Biomedical subjects

M Brunat-Mentigny

Publications and source records attributed to M Brunat-Mentigny.

At least 19 recordsLinked to original sources

PubMed2001

Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years.

The presence of multifocal or diffuse nephrogenic rests (NRs) in one or both kidneys is termed nephroblastomatosis (Nbm). Nbm may be a predisposing factor for Wilms' tumour (WT). The aim of this retrospective study was to evaluate the impact of Nbm on the outcome of WT in children. We assessed the outcome of 81 children with Wilms tumours and practical implications of Nbm in the treatment and follow-up. All the pathology slides have been reviewed in 1997. 63 had WT without Nbm (group A) and 18 had WT associated with Nbm (group B). There was no statistical difference between the two groups according to the age at diagnosis and histology. Clinical abnormalities were more frequent in group B (33 versus 8%). There was no statistical difference between the percentage of stage IV in both groups, but bilaterality (stage V) was present only in the group B. Relapse was observed in 20/81 patients (25%): 11 (17%) in group A and 9 (50%) in group B. Mean delay of relapse was longer (25 months) in group B than in group A (10 months). For the whole population, with a median follow-up of 9 years, the event-free survival (EFS) and the overall survival (OS) probabilities were respectively 74%+/-10 and 83%+/-9 at 120 months. The difference in EFS between groups A (82+/-9%) and B (38%+/-29) was significant (P=0.004). The discovery of Nbm in the non-tumoral part of the kidney with WT can be an adverse factor and in particular favours the subsequent development of a new Wilms tumour. It justifies separate follow-up guidelines.

Adolescent↗

PubMed2001

Osteosarcoma.

Explore the source record for details and available documents.

Bone Neoplasms↗

PubMed1999

High-dose methotrexate and HELP [Holoxan (ifosfamide), eldesine (vindesine), platinum]--doxorubicin in non-metastatic osteosarcoma of the extremity: a French multicentre pilot study. Fédération Nationale des Centres de Lutte contre le Cancer and Société Française d'Oncologie Pédiatrique.

UNLABELLED: This study evaluates histological response, long-term outcome, and toxicity in an intensive chemotherapy program given before surgery. PATIENTS AND METHODS: Sixty-two patients (39 males, 23 females: median age 14) with biopsy, chest computerised-tomography, technetium bone-scan and magnetic resonance imaging, were enrolled. Primary localisations were femur (44%) and tibia (26%). Induction chemotherapy involved seven courses of high-dose methotrexate and two courses of HELP (ifosfamide, eldesine (vindesine), cisplatin (platinum)-doxorubicin. After surgery, patients received six courses of high-dose methotrexate and two courses of HELP-doxorubicin. RESULTS: Pre- and postoperative toxicities were similar. Fifty-nine patients underwent surgery; histological response was good in thirty-eight patients (64%) and poor in twenty-one (36%). Median follow-up is 57 months (range 30-80), with 77% overall survival and 59% progression-free survival. In a multivariate analysis, age under 10 years is the only prognostic factor that significantly correlates with outcome. CONCLUSIONS: This regimen appears to increase histological necrosis, but associates with severe toxicity. Results for patients with less necrosis at surgery are encouraging. Future trials should determine the minimum effective doses to reduce toxicity. New drugs should be added.

Adolescent↗

PubMed1999

Outcome of radiation-related osteosarcoma after treatment of childhood and adolescent cancer: a study of 23 cases.

PURPOSE: We analyzed the clinical features and outcome of patients with radiation-associated osteosarcoma treated during the era of contemporary chemotherapy. PATIENTS AND METHODS: The characteristics and outcome of 23 patients (17 males and six females) treated during childhood or adolescence for a solid tumor who later developed osteosarcomas within the radiation field between 1981 and 1996 were reviewed. RESULTS: The median dose of radiation delivered to the first cancer was 47 Gy. Nineteen patients also received chemotherapy. The median time between radiotherapy and the diagnosis of secondary osteosarcoma was 8 years. Histologic slide review showed conventional central osteosarcoma with various differentiation patterns in 21 cases, together with one case of high-grade surface osteosarcoma and one of periosteal osteosarcoma. The sites of involvement were the craniofacial bones in six cases, the first cervical vertebra in one, the girdle bones in seven, and the extremities of long bones in nine. Three patients had metastatic disease at the diagnosis of osteosarcoma. Palliative therapy was administered to seven patients. The aim of treatment was curative for 16 patients, two of whom underwent amputation without further therapy. Intensive chemotherapy regimens were administered to 14 patients before and/or after surgery. Fifteen patients achieved complete surgical remission. Twelve patients were alive and disease-free at a median follow-up duration of 7.5 years. Overall and event-free survivals at 8 years were 50% and 41%, respectively. CONCLUSION: Patients with radiation-related osteosarcoma and resectable lesions can be cured with surgery and intensive preoperative and postoperative chemotherapy.

Adolescent↗

PubMed1999

[Standards, options and recommendations (SOR) for diagnosis, treatment and follow-up of osteosarcoma. Groupe de travail SOR].

CONTEXT: The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. For pediatric issues, this project is a collaboration between the FNCLCC and the French Society of Pediatric Oncology (SFOP). The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery. OBJECTIVES: To develop clinical practice guidelines according to the definitions of Standards, Options and Recommendations for the clinical care of osteosarcoma in children and adult. METHODS: Data have been identified by literature search using Medline (1985-december 1998) and the expert groups personal reference lists. The main criteria considered were incidence, risk factors, prognostic factors and efficacy of treatment. Once the guidelines were defined, the document was submitted for review to 27 national and international independent reviewers, and to the medical committees of the 20 French Cancer Centres and, in particular, the 4 which have particular expertise in pediatric cancer management. RESULTS: The main recommendations for osteosarcoma management are that: 1) the clinical diagnosis is based on appropriate clinical and radiological findings; 2) the final diagnosis is pathological and the biopsy should be performed by the surgeon who will subsequently perform the definitive surgery; 3) surgical biopsy must be of adequate size and performed by an experienced surgeon; 4) the therapeutic strategy for osteosarcoma is based on surgery with neoadjuvant and adjuvant chemotherapy given in experienced centres. Inclusion of high dose methotrexate is recommended for children, and the dose of methotrexate must be adapted for adults. Inclusion of children in SFOP protocols and adults in EORTC and FNCLCC clinical trials is recommended; 5) treatment of metastatic osteosarcoma is based on chemotherapy and surgery to lung metastases which may be curative. Amputation is rarely appropriate. Inclusion of children in SFOP and of adult in EORTC and FNCLCC clinical trials for metastatic osteosarcoma is recommended; 6) at the present time, there are no clear data on which to base guidelines for timing and duration of follow-up studies in this condition.

Antineoplastic Agents↗

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

Long-term nephrotoxicity of cisplatin, ifosfamide, and methotrexate in osteosarcoma.

The acute renal effects of chemotherapy are known, but long-term nephrotoxicity has rarely been investigated. The aim of the present study was to assess long-term renal function in children and adolescents who received at-risk chemotherapy, including cisplatin, ifosfamide, and methotrexate, to treat an osteosarcoma. Renal function tests [creatinine clearance, microalbuminuria, and renal excretion of sodium, potassium, chloride, calcium, magnesium (Mg), phosphorus (P), and uric acid] were prospectively performed 5.4+/-2.2 (+/-SD) years after chemotherapy (total cumulative dose: methotrexate 41+/-31 g/m2, ifosfamide 39+/-14 g/m2, cisplatin 674+/-188 mg/m2) in 18 children and adolescents. The results were compared with 13 normal volunteers matched for age and sex. Creatinine clearance, which was greater than 80 ml/min per 1.73 m2 in all patients, correlated with the total dose of ifosfamide (r=0.55, P<0.05) and cisplatin (r=0.48, P<0.05). Microalbuminuria was noted in 4 patients. Hypomagnesemia was present in 4 and hypercalciuria in 3 patients; renal excretion of P, Mg, and uric acid was higher in patients than in controls. Glomerular function was not significantly altered and only mild tubular dysfunction was present. Since renal excretion of P and Mg were increased in patients compared with normal volunteers and hypercalciuria was occasionally seen, divalent ion disorders are the most-likely potential complications.

Adolescent↗

PubMed1998

Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84.

The second International Society of Paediatric Oncology (SIOP) study for rhabdomyosarcoma (MMT84) had several goals. The two principal aims were: (1) to improve the survival of children with rhabdomyosarcoma; and (2) to reduce the late effects from therapy by restricting the indications for surgery and/or radiotherapy after good response to initial chemotherapy. A further aim was to investigate the role of high-dose chemotherapy in young patients with parameningeal primary tumours. 186 previously untreated eligible patients entered the study. Patients with completely resected primary tumour received three courses of IVA (ifosfamide, vincristine and actinomycin D). Patients with incompletely resected tumour received six to 10 courses of IVA according to stage. Patients achieving complete remission with chemotherapy alone did not usually receive radiotherapy or undergo extensive surgery, but patients remaining in partial remission received local therapy with surgery and/or radiotherapy. Only patients over 5 years of age with parameningeal disease and patients over 12 years with tumours at any site were given systematic irradiation. Complete remission was achieved in 91% (170/186) of all patients. With a median follow-up of 8 years, the 5-year overall survival was 68% (+/- 3% standard error of the mean (SEM) and the 5-year event-free survival 53% (+/- 4% SEM). These results show an improvement over previous SIOP study (RMS75) in which survival was 52% and event-free survival was 47%. Among the 54 patients who exhibited isolated local relapse, 35% (19/54) survived in further remission longer than 2 years after retreatment, including local therapy (surgery +/- radiotherapy). Analysis of the overall burden of therapy received by all surviving children (including primary treatment and treatment for relapse if required) showed that 24% (28/116) were treated by limited surgery followed by three courses of IVA, 29% (34/116) were treated by chemotherapy alone (after initial biopsy) and 13% (15/116) received chemotherapy plus conservative local treatment (limited surgery or radiotherapy for residual disease). Only 34% (39/116) received intensive local therapy defined as radical wide field radiotherapy or radical surgery or both. Compared with the results obtained in the previous SIOP study, treatment in MMT84 was based on response to initial chemotherapy and, despite an overall reduction of the use of local therapy, significantly improved survival for patients with non-metastatic disease. This trial, also for the first time, provides evidence that retreatment after local relapse can achieve long-term second remissions.

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↗

PubMed1998

[Standards, options and recommendations (SOR) for clinical care of rhabdomyosarcoma (RMS) and other soft tissue sarcoma in children. Federation of the French Cancer Centers. French Society of Pediatric Oncology].

CONTEXT: The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. For pediatric issues, this project is a collaboration between the FNCLCC and the French Society of Pediatric Oncology (SFOP). The main objective is the development of clinical practice guidelines to improve the quality of health care and outcomes for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery. OBJECTIVES: To develop a clinical practice guideline according to the definitions of Standards, Options and Recommendations for the clinical care of rhabdomyosarcoma and other soft tissue sarcoma in children and adolescents. METHODS: Data have been identified by literature search using Medline (1985-may 1997) and experts group personal references lists. The main criteria considered were incidence, risk factors, prognostic factors and efficacy of cancer treatment. Once the guideline was defined, the document was submitted for review to 14 national and international independent reviewers, and to the medical committees of the 20 French Cancer Centres and, in particular the 4 which have expertise in pediatric cancer management, for agreement. RESULTS: The main recommendations for rhabdomyosarcoma management are: 1/ diagnosis is based on appropriate clinical and radiological findings; 2/ pathological and immunohistochemical studies are essential to confirm the diagnosis; 3/ surgery must be performed by an experienced surgeon. Surgery and radiotherapy must be as conservative as possible; 4/ therapeutic strategies for rhabdomyosarcoma depend on location and extends and are based on chemotherapy, surgery and radiotherapy. Inclusion of patients in SFOP, SIOP and IRS clinical trials is recommended; 5/ treatment of metastatic rhabdomyosarcoma is based on intensive chemotherapy, and surgery with or without radiotherapy; 6/ the management of non-rhabdomyosarcoma is based on the likelihood of sensitivity to chemotherapy; 7/ at the present time, there are no clear data on which to base guidelines for timing and duration of follow-up studies in these conditions.

Child↗

PubMed1997

Brain metastases in children with solid tumors.

BACKGROUND: Brain metastases are uncommon among children with solid tumors. However, improvements in survival have increased the period of time during which children are at risk for developing these metastases. The authors reviewed brain metastases in children with solid tumors treated at the Centre Léon Bérard during the 9 years between 1987 and 1995. METHODS: Among 486 patients with solid tumors, 162 eventually developed distant metastases in their disease process, including 12 brain metastases detected by imaging. The tumor type, clinical setting, imaging characteristics, treatment modalities, and outcome were assessed for each patient. RESULTS: The most common tumors causing brain metastases were Ewing's sarcoma (in three patients), neuroblastoma (in three patients), and osteogenic sarcoma (in three patients). At the time of initial diagnosis, 9 of the 12 patients had metastatic disease. All but one patient initially received intensive multiagent chemotherapy, including high dose chemotherapy with bone marrow rescue in six patients. The median time from initial diagnosis to the detection of brain metastases was 15 months. These metastases were clinically detectable in 10 patients and subclinical in 2 patients. Brain metastases were present at the time of first relapse in five patients. In two patients, the brain was the only site of relapse. All other patients had extensive systemic disease. Seven patients had multiple brain metastases. Two children underwent surgical resection of solitary metastases, and eight were irradiated. One child achieved complete remission following chemotherapy and irradiation. All other children died, mostly of their systemic disease, within a median period of 3 months. CONCLUSIONS: The introduction of effective systemic chemotherapy has changed the patterns of brain metastases in children. The increasing incidence of these metastases in patients with sarcoma and neuroblastoma suggests that the brain is a pharmacologically protected site in patients initially diagnosed with metastatic disease.

Adolescent↗

PubMed1997

Screening for neuroblastoma in France: methodological aspects and preliminary observations.

A pilot study of neuroblastoma mass screening was initiated in January 1990 in the Rhône French district. The expected number of births per year is 26,000. The study is designed for a 5-year period with three major goals: 1) measurement of the compliance rate of a voluntary test at 4 months of age; 2) evaluation of the technical value of high-pressure liquid chromatography (HPLC) as a screening method; and 3) detailed biological characterization of all detected tumors. 61,551 children were screened between May 1, 1990 and December 31, 1993. Participation was 69% in 1990, 81.5% in 1991, and over 83% in 1992. HPLC was a satisfactory assay method. The number of clinical examinations required for positive tests as defined in the protocol is 1 per 3,621 tests. The false positive rate is 1 per 3,583 tests. Eight neuroblastomas were discovered by-screening (one stage I, three stage II, one stage III, three stage IVs). All are alive and well but were good prognosis cases according to the main prognostic factors. Five patients were discovered before screening (so called Halo effect): one stage I, one stage III, three stage IVs. One died of disease and four are alive in complete remission after treatment. Two patients were false negative (one stage III with N-myc amplification, one stage IV with bad prognosis features) and three cases of neuroblastoma were missed because of noncompliance with the screening program. This pilot study concludes on the feasibility of a mass screening program in France. The estimated cumulative incidence of neuroblastoma at 3 years is 1 per 4,375 living births and overdiagnosis is probable. All the detected cases were of good prognosis and the false negative ones were poor prognosis cases.

Child, Preschool↗

PubMed1997

High-dose carmustine for high-grade gliomas in childhood.

Carmustine (BCNU) has proved to be of value against a variety of primary brain tumors. This agent exhibits a steep dose-response curve in in vitro and animal tumor models and has been proposed for use in high-dose chemotherapy as a single agent or in combination. We conducted a phase II study to assess high-dose BCNU in children with high-grade gliomas. A total of 13 children with high-grade gliomas were treated in a phase II study using high-dose BCNU (800 mg/m2) followed by autologous bone marrow transplantation. Eight patients were newly diagnosed, and five were treated at the time of tumor recurrence. Seven patients had diffuse intrinsic brain-stem gliomas. The response was assessed at 1 month after treatment. Only one objective effect was observed. Five patients had stable disease and seven progressed. The immediate toxicity was mild; however, one patient developed fatal respiratory distress at 50 days after treatment with high-dose BCNU. Dose escalation of BCNU does not seem beneficial in children with high-grade gliomas.

Adolescent↗

PubMed1997

Spinal cord compression by secondary epi- and intradural metastases in childhood.

Over a 9-year period, 35 out of 614 children with malignant tumours who were treated at the Centre Léon Bérard developed spinal metastases. Of these, 18 with known malignancies before the development of spinal cord compression are reviewed. The most common tumours causing spinal metastases were Ewing's sarcoma, neuroblastoma and renal tumours. Cord compression occurred 5-88 months after the diagnosis of systemic cancer. The median interval from first symptoms to the diagnosis of compression was 17 days. There were 16 patients with neurological deficit, including 5 with paraplegia. Specific imaging procedures were performed in 16 patients. Treatment included operation in 8 patients, followed by chemotherapy (6 patients) and/or radiotherapy (4 patients); 9 of the 10 non-operated patients received radiotherapy. Only 6 patients had a significant neurological improvement. All patients but 1 died within a median time of 2 months. Early diagnosis might prevent permanent disability in these children with a short survival expectancy.

Adolescent↗

PubMed1997

Nephrotic syndrome and Hodgkin disease in children: a report of five cases.

UNLABELLED: This report documents the occurrence of a nephrotic syndrome in five children with Hodgkin disease. In two cases the nephrotic syndrome predated the diagnosis of lymphoma by 6 months and 12 months respectively, while in the other three, the two disorders occurred simultaneously. The nephrotic syndrome resolved in four cases during effective treatment for active Hodgkin disease, while proteinuria remained unchanged in the fifth case with partial control of the lymphoma. The occurrence of a nephrotic syndrome as a manifestation of active Hodgkin disease suggests that some immunological abnormalities play a role in the pathogenesis of the association. CONCLUSION: The possibility of glomerular dysfunction although rare must be considered and actively looked for in all cases of Hodgkin disease. Similarly, any unusual sign or symptom noted in patients with nephrotic syndrome, particularly receiving or having received immunosuppressants, requires thorough investigation to determine the presence or absence of lymphoma.

Adolescent↗

PubMed1997

Juvenile granulosa cell tumor of the ovary in infants: a clinicopathologic study of three cases and review of the literature.

The clinical and pathological features of three cases of juvenile granulosa cell tumors occurring in infants were studied. Precocious pseudopuberty developed in two patients and acute abdominal symptoms related to the rupture of the tumor developed in one. Surgery was the only treatment in each case and no adjuvant therapy was delivered. No patient experienced relapse. Histological examination showed a predominantly diffuse pattern with prominent luteinization. Call-Exner bodies were absent. Two tumors had multilocular thin walled cysts containing large amounts of estradiol, the third one contained rudimentary microfollicles. The prognosis of juvenile granulosa cell tumors in infancy appears more favorable than those occurring in older patients. No case of tumor recurrence has been reported in infancy so far. Surgery appears to be the state-of-the-art treatment of these tumors and additional therapy (chemotherapy or radiotherapy) must be discussed with caution, even in advanced stages.

Female↗

PubMed1997

[Renal function after nephrectomy for Wilms' tumor].

BACKGROUND: Most children with Wilms tumour recover after nephrectomy, chemotherapy and sometimes radiotherapy. It is therefore important to assess their long-term renal function. POPULATION AND METHODS: Thirty-three patients with Wilms tumour experienced unilateral nephrectomy between 1986 and 1993: three were excluded; 23 were staged as grade I, one at grade II, two at grade III and four at grade IV. They were treated with SIOP 6 and SIOP 9 protocols. The results were compared to five controls who underwent unilateral nephrectomy including three for renal trauma. The glomerular filtration rate (GFR) was measured by inulin clearance and the renal plasma flow (RPF) by para-amino-hippuric acid clearance. RESULTS: The mean age at nephrectomy was 3.4 +/- 2.5 years (median: 3, range: 0.2-10.6) and the duration of follow-up was 4.6 +/- 3.1 years (median: 4.5, range: 1-8.5), the GFR was 93 +/- 13 mL/min/1.73 m2 (median: 93, range: 73-130), the RPF was 441 +/- 85 mL/min/1.73 m2 (median: 453, range: 236-650) and the filtrated fraction (FF) was 0.21 +/- 0.03 (median: 0.20, range: 0.18-0.31). The difference in renal function between patients and controls was not significant (GRF: 86 +/- 12 mL/min/1.73 m2, RPF: 486 +/- 185 mL/min/1.73 m2, FF: 0.22 +/- 0.03). The electrolyte reabsorption rate was normal and none of the patients suffered from arterial hypertension. Fourteen children had urinary albumin: creatinine ratio > 2 g/mol. When comparing patients according to the duration of follow-up after nephrectomy (< 4 years vs > 4 years), the renal function was not statistically different. The age at nephrectomy (< 2 years vs > 2 years) did not increase the risk of renal impairment. CONCLUSION: Children with Wilms tumour who were treated with nephrectomy and non-nephrotoxic drugs (actinomycin, vincristine, epiadriamycin) have a good long-term renal outcome. It is speculated that systematic renal investigation should be limited to those children with increased microalbuminuria and/or elevated blood pressure.

Analysis of Variance↗

PubMed1997

Etoposide and thiotepa followed by ABMT (autologous bone marrow transplantation) in children and young adults with high-grade gliomas.

The addition of conventional chemotherapy to irradiation has improved the outcome of children with malignant gliomas. The application of high-dose chemotherapy has been proposed as a possible way to increase the response rate and thus the survival in children with malignant brain tumours. High-dose etoposide (500 mg/m2/day x 3) and thiotepa (300 mg/m2/day x 3) followed by bone marrow transplantation were given to 22 patients (age range 4-20 years) with newly diagnosed or recurrent high-grade glioma. The response rate in the 14 assessable patients was 29% with one complete and three partial responses. 5 patients had stable disease, and 5 progressive disease. 2 patients died of treatment-related toxicity. Only 3 patients remain alive disease free 54, 60 and 65 months after high-dose therapy. For children with high-grade gliomas, survival using high-dose chemotherapy is no better than that reported with conventional treatments.

Adolescent↗