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1.
Pediatr Blood Cancer ; 66(6): e27641, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30746839

RESUMO

OBJECTIVES: According to the Renal Tumor Study Group (RTSG) of the International Society of Paediatric Oncology (SIOP), diagnostic biopsy of renal tumors prior to neoadjuvant chemotherapy is not mandatory unless the presentation is atypical for a Wilms tumor (WT). This study addresses the relevance of this strategy as well as the accuracy and safety of image-guided needle biopsy. METHODS: Clinical, radiological, and pathological data from 317 children (141 males/176 females, mean age: 4 years, range, 0-17.6) consecutively treated in one SIOP-affiliated institution were retrospectively analyzed. RESULTS: Presumptive chemotherapy for WT was decided for 182 patients (57% of the cohort), 24 (8%) were operated upfront, and 111 (35%) were biopsied at diagnosis. A non-WT was confirmed after surgery in 5/182 (3%), 11/24 (46%), and 28/111 (25%), respectively. Age at diagnosis was the most commonly (46%) used criterion to go for biopsy but a nine-year threshold should be retrospectively considered more relevant. Tumor volumes of clear cell sarcoma of the kidney and WT were significantly higher than those of other tumors (P = 0.002). The agreement between core-needle biopsy (CNB) and final histology was 99%. No significant morbidity was associated with CNB. CONCLUSION: The use of SIOP criteria to identify patients eligible for presumptive WT neoadjuvant chemotherapy or upfront surgery avoided biopsy in 65% of children and led to a 97% rate of appropriate preoperative chemotherapy. Image-guided CNB is a safe and accurate diagnostic procedure. The relevance of SIOP biopsy criteria might be improved by using an older age threshold (9 years instead of 6 years) and by adding initial tumor volume.


Assuntos
Carcinoma de Células Renais/diagnóstico , Guias como Assunto , Neoplasias Renais/diagnóstico , Seleção de Pacientes , Tumor de Wilms/diagnóstico , Adolescente , Biópsia , Carcinoma de Células Renais/cirurgia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Neoplasias Renais/cirurgia , Masculino , Terapia Neoadjuvante , Estadiamento de Neoplasias , Prognóstico , Curva ROC , Estudos Retrospectivos , Tumor de Wilms/cirurgia
2.
J Radiol Prot ; 36(1): N1-7, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26878249

RESUMO

To investigate the role of cancer predisposing factors (PFs) on the associations between paediatric computed tomography (CT) scan exposures and subsequent risk of central nervous system (CNS) tumours and leukaemia. A cohort of children who underwent a CT scan in 2000-2010 in 23 French radiology departments was linked with the national childhood cancers registry and national vital status registry; information on PFs was retrieved through hospital discharge databases. In children without PF, hazard ratios of 1.07 (95% CI 0.99-1.10) for CNS tumours (15 cases) and 1.16 (95% CI 0.77-1.27) for leukaemia (12 cases) were estimated for each 10 mGy increment in CT x-rays organ doses. These estimates were similar to those obtained in the whole cohort. In children with PFs, no positive dose-risk association was observed, possibly related to earlier non-cancer mortality in this group. Our results suggest a modifying effect of PFs on CT-related cancer risks, but need to be confirmed by longer follow-up and other studies.


Assuntos
Neoplasias do Sistema Nervoso Central/etiologia , Leucemia Induzida por Radiação/etiologia , Neoplasias Induzidas por Radiação/etiologia , Tomografia Computadorizada por Raios X/efeitos adversos , Neoplasias do Sistema Nervoso Central/mortalidade , Pré-Escolar , Feminino , Humanos , Leucemia Induzida por Radiação/mortalidade , Masculino , Neoplasias Induzidas por Radiação/mortalidade , Risco
3.
Br J Cancer ; 112(1): 185-93, 2015 Jan 06.
Artigo em Inglês | MEDLINE | ID: mdl-25314057

RESUMO

BACKGROUND: Recent epidemiological results suggested an increase of cancer risk after receiving computed tomography (CT) scans in childhood or adolescence. Their interpretation is questioned due to the lack of information about the reasons for examination. Our objective was to estimate the cancer risk related to childhood CT scans, and examine how cancer-predisposing factors (PFs) affect assessment of the radiation-related risk. METHODS: The cohort included 67,274 children who had a first scan before the age of 10 years from 2000 to 2010 in 23 French departments. Cumulative X-rays doses were estimated from radiology protocols. Cancer incidence was retrieved through the national registry of childhood cancers; PF from discharge diagnoses. RESULTS: During a mean follow-up of 4 years, 27 cases of tumours of the central nervous system, 25 of leukaemia and 21 of lymphoma were diagnosed; 32% of them among children with PF. Specific patterns of CT exposures were observed according to PFs. Adjustment for PF reduced the excess risk estimates related to cumulative doses from CT scans. No significant excess risk was observed in relation to CT exposures. CONCLUSIONS: This study suggests that the indication for examinations, whether suspected cancer or PF management, should be considered to avoid overestimation of the cancer risks associated with CT scans.


Assuntos
Neoplasias/epidemiologia , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Criança , Pré-Escolar , Estudos de Coortes , Feminino , França/epidemiologia , Humanos , Incidência , Masculino , Medição de Risco
4.
Eur Radiol ; 24(1): 102-11, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23995879

RESUMO

OBJECTIVES: To assess the benefit and limits of iterative reconstruction of paediatric chest and abdominal computed tomography (CT). METHODS: The study compared adaptive statistical iterative reconstruction (ASIR) with filtered back projection (FBP) on 64-channel MDCT. A phantom study was first performed using variable tube potential, tube current and ASIR settings. The assessed image quality indices were the signal-to-noise ratio (SNR), the noise power spectrum, low contrast detectability (LCD) and spatial resolution. A clinical retrospective study of 26 children (M:F = 14/12, mean age: 4 years, range: 1-9 years) was secondarily performed allowing comparison of 18 chest and 14 abdominal CT pairs, one with a routine CT dose and FBP reconstruction, and the other with 30 % lower dose and 40 % ASIR reconstruction. Two radiologists independently compared the images for overall image quality, noise, sharpness and artefacts, and measured image noise. RESULTS: The phantom study demonstrated a significant increase in SNR without impairment of the LCD or spatial resolution, except for tube current values below 30-50 mA. On clinical images, no significant difference was observed between FBP and reduced dose ASIR images. CONCLUSION: Iterative reconstruction allows at least 30 % dose reduction in paediatric chest and abdominal CT, without impairment of image quality. KEY POINTS: • Iterative reconstruction helps lower radiation exposure levels in children undergoing CT. • Adaptive statistical iterative reconstruction (ASIR) significantly increases SNR without impairing spatial resolution. • For abdomen and chest CT, ASIR allows at least a 30 % dose reduction.


Assuntos
Imagens de Fantasmas , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Radiografia Abdominal/normas , Radiografia Torácica/normas , Tomografia Computadorizada por Raios X/normas , Artefatos , Criança , Pré-Escolar , Interpretação Estatística de Dados , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Radiografia Abdominal/métodos , Radiografia Torácica/métodos , Reprodutibilidade dos Testes , Estudos Retrospectivos , Razão Sinal-Ruído , Tomografia Computadorizada por Raios X/métodos
5.
Pediatr Blood Cancer ; 61(1): 140-4, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23970395

RESUMO

BACKGROUND: Wilms Tumor (WT) can occur in association with tumor predisposition syndromes and/or with clinical malformations. These associations have not been fully characterized at a clinical and molecular genetic level. This study aims to describe clinical malformations, genetic abnormalities, and tumor predisposition syndromes in patients with WT and to propose guidelines regarding indications for clinical and molecular genetic explorations. PROCEDURE: This retrospective study analyzed clinical abnormalities and predisposition syndromes among 295 patients treated for WT between 1986 and 2009 in a single pediatric oncological center. RESULTS: Clinically identified malformations and predisposition syndromes were observed in 52/295 patients (17.6%). Genetically proven tumor predisposition syndromes (n = 14) frequently observed were syndromes associated with alterations of the chromosome WT1 region such as WAGR (n = 6) and Denys-Drash syndromes (n = 3), syndromes associated with alterations of the WT2 region (Beckwith-Wiedeman syndrome, n = 3), and Fanconi anemia (n = 2). Hemihypertrophy and genito-urinary malformations (n = 12 and n = 16, respectively) were the most frequently identified malformations. Other different syndromes or malformations (n = 10) were less frequent. Median age of WT diagnosis was significantly earlier for children with malformations than those without (27 months vs. 37 months, P = 0.0009). There was no significant difference in terms of 5-year EFS and OS between WT patients without or with malformations. CONCLUSIONS: The frequency of malformations observed in patients with WT underline the need of genetic counseling and molecular genetic explorations for a better follow-up of these patients, with a frequently good outcome. A decisional tree, based on clinical observations of patients with WT, is proposed to guide clinicians for further molecular genetic explorations.


Assuntos
Anormalidades Múltiplas , Tumor de Wilms/complicações , Tumor de Wilms/genética , Criança , Pré-Escolar , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Estudos Retrospectivos , Síndrome , Tumor de Wilms/mortalidade
6.
Rev Epidemiol Sante Publique ; 60(5): 363-70, 2012 Oct.
Artigo em Francês | MEDLINE | ID: mdl-22981307

RESUMO

BACKGROUND: The "Cohorte Enfant Scanner", a study designed to investigate the risk of radiation-induced cancer after childhood exposure to CT (computed tomography) examinations, used clinical information contained in the "programme de médicalisation des systèmes d'information" (PMSI) database, the French hospital activities national program based upon diagnosis related groups (DRG). However, the quality and adequacy of the data for the specific needs of the study should be verified. The aim of our work was to estimate the percentage of the cohort's children identified in the PMSI database and to develop an algorithm to individualize the children with a cancer or a disease at risk of cancer from medical diagnoses provided by the DRGs database. METHODS: Of the 1519 children from the "Cohorte Enfant Scanner", who had had a CT scan in the radiology department of a university hospital in 2002, a cross linkage was performed with the DRGs database. All hospitalizations over the period 2002-2009 were taken into account. An algorithm was constructed for the items "cancer" and "disease at risk for cancer" on a sample of 150 children. The algorithm was then tested on the entire population. RESULTS: Overall, 74% of our population was identified in the DRGs database. The algorithm individualized cancer diagnoses with 91% sensitivity (95% confidence interval [95%CI]: 86%; 97%) and 98% specificity (95%CI: 97%; 99%) and 86% positive predictive value (95%CI: 80%; 93%). For the diagnosis of disease at risk for cancer, the sensitivity, specificity and positive predictive value were respectively 91% (95%CI: 84%; 98%), 94% (95%CI: 92%; 95%) and 52% (95%CI: 43%; 61%). CONCLUSION: The DRG database identified with excellent sensitivity and specificity children with diagnoses of cancer or disease at risk for cancer. Hence, potential confounding factors related to the disease of the child can be taken into account for analyses performed with the cohort.


Assuntos
Bases de Dados Factuais/estatística & dados numéricos , Projetos de Pesquisa Epidemiológica , Sistemas de Informação Hospitalar/estatística & dados numéricos , Hospitalização/estatística & dados numéricos , Revisão da Utilização de Seguros/estatística & dados numéricos , Neoplasias Induzidas por Radiação/diagnóstico , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Idade de Início , Algoritmos , Pré-Escolar , Estudos de Coortes , Estudos Epidemiológicos , Feminino , Hospitalização/economia , Humanos , Lactente , Recém-Nascido , Masculino , Neoplasias Induzidas por Radiação/epidemiologia , Prognóstico , Sensibilidade e Especificidade
7.
AJNR Am J Neuroradiol ; 43(1): 151-156, 2022 01.
Artigo em Inglês | MEDLINE | ID: mdl-34887247

RESUMO

BACKGROUND AND PURPOSE: A new brain tumor entity occurring in early childhood characterized by a somatic BCL6 corepressor gene internal tandem duplication was recently described. The aim of this study was to describe the radiologic pattern of these tumors and correlate this pattern with histopathologic findings. MATERIALS AND METHODS: This retrospective, noninterventional study included 10 children diagnosed with a CNS tumor, either by ribonucleic acid-sequencing analysis or deoxyribonucleic acid methylation analysis. Clinical, radiologic, and histopathologic data were collected. A neuropathologist reviewed 9 tumor samples. Preoperative images were analyzed in consensus by 7 pediatric radiologists. RESULTS: All tumors were relatively large (range, 4.7-9.2 cm) intra-axial peripheral masses with well-defined borders and no peritumoral edema. All tumors showed mild and heterogeneous enhancement and marked restriction on DWI of the solid portions. Perfusion imaging showed a relatively lower CBF in the tumor than in the adjacent normal parenchyma. Nine of 10 tumors showed areas of necrosis, with the presence of hemorrhage in 8/10 and calcifications in 4/7. Large intratumoral macroscopic veins were observed in 9/10 patients. No intracranial or spinal leptomeningeal dissemination was noted at diagnosis. CONCLUSIONS: CNS tumors with a BCL6 corepressor gene internal tandem duplication present as large intra-axial peripheral masses with well-defined borders, no edema, restricted diffusion, weak contrast enhancement, frequent central necrosis, hemorrhage and calcifications, intratumoral veins, and no leptomeningeal dissemination at the time of diagnosis. Knowledge of these imaging characteristics may aid in histologic, genomic, and molecular profiling of brain tumors in young children.


Assuntos
Neoplasias Encefálicas , Neoplasias Neuroepiteliomatosas , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Criança , Pré-Escolar , Humanos , Imageamento por Ressonância Magnética , Neoplasias Neuroepiteliomatosas/diagnóstico por imagem , Neoplasias Neuroepiteliomatosas/genética , Neoplasias Neuroepiteliomatosas/patologia , Proteínas Proto-Oncogênicas/genética , Proteínas Repressoras/genética , Estudos Retrospectivos
8.
J Radiol ; 91(11 Pt 2): 1192-8; quiz 1199-200, 2010 Nov.
Artigo em Francês | MEDLINE | ID: mdl-21178891

RESUMO

Radiologists should be able to appreciate the radiation dose delivered to patients for routine diagnostic procedures. The radiology report should include data necessary to calculate the patient dose in Gray. Using the effective dose, it is possible to compare with other source of radiation exposure. Simple formulas, taking into account different anatomical regions, derived from dose-area product (conventional radiography) or dose-length product (CT) are provided to calculate the effective dose in Sievert. For conventional (non-interventional) radiography, the effective dose for a given exam is inferior or equal to the yearly background radiation. For CT, the effective dose corresponds to 1 to 10 years of yearly background radiation.


Assuntos
Leucemia Induzida por Radiação/etiologia , Leucemia Induzida por Radiação/prevenção & controle , Neoplasias Induzidas por Radiação/etiologia , Neoplasias Induzidas por Radiação/prevenção & controle , Lesões por Radiação/prevenção & controle , Radiografia/efeitos adversos , Radiometria/métodos , Adulto , Carga Corporal (Radioterapia) , Criança , Documentação/métodos , Relação Dose-Resposta à Radiação , França , Humanos , Física , Lesões por Radiação/etiologia , Proteção Radiológica/legislação & jurisprudência , Proteção Radiológica/métodos , Sistemas de Informação em Radiologia/normas , Radiologia Intervencionista/normas , Tecnologia Radiológica/legislação & jurisprudência , Tecnologia Radiológica/normas
9.
Eur J Surg Oncol ; 46(7): 1277-1286, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-31839437

RESUMO

BACKGROUND: Outcomes for adults with soft tissue sarcoma are better when managed at referral centers. Care guidelines advise for 5 main criteria: 1-Imaging before biopsy; 2-Tumor biopsy before surgery; 3-Multidiscipinary team discussion (MTD) before biopsy; 4-Biopsy in "expert centers"; 5-Somatic molecular biology feasible. The aim is to describe and assess the prognostic impact of initial management of STS according to the type of referring centers and the number of optimal criteria. METHODS: Monocentric retrospective analysis of the management of 127 youths (0-25 years) with localized STS treated from 2006 to 2015. RESULTS: Median age at diagnosis was 9.6 years (range: 025). Overall, only 41% patients had 5/5, 28% 3-4, 31% ≤2. No adequate imaging was performed before surgery/biopsy for 18% patients, no biopsy before treatment for 29%. Patients referred by "expert centers" had higher compliance to guidelines (P = 0.025). Upfront surgery was performed in 59/127 patients. Immediate re-operation was inversely related to the number of criteria (0% when 5 criteria vs. 14% for 3-4, 46% if ≤ 2; P < 0.001). For malignant tumors, outcome was better when 5 criteria were reached: 5 year EFS 90.8% (81.4-100.0%) vs. 71.6 for (60.4-84.9%; ≤4 criteria; p = 0.033), OS 93.6% (85.5-100%) vs. 79.5% (68.9-91.8%; p = 0.11), and LRFFS 90.6% (81.0-100.0) vs. 73.1% (62.0-86.3%; p = 0.047). CONCLUSION: Less than half of the youths with STS are initially managed according to international guidelines, highlighting the need for better information about optimal management. These results plead for immediate management in reference centers to reduce initial burden of therapy.


Assuntos
Fidelidade a Diretrizes , Recidiva Local de Neoplasia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Adolescente , Adulto , Biópsia , Institutos de Câncer , Quimioterapia Adjuvante , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Terapia Neoadjuvante , Recidiva Local de Neoplasia/patologia , Equipe de Assistência ao Paciente , Guias de Prática Clínica como Assunto , Radioterapia Adjuvante , Encaminhamento e Consulta , Reoperação , Estudos Retrospectivos , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/cirurgia , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
10.
J Neurooncol ; 95(2): 271-279, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19521664

RESUMO

Medulloblastoma patients treated at the Institute Curie between 1980 and 2000 were reviewed. Only patients whose primary treatment included craniospinal radiation were considered. Surviving patients were identified and evaluated by means of self-report questionnaires using the Health Utility Index (HUI). Psychosocial functioning, employment, and other health-related indicators were recorded. Seventy-three patients were treated during the study period. At a median follow-up from diagnosis of 14.4 years, 49 patients were alive and 45 surviving patients could be contacted. Late sequelae were frequent, particularly neurological deficits (71%) and endocrine complications (52%). Impairments of psychosocial functioning, including employment, driving capacity, independent living, and marital status, were identified in most patients. Most long-term medulloblastoma survivors suffer persistent deficits in several domains, with a significant impact on their psychosocial functioning. These findings reinforce the importance of early intervention programs for all survivors in order to reduce the psychosocial impacts of their disease.


Assuntos
Neoplasias Cerebelares/radioterapia , Irradiação Craniana , Meduloblastoma/radioterapia , Qualidade de Vida , Neoplasias da Medula Espinal/radioterapia , Adolescente , Neoplasias Cerebelares/mortalidade , Neoplasias Cerebelares/psicologia , Criança , Pré-Escolar , Feminino , Seguimentos , Nível de Saúde , Humanos , Lactente , Masculino , Meduloblastoma/mortalidade , Meduloblastoma/psicologia , Prognóstico , Neoplasias da Medula Espinal/mortalidade , Neoplasias da Medula Espinal/psicologia , Inquéritos e Questionários , Taxa de Sobrevida , Sobreviventes , Resultado do Tratamento
11.
Phys Med Biol ; 54(7): 1871-92, 2009 Apr 07.
Artigo em Inglês | MEDLINE | ID: mdl-19265204

RESUMO

This study assessed and compared various image quality indices in order to manage the dose of pediatric abdominal MDCT protocols and to provide guidance on dose reduction. PMMA phantoms representing average body diameters at birth, 1 year, 5 years, 10 years and 15 years of age were scanned in a four-channel MDCT with a standard pediatric abdominal CT protocol. Image noise (SD, standard deviation of CT number), noise derivative (ND, derivative of the function of noise with respect to dose) and contrast-to-noise ratio (CNR) were measured. The 'relative' low-contrast detectability (rLCD) was introduced as a new quantity to adjust LCD to the various phantom diameters on the basis of the LCD(1%) assessed in a Catphan phantom and a constant central absorbed dose. The required variations of CTDIvol(16) with respect to phantom size were analyzed in order to maintain each image quality index constant. The use of a fixed SD or CNR level leads to major dose ratios between extreme patient sizes (factor 22.7 to 44 for SD, 31.7 to 51.5 for CNR(2.8%)), whereas fixed ND and rLCD result in acceptable dose ratios ranging between factors of 2.9 and 3.9 between extreme phantom diameters. For a 5-9 mm rLCD1(%), adjusted ND values range between -0.84 and -0.11 HU mGy(-1). Our data provide guidance on dose reduction on the basis of patient dimensions and the required rLCD (e.g., to get a constant 7 mm rLCD(1%) for abdominal diameters of 10, 13, 16, 20 and 25 cm, tube current-time product should be adjusted in order to obtain CTDIvol(16) values of 6.2, 7.2, 8.8, 11.6 and 17.7 mGy, respectively).


Assuntos
Imagens de Fantasmas , Doses de Radiação , Tomografia Computadorizada por Raios X/instrumentação , Abdome , Adolescente , Tamanho Corporal , Criança , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Padrões de Referência , Sensibilidade e Especificidade , Tomografia Computadorizada por Raios X/métodos , Tomografia Computadorizada por Raios X/normas
12.
J Radiol ; 90(2): 207-15, 2009 Feb.
Artigo em Francês | MEDLINE | ID: mdl-19308005

RESUMO

PURPOSE: To evaluate current exposure levels from pediatric MDCT examinations in order to issue recommendations for the routine clinical practice that may be used for establishing future diagnostic reference levels (DRL). Materials and methods. A survey was conducted at hospital sites affiliated with the SFIPP (Société Francophone d'Imagerie Pédiatrique et Prénatale). Tube potential and volume computed tomography dose index (CTDI) vol. values were obtained for three age groups (1, 5 and 10 years) for typical scanning indications of eight anatomical regions. RESULTS: Twenty of 29 sites provided us with a copy of their protocols. All ages groups combined, 97% of protocols used a tension level < or =120 kVp. For age groups 1, 5 and 10 years respectively, the 75th percentiles of dose distributions were: 31, 39.5 and 49.5 mGy for the "head" protocol; 24, 22 and 24 mGy for "head and neck"; 11, 11, and 11 mGy for "paranasal sinuses"; 46, 71 and 87 mGy for "petrous bone"; 3, 3.5 and 5.5 mGy for "chest"; 2, 3 and 4 mGy for "low dose lung"; 4, 4.5 and 7 mGy for "abdomen-pelvis"; 7, 11 and 12 mGy for "bone". The inter-center discrepancy for doses were still high. CONCLUSION: This study provided an evaluation of current practices in reference centers for pediatric imaging and allowed readjustment of MDCT dose recommendations. It could help in the development of DRLS for pediatric MDCT.


Assuntos
Doses de Radiação , Tomografia Computadorizada por Raios X/efeitos adversos , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Criança , Pré-Escolar , Exposição Ambiental , Humanos , Lactente , Inquéritos e Questionários
13.
Ann Urol (Paris) ; 40(5): 280-96, 2006 Oct.
Artigo em Francês | MEDLINE | ID: mdl-17100165

RESUMO

Genitourinary rhabdomyosarcoma (RMS) accounts for approximately 25% of all rhabdomyosarcomas. Management of RMS at this site has changed during the last 5 consecutive Intergroup Rhabdomyosarcoma (IRS) trials, with increasing emphasis of bladder and vaginal conservation. As more effective treatment regimens has improved survival, surgical approaches have evolved to less aggressive management of the primary tumour to improve conservation. Various combinations of chemotherapy, irradiation and surgery have resulted in a decreased late sequelae in the group of patients with sarcoma arising in the genitourinary tract.


Assuntos
Rabdomiossarcoma , Neoplasias Urogenitais , Criança , Feminino , Humanos , Masculino , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/terapia , Neoplasias Urogenitais/diagnóstico , Neoplasias Urogenitais/terapia , Procedimentos Cirúrgicos Urogenitais/métodos
14.
Arch Pediatr ; 23(1): 112-6, 2016 Jan.
Artigo em Francês | MEDLINE | ID: mdl-26679524

RESUMO

Retinoblastoma is the most common intraocular malignancy of infancy with an incidence of 1/15,000 births. Sixty percent of retinoblastomas are unilateral, with a median age at diagnosis of 2 years, and in most cases they are not hereditary. Retinoblastoma is bilateral in 40% of cases, with an earlier median age at diagnosis of 1 year. All bilateral and multifocal unilateral forms are hereditary and are part of a genetic cancer predisposition syndrome. All children with a bilateral or familial form, and 10-15% of children with a unilateral form, constitutionally carry an RB1 gene mutation. The two most frequent symptoms at diagnosis are leukocoria and strabismus. Diagnosis is made by fundoscopy, with ultrasound and magnetic resonance imaging (MRI) contributing both to diagnosis and assessment of the extension of the disease. Treatment of patients with retinoblastoma must take into account the various aspects of the disease (unilateral/bilateral, size, location), the risks for vision, and the possible hereditary nature of the disease. The main prognostic aspects are still early detection and adapted coverage by a multidisciplinary, highly specialized team. Enucleation is still often necessary in unilateral disease; the decision for adjuvant treatment is made according to the histological risk factors. The most important recent therapeutic advances concern conservative treatment, which is proposed for at least one of the two eyes in most bilateral cases: laser alone or in combination with chemotherapy, cryotherapy, or brachytherapy. Recently, the development of new conservative techniques of treatment, such as intra-arterial selective chemotherapy perfusion and intravitreal injections, aims at preserving visual function in these children and decreasing the number of enucleations and the need for external beam radiotherapy. The vital prognosis related to retinoblastoma is now excellent in industrialized countries, but long-term survival is still related to the development of secondary tumors, mainly secondary sarcoma. Retinoblastoma requires multidisciplinary care as well as a long-term specialized follow-up. Early counseling of patients and their family concerning the risk of transmission of the disease and the risk of development of secondary tumors is necessary.


Assuntos
Neoplasias da Retina/diagnóstico , Neoplasias da Retina/terapia , Retinoblastoma/diagnóstico , Retinoblastoma/terapia , Criança , Humanos , Incidência , Prognóstico , Reflexo Pupilar , Neoplasias da Retina/epidemiologia , Neoplasias da Retina/genética , Retinoblastoma/epidemiologia , Retinoblastoma/genética , Proteína do Retinoblastoma/genética , Estrabismo/etiologia
15.
Arch Pediatr ; 22(1): 14-23, 2015 Jan.
Artigo em Francês | MEDLINE | ID: mdl-25435271

RESUMO

INTRODUCTION: Pseudotumoral soft tissue masses in children and adolescents are a frequent reason for consultation and a diagnostic dilemma. Soft tissue malignancies are relatively uncommon, unlike the large number of benign lesions that may be seen in the superficial tissue and that can be diagnosed with clinical characteristics. MATERIALS AND METHODS: This retrospective study concerns 161 children and adolescents less than 20 years old, referred for a soft tissue mass between 2007 and 2011. It describes their epidemiology, clinical characteristics, and course of care to validate a diagnostic strategy for such masses. RESULTS: Final diagnoses were malignant tumors (44%), benign tumors (32%), and pseudotumoral lesions (24%). Clinical features were similar between these three groups except for age and tumor location, with more benign thoracic masses in younger children. Clinical and radiological association led to an accurate diagnosis for 50% of benign masses and with cytological analysis contribution in 79% of benign tumors and 86% of pseudotumoral lesions. Malignant tumors were suspected in only 39% of cases with radiological exams and in 89% after fine-needle aspiration, an essential additional diagnostic tool. Final diagnoses were formally established through simple standard clinical and radiological evaluation in 19 patients (11.8%; benign tumors, seven patients; malformations, eight patients; post-traumatic lesions, two patients; infection and inflammation, one patient each); ultrasound exam in five patients (3.1%; hemangioendotheliomas, two patients, fascial dehiscence, hemangioma, and vascular malformation, one patient each); MRI in four patients (2.5%; three vascular malformations and one lipoma); CT in two cases (1.2%; vascular malformation and myositis ossificans), and radiological examinations associated with cell aspiration in 15 cases (9.3%; ten benign tumors and five malignant tumors). CONCLUSIONS: A multidisciplinary approach should be requested from oncological, radiological, and pathologic experts to optimize soft tissue mass management as soon as initial investigations start. The authors advise a diagnostic strategy for children with pseudotumoral soft tissue masses.


Assuntos
Neoplasias de Tecidos Moles/diagnóstico , Adolescente , Malformações Arteriovenosas/diagnóstico , Biópsia por Agulha Fina , Criança , Diagnóstico Diferencial , Diagnóstico por Imagem , Feminino , Fibromatose Agressiva/diagnóstico , Hemangioendotelioma/diagnóstico , Humanos , Inflamação/diagnóstico , Lipoma/diagnóstico , Masculino , Miosite Ossificante/diagnóstico , Neurilemoma/diagnóstico , Estudos Retrospectivos , Sarcoma/diagnóstico , Infecções dos Tecidos Moles/diagnóstico
16.
J Fr Ophtalmol ; 38(6): 535-41, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25976136

RESUMO

INTRODUCTION: To describe the results of retinoblastoma treatment from 1995-2009 in a single institution. MATERIAL AND METHODS: Retrospective review of the charts of patients treated for retinoblastoma. Clinical characteristics at diagnosis, treatments and outcomes in terms of survival and ocular preservation are described. RESULTS: During the study period 826 children were referred for retinoblastoma and 730 were managed in our institution. Four hundred and eleven children presented with unilateral retinoblastoma and 319 with bilateral retinoblastoma. Median follow-up is of 93 months. Global survival is 98.5% of children, 10 children presented with second tumors, 11 children died (6 of tumor-related causes). Of the 411 children with unilateral retinoblastoma enucleation was needed at diagnosis for 324 (78.8%). Conservative treatments were attempted for 87 patients (21.2%) and ocular preservation obtained for 65 patients (74% of eyes). Three hundred and nineteen patients presented with bilateral retinoblastoma. Three hundred and ten could be treated conservatively for at least one eye. Initial intravenous chemotherapy was necessary for 75% of them. Ocular preservation without external beam radiation was possible for 221 patients (70%). The use of EBR decreased significantly after 2004 (9.1% of eyes vs 25.1%: P<0.001). DISCUSSION: Management and treatment of retinoblastoma are complex, adapted to the extent of the disease. Survival is good. Enucleation is still required for extensive ocular disease, especially for unilateral patients. Intravenous chemotherapy allows good tumor control and eye preservation and decrease the need of EBR. CONCLUSIONS: Retinoblastoma treatment with intravenous chemotherapy and ocular adjuvant therapies is very effective on the local tumor control and eye preservation.


Assuntos
Neoplasias Primárias Múltiplas/terapia , Neoplasias da Retina/terapia , Retinoblastoma/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Quimioterapia Adjuvante , Criança , Pré-Escolar , Terapia Combinada , Enucleação Ocular , Feminino , Seguimentos , Predisposição Genética para Doença , Humanos , Hipertermia Induzida , Lactente , Recém-Nascido , Infusões Intravenosas , Masculino , Neoplasias Primárias Múltiplas/mortalidade , Neoplasias Primárias Múltiplas/patologia , Preservação de Órgãos , Radioterapia/métodos , Neoplasias da Retina/genética , Neoplasias da Retina/mortalidade , Neoplasias da Retina/patologia , Retinoblastoma/genética , Retinoblastoma/mortalidade , Retinoblastoma/patologia , Estudos Retrospectivos , Análise de Sobrevida
17.
Eur J Cancer ; 40(18): 2659-66, 2004 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-15571949

RESUMO

We report five paediatric cases of portal vein thrombosis (PVT) occurring during chemotherapy, observed in two institutions over an 8-year time period. These children aged 2.5-15 years were treated for Burkitt's lymphoma, Ewing's tumour, small cell bone tumour or medulloblastoma. PVT was diagnosed on colour Doppler ultrasonography (US). In four patients, thrombosis occurred 2-45 days after severe hepatic veno-occlusive disease (HVOD) secondary to intensive chemotherapy containing busulfan. In one case, PVT occurred in the absence of HVOD in a patient with pre-existing periportal lymphomatous infiltration. Four patients experienced persistent portal hypertension, which resulted in death in one. PVT during chemotherapy in children is a rare event and appears to be closely related to intensive chemotherapy containing busulfan and to be associated with HVOD.


Assuntos
Antineoplásicos/efeitos adversos , Neoplasias/tratamento farmacológico , Veia Porta , Trombose Venosa/induzido quimicamente , Adolescente , Anticoagulantes/uso terapêutico , Criança , Pré-Escolar , Feminino , Heparina/uso terapêutico , Humanos , Masculino , Fatores de Risco , Resultado do Tratamento , Trombose Venosa/tratamento farmacológico
18.
AJNR Am J Neuroradiol ; 18(8): 1491-7, 1997 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-9296190

RESUMO

PURPOSE: To assess the in vitro MR signal of the developing brain through histologic comparisons. METHODS: Five healthy fetal specimens aged 16, 19, 22, 27, and 34 gestational weeks were studied in vitro using T1- and T2-weighted sequences in frontal and axial planes. Neuropathologic studies included sections in the same frontal plane. Comparison of histologic sections with measurements of the relative widths of the layers of different signal intensities enabled us to assign cellular correspondence to each MR layer. RESULTS: In the cerebral mantle, a layered pattern was observed on both T1- and T2-weighted images. In the basal ganglia, signal from the pallidum and thalamus was isointense with white matter from 16 to 22 weeks' gestation; then, from 27 and 34 weeks' gestation, the signal was relatively high on T1-weighted images and low on T2-weighted images. The neostriatum had a relatively low signal on T1-weighted images and a high signal on T2-weighted images from 16 to 27 weeks' gestation: then, at 34 weeks' gestation, the signal was relatively high on T1-weighted images and low on T2-weighted images. CONCLUSION: MR imaging can clearly show specific patterns of growing fetal brain in vitro.


Assuntos
Encéfalo/embriologia , Imageamento por Ressonância Magnética , Gânglios da Base/embriologia , Gânglios da Base/patologia , Encéfalo/patologia , Córtex Cerebral/embriologia , Córtex Cerebral/patologia , Feminino , Idade Gestacional , Humanos , Técnicas In Vitro , Recém-Nascido , Gravidez , Valores de Referência
19.
AJNR Am J Neuroradiol ; 22(3): 499-504, 2001 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-11237973

RESUMO

SUMMARY: Diffuse infiltrating retinoblastoma is a rare form of retinoblastoma. We report two cases of this disease in which sonographic, CT, and MR imaging findings were compared with histologic studies obtained after enucleation. Although nonspecific, MR imaging provides valuable morphologic data for the diagnosis of diffuse infiltrating retinoblastoma and may help in decisions regarding enucleation.


Assuntos
Imageamento por Ressonância Magnética , Neoplasias da Retina/diagnóstico , Retinoblastoma/diagnóstico , Tomografia Computadorizada por Raios X , Ultrassonografia , Pré-Escolar , Enucleação Ocular , Feminino , Humanos , Masculino , Neoplasias da Retina/patologia , Neoplasias da Retina/cirurgia , Retinoblastoma/patologia , Retinoblastoma/cirurgia
20.
AJNR Am J Neuroradiol ; 22(1): 184-9, 2001 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-11158907

RESUMO

BACKGROUND AND PURPOSE: Few investigators have analyzed the MR imaging patterns of fetal gyration. Our purpose was to establish, with a large prospective series, the normal sulcation landmarks according to gestational age by using in utero MR imaging and to correlate our findings with established neuroanatomic timetables. METHODS: A standardized fetal cerebral MR examination was performed in 173 normal fetuses at 22 to 38 weeks' gestation. Eight T1- and T2-weighted coronal, axial, and sagittal slices were obtained for each fetus and systematically analyzed. The sequential development of the different fissures and sulci of the cerebral cortex with respect to gestational age were tabulated. RESULTS: Sulcation of the medial, lateral, and inferior surfaces of the brain was depicted, and a timetable for the MR depiction of the primary and secondary sulci was established for the 22- to 38-week gestational period. This timetable was in good agreement with the neuroanatomic standards of reference, with a mean lag time of 1 week. CONCLUSION: This analysis of fetal brain sulcation in a large series of fetuses contributes to a better understanding of the maturation of the fetal cortex on MR imaging studies. It furthermore provides a standard of reference that can be used to assess the normality of fetal sulcation and to diagnose gyrational abnormalities with prenatal MR imaging.


Assuntos
Córtex Cerebral/embriologia , Feto/anatomia & histologia , Feto/fisiologia , Imageamento por Ressonância Magnética , Desenvolvimento Embrionário e Fetal , Idade Gestacional , Humanos , Estudos Prospectivos , Valores de Referência
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