Biomedical subjects

Didier Frappaz

Publications and source records attributed to Didier Frappaz.

11 recordsLinked to original sources

PubMed2008

Osteosarcoma metastatic to the kidney and iatrogenic hemorrhage.

A 16-year old female presented painful masses in the lumbar region 5 years after the initial diagnosis of a localized osteosarcoma of the tibia. Abdominal X-ray revealed calcified masses. A bone scan confirmed an increased uptake in the renal areas. An ultrasound-guided fine needle biopsy confirmed the diagnosis of metastases. The procedure was complicated by subcapsular hemorrhage and gross hematuria. Renal metastases from osteosarcoma are usually asymptomatic and invisible on abdominal X-rays. The diagnosis is generally established by radionuclide bone scan or abdominal CT-scan. Our observation suggests that histological documentation of these unusual clinical presentations should be carefully weighed against the risk of the procedure.

Amputation, Surgical↗

PubMed2006

Very late frontal relapse of medulloblastoma mimicking a meningioma in an adult: usefulness of 1H magnetic resonance spectroscopy and diffusion-perfusion magnetic resonance imaging for preoperative diagnosis: case report.

OBJECTIVE AND IMPORTANCE: We present a rare case of very long-term medulloblastoma relapse in an adult patient and discuss the pattern of recurrence and metabolic imaging of the tumor. CLINICAL PRESENTATION: A 45-year-old man was referred for evaluation of a frontobasal midline tumor 21 years after treatment of a cerebellar medulloblastoma by surgery followed by chemotherapy and craniospinal radiotherapy. Magnetic resonance images were suggestive of a meningioma. Several hypotheses were discussed, such as other radio-induced tumors, sarcomas, high-grade gliomas, or lymphomas (previous chemotherapy) and even recurrence of medulloblastoma. Preoperative exploration included H magnetic resonance single-voxel spectroscopy (35 and 135 ms echo time), diffusion imaging, and perfusion magnetic resonance imaging. INTERVENTION: On magnetic resonance spectroscopy, N-acetyl-aspartate and an elevated choline/creatine ratio were retrieved, with a huge unidentified peak at 1.27 parts per million (ppm). Myoinositol signal was present at both echo times. On diffusion imaging, the tumor appeared hyperintense, with a low apparent diffusion coefficient value of 0.689. In the perfusion study, the maximal relative cerebral blood volume was 2. Metabolic imaging favored the diagnosis of medulloblastoma over the initially suspected diagnosis of meningioma. The patient underwent complete removal of the tumor that was confirmed to be a metastasis of his primary medulloblastoma. The postoperative course was uneventful, and complementary courses of radiotherapy and chemotherapy were planned. CONCLUSION: Late relapse should be considered, even after several decades, on occurrence of a second intracranial tumor in this context. Our observation validates the clinical interest of preoperative metabolic imaging for brain tumors with distinctive pattern.

Cerebellar Neoplasms↗

PubMed2006

Complete removal of an intraspinal and extraspinal cervical chordoma in one stage using the lateral approach. Technical note.

The authors describe a precise surgical technique in which a large intraspinal and extraspinal, multivertebral, cervical chordoma was completely removed in one stage using the lateral approach. The patient in this case was a 29-year-old woman who presented with signs of radicular pain in the left C-3 area. Computed tomography and magnetic resonance imaging demonstrated a large intra- and extraspinal multivertebral tumor from C-2 to C-5, a finding that suggested a cervical chordoma. The tumor was completely removed in one stage using the lateral approach while controlling the vertebral artery (VA), and a partial corporectomy of C2-5 was also performed. Results from a postoperative histopathological examination confirmed that the tumor was a typical chordoma. The patient's postoperative course was uneventful. Cervical chordomas are typically excised using a posterior-anterior surgical approach with partial resection of the tumor. The lateral approach was appropriate in this patient for complete resection in one stage, because it enabled the surgeons to control the VA and access both extraspinal and intraspinal components of the chordoma.

Adult↗

PubMed2006

[Immunotherapy of poor-prognosis neuroblastoma in children: from bench to bedside].

During the last two decades, improvements in the induction and consolidation treatment phases in patients with high-risk neuroblastoma have not translated into significant increases in survival rates. Efforts to improve outcome have used high-dose chemotherapy with stem cell rescue and more recently, differentiating (retinoids) and antiangiogenic agents. In parallel, immunotherapy has become an increasingly important part of the treatment of high-risk neuroblastoma. We review here the biological concepts underlying these new approaches and their clinical applications, with a particular emphasis on applications that manipulate the immune system, including monoclonal antibodies, gene-modified tumor cells (vaccines) or immune effectors.

Child↗

PubMed2006

[An interactive and evolutive software program to personalize treatment schedule : implication on daily life: the Centre Léon Bérard experience].

The second plan against cancer, initiated by the French President in 2003, has introduced a two-step procedure to announce cancer to the patient. During initial visit, the doctor tells the patient about his disease. On the second visit, the doctor elaborates the treatment strategy and proposes an individualized treatment plan for each patient. The objective of this plan is to offer to patients and caregivers an accurate, precise and personalized treatment schedule. The Centre Léon Bérard (Regional Cancer Center) has implemented a computerized model to manage all different treatment schedules. We developed a software program based on two different steps. Firstly, standard treatment schedules are programmed for each type of disease. To offer a better view of the influence of treatment on daily life, we added information such as place of treatment (ambulatory, inpatient, outpatient) impact on well being (side effects, risk of aplasia...), future evaluations and medical acts (CT scan, MRI, lumbar puncture...) as well as actions the patient should take (blood cell count, to have eaten nothing...). Secondly, it tailors these standard treatments as well as all complementary information's to the specific needs of each patient, based on the medical information available in their computerized records. This personalized plan may be modified and adjusted at any time including therefore more and more real details and insights for each patient (delay of chemotherapy, dates of CT scan....). This would help patients and caregivers to better understand the different phases of the treatment and, thus, improve patient follow-up and information at every step of patient management. This tool is currently being tested at the Centre Léon Bérard.

Case Management↗

PubMed2005

Treatment of medulloblastoma with postoperative chemotherapy alone: an SFOP prospective trial in young children.

BACKGROUND: Morbidity and mortality are high in young children with medulloblastoma who receive craniospinal radiotherapy. We aimed to assess whether adjuvant treatment with protracted chemotherapy alone could replace radiotherapy. METHODS: We enrolled 79 children aged younger than 5 years who had had surgical resection of medulloblastoma onto a multicentre trial. Patients were treated with combination chemotherapy, which did not include methotrexate, for more than 16 months irrespective of the extent of disease. Early postoperative imaging defined three groups: R0M0 (no residual disease, no metastasis), R1M0 (radiological residual disease alone), and RXM+ (presence of metastases). Patients who did not relapse did not receive radiotherapy. Patients who relapsed or had disease progression received salvage treatment, which consisted of high-dose chemotherapy and stem-cell transplantation followed by local or craniospinal radiotherapy. For children classified as R0M0, the primary endpoint was 5-year overall survival and the secondary endpoint was 5-year progression-free survival. For children classified as R1M0 or RXM+, the primary endpoint was best radiological response and the secondary endpoints were 5-year overall survival and 5-year progression-free survival. Analyses were done by intention to treat. FINDINGS: Two of 15 patients classified as RXM+ and four of 17 patients classified as R1M0 had a complete radiological response. 5-year progression-free survival was 29% (95% CI 18-44) in the R0M0 group, 6% (1-27) in the R1M0 group, and 13% (4-38) in the RXM+ group. 5-year overall survival was 73% (59-84) in the R0M0 group, 41% (22-64) in the R1M0 group, and 13% (4-38) in the RXM+ group. In the R0M0 group, 5-year progression-free survival was 41% (26-58) for the 34 patients who underwent gross total resection compared with 0% for the 13 patients who had subtotal resection (relative risk 2.7 [1.3-5.6], p=0.0065). INTERPRETATION: Conventional chemotherapy alone can be used to cure children with non-metastatic medulloblastoma who have gross total resection confirmed by early radiological assessment, but is not sufficient for treatment of those with metastatic or incompletely resected medulloblastoma. Salvage treatment followed by posterior-fossa radiotherapy can effectively treat local relapses or progression.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2004

Endocrine involvement in pediatric-onset Langerhans' cell histiocytosis: a population-based study.

OBJECTIVE: To document the frequency and outcome of endocrine involvement in pediatric-onset Langerhans' cell histiocytosis (LCH), and the association with other types of organ involvement. STUDY DESIGN: This retrospective nationwide multicenter study involved 589 patients with pediatric-onset LCH, 148 of whom had endocrine dysfunction. Median follow-up was 11.6 years. RESULTS: Pituitary dysfunction was present in 145 patients, and 141 had diabetes insipidus (DI). The estimated 10-year risks of pituitary involvement were 24.2% +/- 1.8%. GH deficiency occurred in 61 patients. Median age at onset was 2.8 years for LCH, 3.9 years for DI, and 7.7 years for GH deficiency. The risk of cranial involvement; ear, nose, and throat involvement; pneumothorax; and cholangitis was significantly higher in patients with endocrinopathy. The chronology of episodes did not support a causal link between pituitary involvement and involvement of other organs. Systemic treatment of LCH did not prevent pituitary involvement. The most severe complication was a neurodegenerative syndrome, which affected 4.3% and 10.8% of patients, respectively, 5 and 15 years after initial diagnosis, and appeared to be linked to pituitary involvement. CONCLUSION: Patients who develop endocrine LCH disorders are at a high risk of neurodegenerative LCH and require long-term follow-up.

Adolescent↗

PubMed2004

Diffuse medulla oblongata and pontine gliomas in childhood. A review of 37 cases.

From 1975 to 1997, thirty seven newly diagnosed children aged 2 mos. to 14.3 years with diffuse medulla oblongata or pontine tumours were referred to the Centre Léon Bérard. Surgical biopsies were performed in 9 patients. All but one received radiation therapy. The mean dose of radiation was 53 Gy. Thirty two received chemotherapy. All patients died, one from related toxicity, and the rest from progressive disease. Relapses always occurred in the radiotherapy field. Medulla oblongata and pontine tumours would appear to have the worst outcome of all brain stem gliomas and should be separately analysed.

Adolescent↗

PubMed2003

Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy--results of the first French prospective study for the French Society of Pediatric Oncology.

PURPOSE: To evaluate a strategy aimed at avoiding radiotherapy during first-line treatment of children with progressive optic pathway tumors (OPT), by exclusively administering multiagent chemotherapy during 16 months. PATIENTS AND METHODS: Between 1990 and 1998, 85 children with progressive OPT were enrolled onto this multicenter nationwide trial. Chemotherapy alternating procarbazine plus carboplatin, etoposide plus cisplatin, and vincristine plus cyclophosphamide was given every 3 weeks. At the time of relapse or progression, second-line chemotherapy was authorized before recourse to radiotherapy. RESULTS: Objective response rate (partial response [PR] + complete response [CR]) to chemotherapy was 42%. Five-year progression-free survival (PFS) and overall survival rates were 34% and 89%, respectively. The 5-year radiotherapy-free survival rate was 61%. In the multivariate analysis of the 85 patients that entered onto the study, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.047) and absence of neurofibromatosis type 1 (P =.035). In the multivariate analysis of the 74 patients that remained on study after the first cycle of chemotherapy, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.0053) and no objective response to chemotherapy (P =.0029). Three-year PFS was 44% in infants < or = 1 year versus 66% in children older than 1 year. Three-year PFS was 53% in the absence of an objective response to chemotherapy versus 68% after a PR or CR. CONCLUSION: A significant proportion of children with OPT can avoid radiotherapy after prolonged chemotherapy. Deferring irradiation with chemotherapy protocols did not compromise overall survival of the entire population or visual function.

Age Factors↗

PubMed2003

A phase I study of irinotecan as a 3-week schedule in children with refractory or recurrent solid tumors.

PURPOSE: A phase I study was performed to determine the maximum-tolerated dose (MTD) and safety profile of irinotecan (CPT-11) administered as a single intravenous infusion every 3 weeks in children with recurrent or refractory solid tumors. PATIENTS AND METHODS: Eighty-one patients were enrolled, including 48 less heavily, and 33 heavily pretreated patients (cranial irradiation and/or high-dose chemotherapy). Children received CPT-11 as a 120-minute infusion at doses ranging from 200 to 720 mg/m2. The dose-limiting toxicities (DLT) on first cycle were determined in both cohorts. RESULTS: One hundred twenty-two cycles and 81 cycles were administered in less heavily, and heavily pretreated patients, respectively. The primary DLT was delayed diarrhea in less heavily pretreated patients, and neutropenia in heavily pretreated patients. MTD was 600 mg/m2 in both cohorts. Grade 3 to 4 neutropenia occurred in 33% and 38% of cycles in less heavily, and heavily pretreated patients, respectively. Grade 3 to 4 nonhematologic toxicities included nausea/vomiting (7% and 4% of cycles in less heavily, and heavily pretreated patients, respectively), asthenia (7% and 4% of cycles, respectively), and delayed diarrhea (6% and 2.5% of cycles, respectively). Four partial responses at 600 mg/m2 (high-grade glioma, neuroblastoma, medulloblastoma, and rhabdomyosarcoma) and 21 minor responses and stable diseases were observed. Pharmacokinetic analysis of CPT-11 and SN-38 was performed in 77 patients. The mean +/- standard deviation (SD) CPT-11 plasma clearance was 20.7 +/- 9.5 L/h/m2 (range, 5 to 54). The mean +/- SD SN-38 metabolic ratio was 1.5% +/- 1.1% (range, 0.15% to 5.55%). CONCLUSION: The recommended phase II dose of CPT-11 in a 3-week schedule is 600 mg/m2 in less heavily, and heavily pretreated children with solid tumors.

Adolescent↗

PubMed2003

[Standards, Options and Recommendations 2002 for the management of adult patients with intracranial gliomas (summary report)].

CONTEXT: The "Standards, Options and Recommendations" (SOR) project, which started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French Regional Cancer Centers, and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and the outcome of cancer patients. OBJECTIVE: To define clinical practice guidelines for the management of adult patients with intracranial glioma in collaboration with the Association of French-speaking Neuro-oncologists (Anocef) and the French society of neurosurgeons (SNCLF). These recommendations cover diagnosis, classification, treatment and follow-up of patients with these tumors. METHOD: The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines has been defined, the document is submitted for review by independent reviewers. RESULTS: This article is a summary version of the full document presenting the clinical practice guidelines with algorithms. The main recommendations concern the place of the surgery, radiotherapy, chemiotherapy, imagery and concomitant medical treatments in the specific treatment strategy of grade III and IV glioma, grade II glioma, gliomatosis cerebri, pilocytic astrocytoma, subependymoma, xanthoastrocytoma, intracranial ependymoma and brain stem glioma.

Adult↗