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E Bouffet

Publications and source records attributed to E Bouffet.

At least 37 records · Page 2Linked to original sources
PubMed2000

Growth hormone hypersecretion in a girl with neurofibromatosis type 1 and an optic nerve glioma: resolution following chemotherapy.

Growth hormone hypersecretion is extremely rare in childhood. We report a girl with neurofibromatosis type 1, an extensive optic nerve glioma and growth hormone hypersecretion. She was treated with chemotherapy to prevent further extension of her sight-threatening tumour. Three years after chemotherapy her growth hormone hypersecretion has resolved although she has gone on to develop precocious puberty.

Age Determination by Skeleton↗
PubMed2000

LMCE3 treatment strategy: results in 99 consecutively diagnosed stage 4 neuroblastomas in children older than 1 year at diagnosis.

PURPOSE: To tailor postinduction therapy for stage 4 neuroblastoma in children who are older than 1 year at diagnosis according to status after induction. PATIENTS AND METHODS: From March 1987 to December 1992, 99 patients who were consecutively admitted were included in the Lyon-Marseille-Curie East of France (LMCE)3 strategy. After induction with the French Society of Pediatric Oncology NB87 regimen and surgery, patients who were in complete remission immediately proceeded to consolidation therapy with vincristine, melphalan, and fractionated total-body irradiation (VMT). All other patients underwent a postinduction strategy before VMT, either an additional megatherapy regimen or further chemotherapy with etoposide/carboplatin. RESULTS: The progression-free survival (PFS) is 29% at 7 years from diagnosis, which compares favorably with that of a similar cohort of 72 patients previously reported by our group (LMCE1; PFS of 20% at 5 years and 8% at 14 years, P =.004). In the multivariate analysis, only age younger than 3 years at diagnosis (P =.0085) and achievement of complete or very good partial remission after NB87 and surgery (P =.00024) remained significant. The PFS of the 87 patients who were included in the postinduction strategy was significantly better than that of the comparable 62 patients on the LMCE1 study (32% v 11% at 7 years; P =.005). CONCLUSION: The progressive improvements in the LMCE results over the last 10 years suggest that improvements in supportive care measures and increases in each component of this strategy (induction, postinduction, consolidation) may all contribute to increased survival rates.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1999

Molecular analysis of glioma and skin-tumour alterations in a xeroderma-pigmentosum child.

Xeroderma pigmentosum (XP) is a rare hereditary disease characterized by a very high frequency of skin tumours due to a defect in the nucleotide-excision-repair process. Some of these patients have also been reported to develop internal tumours with higher frequency than the normal population. Reported here are the clinical features and molecular analysis of an XP patient who developed multiple skin cancers as well as a thalamic glioma. Complementation analysis with recombinant retrovirus, cloning efficiency and unscheduled DNA synthesis after UV-C indicate that the patient belongs to the C group. Characterization of the p53 mutations in the 2 tumours of the patient leads to speculation on the aetiological agents involved in tumour initiation. The skin tumour is clearly induced by the presence of unrepaired UVB-induced DNA damage on the non-transcribed strand of the p53 gene, while the glioma may be induced by unrepaired DNA lesions produced by free radicals.

Brain Neoplasms↗
PubMed1999

Chemotherapy for intracranial ependymomas.

Chemotherapy has been used extensively in the management of children with intracranial ependymoma. In this review, we discuss the results of phase II studies and clinical trials conducted in newly diagnosed and recurrent ependymomas. There is little evidence that chemotherapy is effective in this tumour. The response rate to single agents is 11%, with less than 5% complete responses, cisplatin being the most active agent in phase II studies. Combinations may be more effective, although the response rate with high-dose regimens is disappointing. Early results of protocols conducted in infants and young children do not suggest that chemotherapy is beneficial. A more rigorous assessment of chemotherapy is required in order to define its role in patients with intracranial ependymomas. Indeed, it is difficult to justify the use of chemotherapy outside such studies. More large studies, perhaps intergroup, limited to children with ependymomas would be of particular value.

Antineoplastic Agents↗
PubMed1999

Combined treatment modality for intracranial germinomas: results of a multicentre SFOP experience. Société Française d'Oncologie Pédiatrique.

Conventional therapy for intracranial germinomas is craniospinal irradiation. In 1990, the Société Française d'Oncologie Pédiatrique initiated a study combining chemotherapy (alternating courses of etoposide-carboplatin and etoposide-ifosfamide for a recommended total of four courses) with 40 Gy local irradiation for patients with localized germinomas. Metastatic patients were allocated to receive low-dose craniospinal radiotherapy. Fifty-seven patients were enrolled between 1990 and 1996. Forty-seven had biopsy-proven germinoma. Biopsy was not performed in ten patients (four had diagnostic tumour markers and in six the neurosurgeon felt biopsy was contraindicated). Fifty-one patients had localized disease, and six leptomeningeal dissemination. Seven patients had bifocal tumour. All but one patient received at least four courses of chemotherapy. Toxicity was mainly haematological. Patients with diabetus insipidus (n = 25) commonly developed electrolyte disturbances during chemotherapy. No patient developed tumour progression during chemotherapy. Fifty patients received local radiotherapy with a median dose of 40 Gy to the initial tumour volume. Six metastatic patients, and one patient with localized disease who stopped chemotherapy due to severe toxicity, received craniospinal radiotherapy. The median follow-up for the group was 42 months. Four patients relapsed 9, 10, 38 and 57 months after diagnosis. Three achieved second complete remission following salvage treatment with chemotherapy alone or chemo-radiotherapy. The estimated 3-year survival probability is 98% (CI: 86.6-99.7%) and the estimated 3-year event-free survival is 96.4% (CI: 86.2-99.1%). This study shows that excellent survival rates can be achieved by combining chemotherapy and local radiotherapy in patients with non-metastatic intracranial germinomas.

Adolescent↗
PubMed1999

Prognostic factors in children with localized malignant nonseminomatous germ cell tumors.

PURPOSE: Prognostic factors were studied in children older than 1 year who were treated with chemotherapy for extracranial localized malignant non seminomatous germ cell tumors. PATIENTS AND METHODS: Data from two consecutive protocols were pooled. The TGM 85 (1985-1989) protocol consisted of alternating courses of cyclophosphamide, dactinomycin and vinblastine, bleomycin, and cisplatin at a dose of 100 mg/m(2) per course. The TGM 90 (1990-1994) protocol was initiated with carboplatin 400 mg/m(2) substituted for cisplatin as the only modification to the previous protocol. RESULTS: We examined alpha-fetoprotein (AFP) levels, disease stage, and primary site and identified three prognostic groups. Patients with a poor prognosis had either an AFP level >/= 10,000 ng/mL or stage III disease and a sacrococcygeal or mediastinal primary site; such patients represented 46% of the patient population and experienced a 43% 3-year failure-free survival rate and a 77% overall survival rate. Patients with a good prognosis had an AFP level less than 10,000 ng/mL, stage I or II disease, and a testicular, ovarian, perineal, or retroperitoneal primary site; such patients represented 22% of the patient population and experienced no treatment failures. The other patients were classified in the intermediate prognosis group and represented 37% of the patient population, with an 81% 3-year failure-free survival rate and a 92% overall survival rate. CONCLUSION: Initial AFP level, disease stage, and primary site are the most important prognostic factors in this analysis. Prognostic models for pediatric germ cell tumors should allow the stratification of patients for a risk-adapted approach to treatment.

Adolescent↗
PubMed1999

[Malignant non-seminomatous germinal tumors of the ovary. Experience of the French Society of Pediatric Oncology. 64 cases].

Ovary seminomatous malignant germ cell tumours are a particular histopathologica entity. The presence of yolk salk tumor or choriocarcinoma is respectively correlated with elevation of alpha FP or beta hCG. This markers elevation permits to assess diagnosis, appreciate response to treatment, and detect relapses. The study of 64 patients registered in two successive S.F.O.P. protocols (TGM 85-TGM 90) precise indications of surgery, platin-based chemotherapy and results. Malignant non seminomatous germ cell tumors are separated in not secreting pure immature teratoma and secreting malignant germ cell tumours. Surgery is essential for treatment of not secreting pure immature teratoma. Secreting germ cell tumors are very chemosensitive and surgery must be as conservative as possible in all cases even metastatic tumour or relapse. If markers are normalized second-look surgery of secreting malignant germ cell tumours is only necessary in case of evident residual tumour. However in case of initial chemotherapy, resection of a pathological ovary is always performed at the end of treatment. These tumours have a good prognosis: 5-years overall survival and disease-free survival are 85%.

Adolescent↗
PubMed1998

Prognostic factors in pediatric spinal cord astrocytoma.

BACKGROUND: Factors that could optimize the management of pediatric spinal cord astrocytoma remain unclear and controversial. METHODS: To determine the factors that influence the prognosis of pediatric patients with spinal cord astrocytomas, a series of 73 consecutive patients at 13 French treatment centers with histologically proven spinal cord astrocytomas was retrospectively reviewed. Hospital records, operative records, and results of radiologic investigations were available in all cases. Follow-up was achieved in 94% of cases. RESULTS: Seventy percent of the patients had low grade (1 or 2) tumors. Total or subtotal surgical resection was achieved in 43%. Thirty-six patients were irradiated following surgery. Fifty-one patients were alive at a median follow-up of 54 months. Twenty-three patients relapsed. Univariate analysis showed that good outcome was correlated with male gender, age younger than 7 years, duration of presenting symptoms longer than 2 months, the presence of spinal deformities, and low grade histology, whereas sensory loss was associated with decreased survival. Multivariate analysis using the Cox proportional hazards model confirmed that histology (relative risk [RR] = 7.69) and the interval between first symptoms and diagnosis (RR = 4.93) were significant independent prognostic factors. The extent of surgery or radiotherapy had no clear influence on survival. CONCLUSIONS: This review sheds light on the prognoses of pediatric patients with spinal cord astrocytomas and may help to determine therapeutic strategies based on patients' clinical, radiologic, and pathologic features.

Adolescent↗
PubMed1998

Central nervous system metastasis in Wilms' tumor: a review of three consecutive United Kingdom trials.

BACKGROUND: Central nervous system (CNS) metastasis is an uncommon event in pediatric oncology, and typically occurs at the end stage of the disease. Previous reports suggest that brain metastases in patients with Wilms' tumor might behave differently. METHODS: This study reviews the data from three consecutive United Kingdom Children's Cancer Study Group trials (UKW 1, 2, and 3) focusing on this entity. RESULTS: Seven children of 1249 (0.6%) entered into Wilms' tumor studies developed CNS metastases between 2-27 months after initial diagnosis. At last follow-up 3 patients still were alive and 4 had died; the mean follow-up from recurrence in the surviving patients was 63 months. Radiotherapy and chemotherapy were administered to all surviving patients. Those patients who died had tumors with particularly aggressive features or extensive disease. CONCLUSIONS: CNS metastasis of Wilms' tumor is not in itself a terminal event. With regard to other sites of recurrence, salvage therapy can be expected to be effective in patients without other adverse prognostic features.

Central Nervous System Neoplasms↗
PubMed1998

Phase II study of cisplatinum and carboplatinum (CACIS) combination in advanced stage neuroblastomas.

BACKGROUND: Platinum derivatives are, among others (cyclophosphamide, etoposide, doxorubicin), the most active drugs in neuroblastomas. As the combination of carboplatin (CBDCA) with cisplatinum (CDDP) was proven effective in some carcinomas, we proposed it as a second-line therapy in neuroblastoma. PROCEDURE: Nineteen children with neuroblastoma and primary refractory disease (seven cases) or relapse either untreated (eight cases) or resistant to second-line therapy (four cases), were treated with cisplatinum and carboplatinum (CACIS) combination. All but one patient had previously received CDDP (median 400: 200 to 1,200 mg/m2) and 15 out of 19 had also received CBDCA (median 1,600:800 to 5,000 mg/m2). Twelve had previously received intensification with megatherapy. The CACIS regimen included CBDCA (100 mg/m2/day as a 1-hour infusion, for 4 days) and simultaneous CDDP (25 mg/m2/day as a 3-hour infusion, for 4 days). RESULTS: Eight out of 19 patients (42%) achieved a partial response with a duration of response of 3 to 12 months (median 6). No patient achieved a complete response. The toxicity was mainly hematological, though one patient died after two courses of an interstitial pneumonia of unknown origin. Only one patient developed alopecia. The renal toxicity was low. CONCLUSIONS: The CACIS regimen is an effective combination of platinum derivatives. It may be proposed as second line protocol, especially for children with neuroblastoma who relapse after megatherapy.

Adolescent↗
PubMed1998

Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation.

BACKGROUND: Current controversies in pediatric intracranial ependymoma include histologic categorization and management. Most of our knowledge of this disease comes from single-institution reports. METHODS: A literature search was done, covering the period 1976-1996. The aim of this review is to analyze the prognostic factors reported in the literature over the last 20 years. RESULTS: Forty-five series were reviewed, including more than 1,400 children. The largest series reported on 92 patients, and the accrual rate ranged from 0.32-12 patients per year. None of the prognostic factors reported achieved a consensus throughout the different series. Histology remains a major issue, and the range in the incidence of anaplastic ependymo mas (7-89%) highlights the difficulty in agreeing on a histological grading system. The role of surgery on the outcome seems to be determinant. Recent series based on homogeneous imaging-documented extents of resection strongly support the benefit of postoperative radiotherapy. The lack of a proven, effective chemotherapy regimen precludes its use except in prospective pilot studies. CONCLUSIONS: Limited information is available from single-institution reports in ependymoma. Only large national or international studies can provide enough information to allow a multivariate analysis of the prognostic factors, and thus lead to new therapeutic proposals.

Brain Neoplasms↗
PubMed1998

Rhabdoid tumor of the kidney associated with a tumor of the posterior fossa.

We report a case of a malignant rhabdoid tumor of the kidney (MRTK) associated with a cerebellar tumor. Diagnosis was confirmed before neoadjuvant chemotherapy by a percutaneous fine-needle biopsy of the abdominal tumor. The clinical and radiologic features of this rare association of childhood neoplasms are reviewed.

Biopsy, Needle↗
PubMed1998

Chemotherapy for spinal cord astrocytoma: can natural history be modified?

Standard treatment of spinal cord astrocytomas is based upon surgery, followed by radiotherapy when resection is incomplete or when histology is of high grade. Owing to the major consequences of radiotherapy on the spine in childhood, alternative therapies must be explored. The potential role of chemotherapy in the management of spinal cord astrocytoma remains to be defined. Two patients are described. The first was a 19-month-old child with an anaplastic astrocytoma of the cervical spinal cord that progressed rapidly after initial partial resection. Chemotherapy was begun according to the UKCCSG Baby Brain Protocol, with marked clinical improvement. Reassessment by MRI at 4 months showed improvement, and at the end of treatment no evaluable disease remained. The second was a 4-year-old child with a recurrent low-grade astrocytoma. Chemotherapy according to the SIOP Protocol for Low Grade Gliomas was administered for 3 months, after which marked tumour regression was seen, with neurological recovery. These patients demonstrate the potential value and low morbidity of chemotherapy in spinal cord astrocytoma. The management of this rare tumour is discussed.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

An open-label, multicentre, randomised phase 2 study of recombinant human granulocyte colony-stimulating factor (filgrastim) as an adjunct to combination chemotherapy in paediatric patients with metastatic neuroblastoma.

Administration of combination chemotherapy to children with metastatic neuroblastoma induces profound myelosuppression resulting in chemotherapy treatment delays and febrile neutropenic episodes. The objective of this randomised multicentre study was to assess the incidence, duration and severity of neutropenia when filgrastim is added to induction chemotherapy administered to patients with metastatic neuroblastoma. In this study, 59 patients with metastatic neuroblastoma were randomised to receive chemotherapy (control group, n = 28) or chemotherapy plus filgrastim (filgrastim group, n = 31). Chemotherapy consisted of four cycles of cyclophosphamide, vincristine and doxorubicin (CADO) alternating at 21-day intervals with cisplatin and etoposide (CDDP-VP16). Filgrastim was administered subcutaneously at a dose of 5 micrograms/kg/day from day 7 for up to 14 days. The incidence of neutropenia (absolute neutrophil count [ANC] < 0.5 x 10(9)/l) in the filgrastim group was not reduced after the first CADO course. However, significant reductions were observed after courses 2, 3 and 4. The duration of neutropenia and of intravenous antibiotic use were significantly reduced in the filgrastim group over the whole study period (9 days versus 26 days, P < 0.001; 12 days versus 20 days, P = 0.04, respectively). However, the duration of hospitalisation and the incidence of febrile neutropenia were not significantly reduced. Compliance to treatment was good and the ability to administer chemotherapy without treatment delays was significantly better in the filgrastim group (P < 0.05). Event-free survival was greater in the filgrastim than in the control group (2.4 years versus 1.3 years; P = 0.072). Filgrastim is a beneficial adjunct to combination induction chemotherapy used in the treatment of metastatic neuroblastoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

An attempt to treat pediatric intracranial alphaFP and betaHCG secreting germ cell tumors with chemotherapy alone. SFOP experience with 18 cases. Société Française d'Oncologie Pédiatrique.

PURPOSE: Intracranial alphaFP and betaHCG secreting germ cell tumors have a very poor prognosis with local treatment alone. Because of efficacy of chemotherapy in extracranial forms, we tried in the SFOP (Société Française d'Oncologie Pédiatrique) to treat them with chemotherapeutic treatment exclusively. PATIENTS AND METHODS: Eighteen patients (10 alphaFP, 2 betaHCG, 6 alphaFP and betaHCG secretion) were enrolled from January 1988 to December 1992. After biological diagnosis patients were to receive 6 cycles of chemotherapy (vinblastine bleomycin - carboplatin or etoposide - carboplatin/ifosfamide - etoposide). After completion of chemotherapy, surgery was to be performed in case of residual tumor. Focal radiation was only to be delivered in case of viable residual tumor. RESULTS: All patients had biological remission and tumor reduction. Fifteen patients were treated according to the protocol by chemotherapy alone (13) or chemotherapy and radiation of residue (2). Twelve of the 13 non irradiated patients relapsed, 8 in local and/or regional area, 3 in cerebrospinal area and 1 in undeterminated area with mild elevation of markers except in one case. Six patients are alive in second complete remission after chemotherapy and/or surgery and then a consolidation treatment with radiation and/or high dose chemotherapy (5) or craniospinal radiation (1). Three patients received radiation after 2 or 3 cycles of chemotherapy as protocol violations and didn't relapse. Thus, 12 patients out of the 18 patients are alive with a median follow up of 68 months. All but 1 had focal radiation as part of treatment. No toxic death was observed. CONCLUSION: Although survival rate is noteworthy (66%), these tumors were not curable with this conventional chemotherapy alone and focal radiotherapy should be part of the treatment.

Adolescent↗
PubMed1998

Improving survival in recurrent medulloblastoma: earlier detection, better treatment or still an impasse?

Early detection of relapse has been advocated to improve survival in children with recurrent medulloblastoma. However, the prognostic factors and the longer term outcome of these patients remains unclear. Pattern of recurrences were analysed in three consecutive protocols of the Société Française d'Oncologie Pédiatrique (1985-91). A uniform surveillance programme including repeated lumbar puncture combined with computerized tomography (CT) or magnetic resonance imaging (MRI) scan was applied for all registered patients. Forty-six out of 116 patients had progressive or recurrent disease. The median time from diagnosis to recurrence was 10.5 months and 76% relapses occurred during the first 2 years. Seventeen patients had asymptomatic relapses that were detected by the surveillance protocol. Forty-one patients were treated at time of progression. Twenty-three responded to salvage therapy and 11 achieved a second complete remission. The median survival time after progression was 5 months (<1-41 months), and only two patients remained alive at time of follow-up. Length of survival is primarily related to some specific patterns of relapse (time from diagnosis to recurrence, circumstances of relapse, extent of relapse) and to the response to salvage therapy. No evidence of long-term benefit appeared from any form of treatment.

Adolescent↗