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PURPOSE: Our study aimed to explore real-world treatment scenarios for children and adolescents with neurotrophic tropomyosin receptor kinase (NTRK)-fused tumors, emphasizing access, responses, side effects, and outcomes. PATIENTS AND METHODS: Pooled clinical data from 17 pediatric cases (11 soft-tissue sarcomas, five brain tumors, and one neuroblastoma) treated with larotrectinib and radiologic images for 14 patients were centrally reviewed. Testing for gene fusions was prompted by poor response to treatment, tumor progression, or aggressiveness. RESULTS: Six different NTRK fusion subtypes were detected, and various payment sources for testing and medication were reported. Radiologic review revealed objective tumor responses (OR) in 11 of 14 patients: Complete responses: two; partial responses: nine; and stable disease: three cases. Grades 1 or 2 Common Terminology Criteria for Adverse Events adverse effects were reported in five patients. Regarding the entire cohort's clinical information, 15 of 17 patients remain alive (median observation time: 25 months): four with no evidence of disease and 11 alive with disease (10 without progression). One patient developed resistance to the NTRK inhibitor and died from disease progression while another patient died due to an unrelated cause. CONCLUSION: This real-world study confirms favorable agnostic tumor OR rates to larotrectinib in children with NTRK-fused tumors. Better coordination to facilitate access to medication remains a challenge, particularly in middle-income countries like Brazil.
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Inibidores de Proteínas Quinases , Pirazóis , Humanos , Criança , Masculino , Feminino , Adolescente , Pirazóis/uso terapêutico , Pré-Escolar , Inibidores de Proteínas Quinases/uso terapêutico , Pirimidinas/uso terapêutico , Receptor trkA/genética , Receptor trkA/antagonistas & inibidores , Neoplasias Encefálicas/tratamento farmacológico , Neoplasias Encefálicas/genética , Sarcoma/tratamento farmacológico , Sarcoma/genética , Neuroblastoma/tratamento farmacológico , Neuroblastoma/genética , Lactente , Receptor trkB/genética , Receptor trkC/genética , Ensaios Clínicos como AssuntoRESUMO
Retinoblastoma, the most common intraocular tumor in childhood, still faces challenges in diagnosis and treatment, particularly in low- and middle-income countries. Identifying strategies to improve the time to diagnosis and access to treatment is crucial to enhance survival rates and preserve ocular health. We conducted a systematic review to identify interventions that have demonstrated potential in addressing these challenges. We performed a comprehensive search across databases until March 2023. Out of the studies reviewed, 21 met the inclusion criteria and were categorized into five main areas: surveillance strategies, genetic counseling, education, public assistance, and international partnership. Despite the obstacles faced, the initiatives identified in this review present acts toward improving the time to diagnosis and access to treatment for retinoblastoma. Based on the extracted data, we propose a comprehensive chain of initiatives. We firmly believe that implementing this chain of initiatives can lead to improved clinical outcomes for retinoblastoma patients.
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Países em Desenvolvimento , Acessibilidade aos Serviços de Saúde , Neoplasias da Retina , Retinoblastoma , Retinoblastoma/terapia , Retinoblastoma/diagnóstico , Humanos , Neoplasias da Retina/terapia , Neoplasias da Retina/diagnósticoRESUMO
OBJECTIVE: This study investigated the concentrations of neutrophil extracellular traps (NET) and salivary cytokines (IL-1ß, IL-6, IL-8/CXCL8, TNF, and TGF-ß1) in patients undergoing chemotherapy and their associations with oral mucositis (OM) and Candida infection. MATERIALS AND METHODS: This prospective longitudinal study performed at a Brazilian service included 60 adults diagnosed with hematolymphoid diseases. Saliva samples were collected on days D0, D3, D10, and D15. Cytokines were analyzed by ELISA and NET formation by identification of the myeloperoxidase-DNA complex. Oral Candida spp. was cultured. RESULTS: OM occurred in 43.3% of patients and oral candidiasis in 20%. However, 66% of individuals had positive cultures for C. albicans. Higher concentrations of IL-6, IL-8/CXCL8, and TNF and lower concentrations of TGF-ß1 were observed in patients with OM. C. albicans infection contributed to the increase in IL-8/CXCL8, TGF-ß1, and TNF. Individuals with OM or with oral candidiasis had significant reductions in NET formation. In contrast, individuals with C. albicans and with concomitant C. albicans and OM exhibited higher NET formation. CONCLUSION: The kinetics of cytokine levels and NET formation in chemotherapy-induced OM appears to be altered by Candida infection, even in the absence of clinical signs of oral candidiasis.
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Candidíase Bucal , Citocinas , Armadilhas Extracelulares , Saliva , Estomatite , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Citocinas/metabolismo , Candidíase Bucal/microbiologia , Estomatite/microbiologia , Estomatite/metabolismo , Estudos Prospectivos , Adulto , Armadilhas Extracelulares/metabolismo , Saliva/microbiologia , Saliva/metabolismo , Idoso , Estudos Longitudinais , Fator de Crescimento Transformador beta1/metabolismo , Interleucina-8/metabolismo , Interleucina-8/análise , Fator de Necrose Tumoral alfa/metabolismo , Candida albicans , Antineoplásicos , Interleucina-6/análise , Interleucina-6/metabolismo , Interleucina-1beta/metabolismo , Neoplasias Hematológicas/complicaçõesRESUMO
Abstract Objective The aim of this study was to analyze the effects of fluid overload related to mechanical ventilation, renal replacement therapy, and evolution to discharge or death in critically ill children. Methods A retrospective study in a Pediatric Intensive Care Unit for two years. Patients who required invasive ventilatory support and vasopressor and/or inotropic medications were considered critically ill. Results 70 patients were included. The mean age was 6.8 ± 6 years. There was a tolerable increase in fluid overload during hospitalization, with a median of 2.45% on the first day, 5.10% on the third day, and 8.39% on the tenth day. The median fluid overload on the third day among those patients in pressure support ventilation mode was 4.80% while the median of those who remained on controlled ventilation was 8.45% (p = 0.039). Statistical significance was observed in the correlations between fluid overload measurements on the first, third, and tenth days of hospitalization and the beginning of renal replacement therapy (p = 0.049) and between renal replacement therapy and death (p = 0.01). The median fluid overload was 7.50% in patients who died versus 4.90% in those who did not die on the third day of hospitalization (p = 0.064). There was no statistically significant association between death and the variables sex or age. Conclusions The fluid overload on the third day of hospitalization proved to be a determinant for the clinical outcomes of weaning from mechanical ventilation, initiation of renal replacement therapy, discharge from the intensive care unit, or death among these children.
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INTRODUCTION: Febrile neutropenia (FN) is a serious complication of cancer chemotherapy. The present study aimed to identify risk factors for documented infection in pediatric patients with FN and cancer. METHODS: This prospective cohort study included patients under 18 years from 2016 to 2018. Infection was defined according to the Centers for Disease Control and Prevention criteria. RESULTS: A total of 172 febrile neutropenic episodes were evaluated. From univariate analysis, the risk factors were: female gender; monocyte count < 100 cell/mm³, platelets < 50,000, C-reactive protein (CRP) > 90 mg/dl and hemoglobin < 7mg/dl at the onset of an episode; two or more episodes of FN, and; fever onset; positive blood culture at the fever onset. Independent risk factors according to the multivariate analysis were: CRP at the onset of a febrile episode > 90mg/dl, fever onset and first blood culture with a positive result. The lowest probability of infection was related to first episode and to platelets > 50,000 at the onset of fever. CONCLUSION: A CRP > 90 at the onset of a febrile episode, platelets < 50,000, second episode or more, first fever episode during hospitalization and positive first blood culture were found to be associated with a higher risk of infection and they could be useful for the establishment of risk scores for infection in neutropenic children.
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BACKGROUND: Obese individuals have higher rates of cancer incidence and cancer- related mortality. The worse chemotherapy outcomes observed in this subset of patients are multifactorial, including the altered physiology in obesity and its impact on pharmacokinetics, the possible increased risk of underdosing, and treatment-related toxicity. AIMS: The present review aimed to discuss recent data on physiology, providing just an overall perspective and pharmacokinetic alterations in obesity concerning chemotherapy. We also reviewed the controversies of dosing adjustment strategies in adult and pediatric patients, mainly addressing the use of actual total body weight and ideal body weight. METHODS: This narrative review tried to provide the best evidence to support antineoplastic drug dosing strategies in children, adolescents, and adults. RESULTS: Cardiovascular, hepatic, and renal alterations of obesity can affect the distribution, metabolism, and clearance of drugs. Anticancer drugs have a narrow therapeutic range, and variations in dosing may result in either toxicity or underdosing. Obese patients are underrepresented in clinical trials that focus on determining recommendations for chemotherapy dosing and administration in clinical practice. After considering associated comorbidities, the guidelines recommend that chemotherapy should be dosed according to body surface area (BSA) calculated with actual total body weight, not an estimate or ideal weight, especially when the intention of therapy is the cure. CONCLUSION: The actual total body weight dosing appears to be a better approach to dosing anticancer drugs in both adults and children when aiming for curative results, showing no difference in toxicity and no limitation in treatment outcomes compared to adjusted doses.
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Antineoplásicos , Neoplasias , Adulto , Adolescente , Humanos , Criança , Peso Corporal/fisiologia , Obesidade/tratamento farmacológico , Neoplasias/tratamento farmacológico , Preparações FarmacêuticasRESUMO
OBJECTIVE: The aim of this study was to analyze the effects of fluid overload related to mechanical ventilation, renal replacement therapy, and evolution to discharge or death in critically ill children. METHODS: A retrospective study in a Pediatric Intensive Care Unit for two years. Patients who required invasive ventilatory support and vasopressor and/or inotropic medications were considered critically ill. RESULTS: 70 patients were included. The mean age was 6.8 ± 6 years. There was a tolerable increase in fluid overload during hospitalization, with a median of 2.45% on the first day, 5.10% on the third day, and 8.39% on the tenth day. The median fluid overload on the third day among those patients in pressure support ventilation mode was 4.80% while the median of those who remained on controlled ventilation was 8.45% (pâ¯=â¯0.039). Statistical significance was observed in the correlations between fluid overload measurements on the first, third, and tenth days of hospitalization and the beginning of renal replacement therapy (pâ¯=â¯0.049) and between renal replacement therapy and death (pâ¯=â¯0.01). The median fluid overload was 7.50% in patients who died versus 4.90% in those who did not die on the third day of hospitalization (pâ¯=â¯0.064). There was no statistically significant association between death and the variables sex or age. CONCLUSIONS: The fluid overload on the third day of hospitalization proved to be a determinant for the clinical outcomes of weaning from mechanical ventilation, initiation of renal replacement therapy, discharge from the intensive care unit, or death among these children.
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Estado Terminal , Desequilíbrio Hidroeletrolítico , Criança , Humanos , Lactente , Pré-Escolar , Estudos Retrospectivos , Estado Terminal/terapia , Desequilíbrio Hidroeletrolítico/etiologia , Desequilíbrio Hidroeletrolítico/terapia , Unidades de Terapia Intensiva Pediátrica , Terapia de Substituição Renal , Unidades de Terapia IntensivaRESUMO
RATIONALE: To understand the risk factors for catheter-related infection during treatment of pediatric cancer is essential to implement measures to prevent its occurrence. BACKGROUND: We performed a comprehensive systematic review of the literature with meta-analysis to identify and synthesize the main risk factors for catheter-related infection in children undergoing oncological treatment. METHODS: Systematic searches were conducted in Medline, Embase, Lilacs, and BVS (Biblioteca Virtual em Saúde) until January 2022, following PRISMA guidelines. The search was not limited to language or dates. Risk factors were divided into host-related, assistance-related, and catheter types. We also describe the most common pathogens. RESULTS: Thirteen studies were included in the review. Diagnosis of hematological neoplasm, the intensity of treatment, blood transfusion in the 4-7 days before the infection, type of long-term catheters (tunneled externalized catheters, double lumen, greater diameter), inpatient treatment, and a longer period of hospitalization were the most consistent risk factors. Metanalysis showed that neutropenia at the moment of catheter placement is not a risk factor for central-line-associated bloodstream infections, however, there is high heterogeneity between studies. Staphylococcus epidermidis was the most common pathogen reported. CONCLUSIONS: Understanding risk factors is an essential step to reduce morbidity and mortality of catheter-related infection. Education for preventive measures, reduction of hospitalization, wisely choosing the most adequate type of catheter, and the best moment for catheter insertion may reduce the occurrence of catheter-related infection.
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Infecções Relacionadas a Cateter , Cateterismo Venoso Central , Cateteres Venosos Centrais , Neoplasias , Criança , Humanos , Infecções Relacionadas a Cateter/prevenção & controle , Cateterismo , Cateteres Venosos Centrais/efeitos adversos , Fatores de Risco , Neoplasias/complicações , Cateterismo Venoso Central/efeitos adversosRESUMO
Abstract Introduction Febrile neutropenia (FN) is a serious complication of cancer chemotherapy. The present study aimed to identify risk factors for documented infection in pediatric patients with FN and cancer. Methods This prospective cohort study included patients under 18 years from 2016 to 2018. Infection was defined according to the Centers for Disease Control and Prevention criteria. Results A total of 172 febrile neutropenic episodes were evaluated. From univariate analysis, the risk factors were: female gender; monocyte count < 100 cell/mm³, platelets < 50,000, C-reactive protein (CRP) > 90 mg/dl and hemoglobin < 7mg/dl at the onset of an episode; two or more episodes of FN, and; fever onset; positive blood culture at the fever onset. Independent risk factors according to the multivariate analysis were: CRP at the onset of a febrile episode > 90mg/dl, fever onset and first blood culture with a positive result. The lowest probability of infection was related to first episode and to platelets > 50,000 at the onset of fever. Conclusion A CRP > 90 at the onset of a febrile episode, platelets < 50,000, second episode or more, first fever episode during hospitalization and positive first blood culture were found to be associated with a higher risk of infection and they could be useful for the establishment of risk scores for infection in neutropenic children.
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Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Sepse , Neutropenia Febril , Fatores de Risco , Bacteriemia , Tratamento Farmacológico , NeoplasiasRESUMO
Abstract Introduction Invasive fungal diseases represent important causes of morbidity and mortality among pediatric oncohematological patients. Acute invasive fungal rhinosinusitis is a rare and aggressive disease that occurs mainly in immunocompromised patients. The mortality rate is high and therefore, accurate and early diagnosis is essential. Objectives The aim of this study was to describe the frequency of acute invasive fungal rhinosinusitis among pediatric oncohematological patients and characterize them with confirmed diagnoses. Methods This was a retrospective study that analyzed the medical records of pediatric patients diagnosed with oncohematological diseases and suspected fungal infections, who were included after obtaining informed consent, from January to December 2017, in the pediatric unit of a tertiary university hospital. Data collected from medical record analysis included the following: underlying diagnosis, absolute neutrophil count, clinical presentation, culture and biopsy results, surgical procedures performed, survival and mortality. Results A total of 27 patients were evaluated, with three suspected cases of acute invasive fungal rhinosinusitis. Histopathological and microbiological analyses confirmed two cases. In both cases, the pathogen isolated in the culture was Fusarium sp. The two confirmed cases were female, aged 12 and 14 years, both with an absolute neutrophil count of 10 cells/μL. The underlying disease of the first patient was acute myeloid leukemia (subtype M5), whereas the second patient presented idiopathic bone marrow aplasia. Conclusion Both confirmed cases of acute invasive fungal rhinosinusitis presented with constitutional symptoms and signs of nasal and sinusital inflammation. This demonstrates the importance of fever as a symptom in immunocompromised patients and it should prompt otorhinolaryngological investigation.
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Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Fusariose , Infecções Fúngicas Invasivas , Doenças Hematológicas , Sinusite , Neutropenia Febril , FusariumAssuntos
Escorbuto , Ácido Ascórbico/uso terapêutico , Criança , Dieta , Humanos , Escorbuto/diagnóstico , Escorbuto/etiologiaRESUMO
INTRODUCTION: Invasive fungal diseases represent important causes of morbidity and mortality among pediatric oncohematological patients. Acute invasive fungal rhinosinusitis is a rare and aggressive disease that occurs mainly in immunocompromised patients. The mortality rate is high and therefore, accurate and early diagnosis is essential. OBJECTIVES: The aim of this study was to describe the frequency of acute invasive fungal rhinosinusitis among pediatric oncohematological patients and characterize them with confirmed diagnoses. METHODS: This was a retrospective study that analyzed the medical records of pediatric patients diagnosed with oncohematological diseases and suspected fungal infections, who were included after obtaining informed consent, from January to December 2017, in the pediatric unit of a tertiary university hospital. Data collected from medical record analysis included the following: underlying diagnosis, absolute neutrophil count, clinical presentation, culture and biopsy results, surgical procedures performed, survival and mortality. RESULTS: A total of 27 patients were evaluated, with three suspected cases of acute invasive fungal rhinosinusitis. Histopathological and microbiological analyses confirmed two cases. In both cases, the pathogen isolated in the culture was Fusarium sp. The two confirmed cases were female, aged 12 and 14 years, both with an absolute neutrophil count of 10cells/µL. The underlying disease of the first patient was acute myeloid leukemia (subtype M5), whereas the second patient presented idiopathic bone marrow aplasia. CONCLUSION: Both confirmed cases of acute invasive fungal rhinosinusitis presented with constitutional symptoms and signs of nasal and sinusital inflammation. This demonstrates the importance of fever as a symptom in immunocompromised patients and it should prompt otorhinolaryngological investigation.
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BACKGROUND: Pediatric tumors remain the highest cause of death in developed countries. Research on novel therapeutic strategies with lesser side effects is of utmost importance. In this scenario, the role of Renin-Angiotensin System (RAS) axes, the classical one formed by angiotensinconverting enzyme (ACE), Angiotensin II and AT1 receptor and the alternative axis composed by ACE2, Angiotensin-(1-7) and Mas receptor, have been investigated in cancer. OBJECTIVE: This review aimed to summarize the pathophysiological role of RAS in cancer, evidence for anti-tumor effects of ACE2/Angiotensin-(1-7)/Mas receptor axis and future therapeutic perspectives for pediatric cancer. METHODS: Pubmed, Scopus and Scielo were searched in regard to RAS molecules in human cancer and pediatric patients. The search terms were "RAS", "ACE", "Angiotensin-(1-7)", "ACE2", "Angiotensin II", "AT1 receptor", "Mas receptor", "Pediatric", "Cancer". RESULTS: Experimental studies have shown that Angiotensin-(1-7) inhibits the growth of tumor cells and reduces local inflammation and angiogenesis in several types of cancer. Clinical trials with Angiotensin-( 1-7) or TXA127, a pharmaceutical grade formulation of the naturally occurring peptide, have reported promising findings, but not enough to recommend medical use in human cancer. In regard to pediatric cancer, only three articles that marginally investigated RAS components were found and none of them evaluated molecules of the alternative RAS axis. CONCLUSION: Despite the potential applicability of Angiotensin-(1-7) in pediatric tumors, the role of this molecule was never tested. Further clinical trials are necessary, also including pediatric patients, to confirm safety and efficiency and to define therapeutic targets.
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Angiotensina I/metabolismo , Enzima de Conversão de Angiotensina 2/metabolismo , Neoplasias/metabolismo , Fragmentos de Peptídeos/metabolismo , Proteínas Proto-Oncogênicas/metabolismo , Receptores Acoplados a Proteínas G/metabolismo , Criança , Humanos , Neoplasias/complicações , Pediatria , Proto-Oncogene Mas , Sistema Renina-AngiotensinaRESUMO
SUMMARY Childhood renal tumors account for ~7% of all childhood cancers, and most cases are embryonic Wilms' tumors (WT). Children with WT are usually treated by either COG or SIOP. The later treats the children using preoperative chemotherapy, but both have around 90% of overall survival in five years. WT is a genetically heterogeneous group with a low prevalence of known somatic alterations. Only around 30% of the cases present mutation in known genes, and there is a relatively high degree of intra-tumor genetic heterogeneity (ITGH). Besides potentially having an impact on the clinical outcome of patients, ITGH may interfere with the search for molecular markers that are prospectively being tested by COG and SIOP. In this review, we present the proposal of the current UMBRELLA SIOP Study 2017/Brazilian Renal Tumor Group that requires the multi-sampling collection of each tumor to better evaluate possible molecular markers, as well as to understand WT biology
RESUMO Os tumores renais pediátricos correspondem a aproximadamente 7% de todos os tumores infantis, sendo o mais frequente o tumor de Wilms (TW). Crianças com TW são geralmente tratadas seguindo dois distintos protocolos terapêuticos (COG ou SIOP), sendo que no último, os pacientes recebem tratamento quimioterápico pré-operatório. Ambos apresentam sobrevida global em cinco anos em torno de 90%. TW é geneticamente heterogêneo, apresentando baixa prevalência de alterações somáticas conhecidas, com cerca de 30% dos casos apresentando mutações em genes conhecidos e um alto grau de heterogeneidade genética intratumoral (HGIT). Além de potencialmente ter um impacto sobre o desfecho clínico dos pacientes, a HGIT pode interferir na busca de marcadores moleculares que estão sendo testados prospectivamente pelos grupos COG e Siop. Nesta revisão, apresentamos a proposta do atual estudo Umbrella Siop 2017/Grupo de Tumores Renais Brasileiros (GTRB), que orienta a coleta de três diferentes regiões do tumor para melhor avaliar possíveis marcadores moleculares, bem como para compreender a biologia do TW.
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Humanos , Criança , Tumor de Wilms/genética , Tumor de Wilms/patologia , Heterogeneidade Genética , Neoplasias Renais/genética , Neoplasias Renais/patologia , Prognóstico , Brasil , Biomarcadores Tumorais/análise , MutaçãoRESUMO
RESUMO Objetivo: Analisar as características clínicas, laboratoriais e histopatológicas e o percurso até o estabelecimento do diagnóstico e do tratamento de pacientes com carcinoma de suprarrenal (CSR). Métodos: Estudo retrospectivo com 13 pacientes tratados no serviço de oncologia pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais (HC-UFMG) entre 2004 e 2015. Resultados: A idade ao diagnóstico variou de 1,0 a 14,8 anos (mediana: 2,0 anos). As manifestações de hipercortisolismo foram identificadas em todos os casos, e as de virilização, em todas as meninas. Todos os pacientes preencheram os critérios de Weiss para diagnóstico histopatológico de CSR. A imuno-histoquímica foi realizada em 61,5% dos casos. A maioria dos pacientes apresentou doença em estádio I (76,9%). Todos foram submetidos à ressecção tumoral total. Dois pacientes (estádios III e IV) receberam quimioterapia associada ao mitotano. O único óbito observado foi do paciente com doença em estádio IV. A probabilidade de sobrevida global para todo o grupo aos 5,0 anos foi de 92,3±7,4%. A mediana de tempo entre o início dos sintomas e o diagnóstico foi de 9,5 meses, e de 6,0 meses entre a primeira consulta e o início do tratamento. Conclusões: A baixa idade ao diagnóstico, o predomínio de casos com doença localizada e a ressecção tumoral completa - com apenas um caso de ruptura de cápsula tumoral - são possivelmente a explicação para a evolução favorável da população estudada. O longo percurso entre o início dos sintomas e o diagnóstico sugere a importância da capacitação dos pediatras para o reconhecimento precoce dos sinais e dos sintomas do CSR.
ABSTRACT Objective: To analyze clinical, laboratory and histopathological features and the path to diagnosis establishment and treatment of patients with adrenal carcinoma (AC). Methods: Retrospective study with 13 patients assisted at the pediatric oncology service of Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 2004 and 2015. Results: Age at diagnosis ranged from 1.0 to 14.8 years (median: 2.0 years). Manifestations of hypercortisolism were identified in all cases and virilization in all girls. All patients met the Weiss criteria to AC histopathological diagnosis. Immunohistochemistry was performed in 61.5% of the cases. Most patients had stage I disease (76.9%). All subjects were submitted to total tumor resection. Two patients (stages III and IV disease) received chemotherapy associated to mitotane. The only death case was that of a patient with stage IV disease. The probability of overall survival for the entire group up to 5.0 years was 92.3±7.4%. The median time between the onset of symptoms and diagnosis was 9.5 months, and 6.0 months between first visit and start of treatment. Conclusions: Low age at diagnosis, predominance of cases with localized disease and complete tumor resection - with only one case of tumor capsule rupture - can possibly explain the favorable evolution of the studied population. The long period between onset of symptoms and diagnosis highlights the importance of training pediatricians for early recognition of AC signs and symptoms.
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Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Antineoplásicos/uso terapêutico , Avaliação de Processos e Resultados em Cuidados de Saúde , Brasil/epidemiologia , Carcinoma/mortalidade , Carcinoma/patologia , Carcinoma/terapia , Estudos Retrospectivos , Neoplasias das Glândulas Suprarrenais/mortalidade , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/terapia , Glândulas Suprarrenais/patologia , Adrenalectomia/métodos , Adrenalectomia/estatística & dados numéricos , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/etiologia , Detecção Precoce de Câncer , Tempo para o Tratamento/estatística & dados numéricos , Estadiamento de NeoplasiasRESUMO
Childhood renal tumors account for ~7% of all childhood cancers, and most cases are embryonic Wilms' tumors (WT). Children with WT are usually treated by either COG or SIOP. The later treats the children using preoperative chemotherapy, but both have around 90% of overall survival in five years. WT is a genetically heterogeneous group with a low prevalence of known somatic alterations. Only around 30% of the cases present mutation in known genes, and there is a relatively high degree of intra-tumor genetic heterogeneity (ITGH). Besides potentially having an impact on the clinical outcome of patients, ITGH may interfere with the search for molecular markers that are prospectively being tested by COG and SIOP. In this review, we present the proposal of the current UMBRELLA SIOP Study 2017/Brazilian Renal Tumor Group that requires the multi-sampling collection of each tumor to better evaluate possible molecular markers, as well as to understand WT biology.
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Heterogeneidade Genética , Neoplasias Renais/genética , Neoplasias Renais/patologia , Tumor de Wilms/genética , Tumor de Wilms/patologia , Biomarcadores Tumorais/análise , Brasil , Criança , Humanos , Mutação , PrognósticoRESUMO
OBJECTIVE: To analyze clinical, laboratory and histopathological features and the path to diagnosis establishment and treatment of patients with adrenal carcinoma (AC). METHODS: Retrospective study with 13 patients assisted at the pediatric oncology service of Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 2004 and 2015. RESULTS: Age at diagnosis ranged from 1.0 to 14.8 years (median: 2.0 years). Manifestations of hypercortisolism were identified in all cases and virilization in all girls. All patients met the Weiss criteria to AC histopathological diagnosis. Immunohistochemistry was performed in 61.5% of the cases. Most patients had stage I disease (76.9%). All subjects were submitted to total tumor resection. Two patients (stages III and IV disease) received chemotherapy associated to mitotane. The only death case was that of a patient with stage IV disease. The probability of overall survival for the entire group up to 5.0 years was 92.3±7.4%. The median time between the onset of symptoms and diagnosis was 9.5 months, and 6.0 months between first visit and start of treatment. CONCLUSIONS: Low age at diagnosis, predominance of cases with localized disease and complete tumor resection - with only one case of tumor capsule rupture - can possibly explain the favorable evolution of the studied population. The long period between onset of symptoms and diagnosis highlights the importance of training pediatricians for early recognition of AC signs and symptoms.
OBJETIVO: Analisar as características clínicas, laboratoriais e histopatológicas e o percurso até o estabelecimento do diagnóstico e do tratamento de pacientes com carcinoma de suprarrenal (CSR). MÉTODOS: Estudo retrospectivo com 13 pacientes tratados no serviço de oncologia pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais (HC-UFMG) entre 2004 e 2015. RESULTADOS: A idade ao diagnóstico variou de 1,0 a 14,8 anos (mediana: 2,0 anos). As manifestações de hipercortisolismo foram identificadas em todos os casos, e as de virilização, em todas as meninas. Todos os pacientes preencheram os critérios de Weiss para diagnóstico histopatológico de CSR. A imuno-histoquímica foi realizada em 61,5% dos casos. A maioria dos pacientes apresentou doença em estádio I (76,9%). Todos foram submetidos à ressecção tumoral total. Dois pacientes (estádios III e IV) receberam quimioterapia associada ao mitotano. O único óbito observado foi do paciente com doença em estádio IV. A probabilidade de sobrevida global para todo o grupo aos 5,0 anos foi de 92,3±7,4%. A mediana de tempo entre o início dos sintomas e o diagnóstico foi de 9,5 meses, e de 6,0 meses entre a primeira consulta e o início do tratamento. CONCLUSÕES: A baixa idade ao diagnóstico, o predomínio de casos com doença localizada e a ressecção tumoral completa - com apenas um caso de ruptura de cápsula tumoral - são possivelmente a explicação para a evolução favorável da população estudada. O longo percurso entre o início dos sintomas e o diagnóstico sugere a importância da capacitação dos pediatras para o reconhecimento precoce dos sinais e dos sintomas do CSR.
Assuntos
Neoplasias das Glândulas Suprarrenais , Adrenalectomia , Antineoplásicos/uso terapêutico , Carcinoma , Síndrome de Cushing , Adolescente , Neoplasias das Glândulas Suprarrenais/mortalidade , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/terapia , Glândulas Suprarrenais/patologia , Adrenalectomia/métodos , Adrenalectomia/estatística & dados numéricos , Brasil/epidemiologia , Carcinoma/mortalidade , Carcinoma/patologia , Carcinoma/terapia , Criança , Pré-Escolar , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/etiologia , Detecção Precoce de Câncer , Feminino , Humanos , Lactente , Masculino , Estadiamento de Neoplasias , Avaliação de Processos e Resultados em Cuidados de Saúde , Estudos Retrospectivos , Tempo para o Tratamento/estatística & dados numéricosRESUMO
OBJECTIVE: to determine, in pediatric patients with malignant neoplasms, the characteristics of pulmonary nodules identified on computed tomography, as well as the possibility of differentiating benign lesions from metastases. METHODS: we conducted a retrospective study of patients submitted to pulmonary resections of nodules diagnosed as metastases in a period of seven years. We compared computed tomography and surgery findings, as well as results of anatomopathological examinations. RESULTS: we studied nine patients submitted to 11 surgical interventions. Among the studied variables, only nodule size greater than 12.5mm proved to be statistically significant to predict malignancy. CONCLUSION: among the tomographic characteristics of pulmonary nodules in children with malignant neoplasms, only the size of the lesion was a predictor of malignancy.
OBJETIVO: determinar, em pacientes pediátricos portadores de neoplasias malignas, as características de nódulos pulmonares identificados à tomografia computadorizada, capazes de diferenciar nódulos benignos de metástases. MÉTODOS: estudo retrospectivo de pacientes submetidos a ressecções pulmonares de nódulos diagnosticados como metástases em um período de sete anos. Achados de tomografia e da cirurgia, assim como resultados dos exames anatomopatológicos foram comparados. RESULTADOS: nove pacientes, submetidos a 11 intervenções cirúrgicas, foram estudados. Entre as variáveis estudadas, apenas o tamanho do nódulo, maior do que 12,5mm provou ser estatisticamente significante para predizer malignidade. CONCLUSÃO: esse estudo sugere que, entre as características tomográficas de nódulos pulmonares de crianças portadoras de neoplasias malignas, apenas o tamanho da lesão foi preditor de malignidade.
Assuntos
Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/cirurgia , Procedimentos Desnecessários , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Hepatoblastoma/patologia , Humanos , Neoplasias Renais/patologia , Neoplasias Hepáticas/patologia , Neoplasias Pulmonares/diagnóstico por imagem , Osteossarcoma/patologia , Estudos Retrospectivos , Sensibilidade e Especificidade , Teratoma/patologia , Toracoscopia/métodos , Tomografia Computadorizada por Raios X , Tumor de Wilms/patologiaRESUMO
OBJECTIVE: To describe the profile of reported healthcare-associated infections (HAIs) in pediatric patients submitted to hematopoietic stem cell transplantation (HSCT) at a reference center. METHODS: Retrospective cohort of pediatric patients who were submitted to HSCT from 2008 to 2016. The criteria for HAI were based on those established by the National Healthcare Safety Network. Data were collected by active surveillance performed daily by professionals. This study was approved by the institutional research ethics committee. RESULTS: A total of 86 HSCTs were performed in 81 patients younger than 18 years of age (median, 10 years). Of these, 69 (85%) were males. Aplastic anemia and leukemia were the main diagnoses. A total of 140 HAIs were diagnosed with an incidence density of 28.2 infections/1000 patient-days. The most common HAI was laboratory-confirmed bloodstream infection (46), the majority of which was reported to be central venous catheter-associated (43). Gram-negative bacteria were the most prevalent microorganisms (58.5%). Almost all the infections occurred until 30 days after transplantation, and 17 deaths were observed within 180 days after the procedure. CONCLUSION: Active surveillance of HAIs in HSCT children allowed the evaluation of the incidence and profile of HAIs, which is essential for the health care of these patients.