosarcoma still remain the major side effects after radiotherapy. The authors discuss the results of the main trials on Ewing's sarcoma on the choice of dose and target volume. Surgery is still the preferred choice for small tumors if the foreseen outcome is identical.

Bone Neoplasms↗
PubMed1994

Desmoplastic small round cell tumors of the abdomen.

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.

Antigens, Neoplasm↗
PubMed1994

The role of fast neutron therapy in unresectable pelvic osteosarcoma: preliminary report.

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.

Adolescent↗
PubMed1994

Carboplatin and VP 16 in medulloblastoma: a phase II Study of the French Society of Pediatric Oncology (SFOP).

The purpose of this study is to evaluate the antitumor activity of combination carboplatin and etoposide in measurable medulloblastoma. From January '89 to January '92, 26 patients with medulloblastoma were included in a multicentric phase II study of 2 courses of carboplatin 160 mg/m2/d day 1 to day 5 and VP16 100 mg/m2/d day 1 to day 5. Median age was 10 years (19 months-14.5 years). First treatment was surgery alone in 1 patient, surgery + radiotherapy in 4 patients, surgery + chemotherapy in 2 patients less than 3 years old, surgery + radiotherapy + chemotherapy in 19 patients ("8 drugs in 1 day" based:17, SIOP I:1, SIOP II:1). Previous treatment included cisplatin (20 cases), carboplatin (1 case), and VP16 (7 cases). Measurable disease was evaluated by CT scan, MRI or myelogram and CSF. Response rate (RR) was 72 +/- 10%:8 complete responses (CR), 10 partial responses (PR), 1 objective effect (OE), 6 progressive disease (PD), 1 non-evaluable. Thirty-six courses were evaluated for toxicity. Median duration of WHO grade 4 neutropenia was 8 days (0-23). One patient died at day 18 after the first course because of diffuse haemorrhage during septic aplasia. Five other non-life-threatening septicemias were recorded. Median number of platelet transfusions was 1 (0-4). One child who had achieved a PR after two courses died from CNS bleeding after the third course. This drug combination achieves a high response rate in childhood medulloblastoma. Severe toxicity has been mainly encountered in previously heavily treated patients. Tolerance may be acceptable in newly diagnosed children, but careful hematological follow-up and platelet transfusional support are definitely mandatory.

Adolescent↗
PubMed1994

High-dose VP16 cisplatinum in soft tissue sarcoma of children.

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.

Adolescent↗
PubMed1994

Metastatic medulloblastoma: the experience of the French Cooperative M7 Group.

A retrospective analysis was performed to determine the outcome of children with metastatic medulloblastoma given a standardised treatment programme. Of 68 consecutive patients treated in the French M7 protocol for medulloblastoma, 23 presented with metastatic disease. They were uniformly treated with surgery, and the same protocol of chemotherapy and craniospinal radiotherapy. The 7-year relapse-free survival rate is 43% for metastatic patients compared to 68% for patients with localised disease. Survival did not correlate with age, sex, location of metastases, extent of initial surgery and the dose of radiation therapy on the posterior fossa. Survival did correlate with the dose to the cranial field with a threshold dose of 30 Gy. Patients with metastatic disease have a worse prognosis and require more aggressive therapies at initial presentation. The prognostic impact of the different sites of metastatic disease requires further evaluation in cooperative studies.

Adolescent↗
PubMed1994

Once daily antibiotic regimen in paediatric oncology.

The feasibility and efficacy of a once daily antibiotic regimen were assessed in children with malignant tumours. Over a 44 month period, 296 febrile episodes were treated with a regimen of once daily ceftriaxone-amikacin (and teicoplanin or vancomycin if the patient had a central line). The treatment was successful in 272 (92%) episodes without modification of the antibiotic regimen, and only one patient died of uncontrolled sepsis. A once daily antibiotic regimen is therefore feasible and worthwhile in the treatment of febrile episodes in children with cancer.

Adolescent↗