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1.
Medisan ; 27(2)abr. 2023. ilus
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1440584

RESUMO

Se describe el caso clínico de una niña de 13 meses de edad, quien fue asistida en el Servicio de Neurocirugía del Hospital Infantil Sur Dr. Antonio María Béguez César de Santiago de Cuba, y posteriormente en el Servicio de Oncología de la institución, remitida del área de salud por presentar proptosis, exoftalmos del ojo derecho y aumento del perímetro craneal. Luego de realizados el examen clínico y los estudios complementarios pertinentes, incluidos resonancia magnética simple y contrastada de cráneo, estudio histopatológico, así como aspiración y biopsia bilateral de médula ósea, se confirmó que se trataba de un neuroblastoma parameníngeo con infiltración medular (estadio IV), sin evidencia de tumor primario. La evolución luego de la quimioterapia fue satisfactoria.


The case report of a 13 months girl is described who was assisted in the Neurosurgery Service of Antonio María Béguez Cesar Southern Children Hospital in Santiago de Cuba, and later on in the Oncology Service of the institution, referred from the health area due to proptosis, exophthalmos of the right eye and increase of the cranial perimeter. After the clinical exam and the pertinent complementary studies, including simple and contrasted magnetic resonance of skull, histopatologic study, as well as aspiration and bilateral biopsy of bone marrow, the presence of a parameningeal neuroblastoma with medullary infiltration (stage IV), without evidence of primary tumor was confirmed. The evolution after the chemotherapeutic treatment was satisfactory.


Assuntos
Imageamento por Ressonância Magnética , Neuroblastoma , Atenção Secundária à Saúde , Lactente
2.
Rev. Bras. Cancerol. (Online) ; 69(4)out-dez. 2023.
Artigo em Inglês, Espanhol, Português | LILACS, Sec. Est. Saúde SP | ID: biblio-1537372

RESUMO

neuroblastoma é o tumor maligno sólido extracraniano mais comum na infância. Entre seis e dez milhões de crianças são identificadas com essa doença por ano. O tratamento cirúrgico, que envolve a ressecabilidade tumoral, é fator decisivo na estratégia terapêutica desses pacientes, com evidente influência no prognóstico da doença. Relato do caso: Lactente submetido à anestesia geral e a bloqueio de nervo periférico para ressecção de neuroblastoma com monitorização neurofisiológica intraoperatória (MNIO). Como estratégia anestésica, foi realizado o bloqueio do plano do eretor da espinha (ESP Block) associado à técnica de analgesia multimodal visando ao controle da dor. A dor pós-operatória foi avaliada por meio da Children's and Infants' Postoperative Pain Scale (CHIPPS). Conclusão:A MNIO, apesar de restringir as técnicas anestésicas, tem se firmado como uma importante ferramenta para monitorar as funções neurológicas durante as abordagens cirúrgicas com envolvimento do sistema nervoso central, com a finalidade de evitar danos permanentes de importantes estruturas. A interação e a comunicação de toda a equipe são de suma importância nos resultados pós-operatórios


Neuroblastoma is the most common non-central nervous system in childhood. From six to ten million children are identified with this disease annually. Surgical treatment, which involves tumor resectability, is a decisive factor in the therapeutic strategy of these patients, with clear influence on disease's prognosis. Case report: Nursling submitted to general anesthesia and peripheral nerve block for neuroblastoma surgical resection with intraoperative neurophysiological monitoring (IONM) and spinal erector plane block(ESP), associated with the multimodal analgesia technique for pain control. The postoperative pain was evaluated using the Children's and Infants' Postoperative Pain Scale (CHIPPS). Conclusion: IONM has established itself as an important tool to monitor neurological functions during surgical approaches with involvement of the central nervous system, in order to avoid permanent damage to important structures. The interaction and communication of the entire team are of paramount importance in the postoperative results.


Neuroblastoma es el tumor maligno sólido extracraneano más común en la infancia. Entre seis y diez millones de niños son identificados con esta enfermedad por año. El tratamiento quirúrgico, que implica la resecabilidad tumoral, es un factor decisivo en la estrategia terapéutica de estos pacientes, con una influencia evidente en el pronóstico. Informe del caso: Lactante sometido a anestesia general y bloqueo nervioso periférico para resección de neuroblastoma con monitoreo neurofisiológico intraoperatorio (MNIO). Como estrategia anestésica, para resección del neuroblastoma, se realizó bloqueo del plano erector espinal (Bloqueo ESP), asociado a la técnica de analgesia multimodal para controlar el dolor. El dolor postoperatorio se evaluó mediante la Children's and Infants' Postoperative Pain Scale (CHIPPS). Conclusión: La MNIO, a pesar de restringir las técnicas anestésicas, se ha posicionado como una importante herramienta para monitorear las funciones neurológicas durante los abordajes quirúrgicos que involucran al sistema nervioso central, con el fin de evitar daños permanentes a estructuras importantes. La interacción y la comunicación de todo el equipo son de suma importancia en los resultados posoperatorios


Assuntos
Neuroblastoma , Dor Pós-Operatória , Oncologia Cirúrgica
3.
Hematol., Transfus. Cell Ther. (Impr.) ; 44(4): 535-541, Oct.-dec. 2022. tab, ilus
Artigo em Inglês | LILACS | ID: biblio-1421540

RESUMO

ABSTRACT Introduction: An important component of the advances made in neuroblastoma treatment has been the use of peripheral blood stem cells to support high-dose chemotherapy. In this study, we report our experience on a series of small children who have undergone standard and large volume leukaphersis (LVL) procedures, provide an update on a single institution's experience with cryopreservation of autologous peripheral blood stem cells (PBSCs), using 10% dimethyl sulfoxide (DMSO) and applying post-thaw DMSO depletion and analyze a number of variables that may affect viability. Methods: A total of 36 aphereses were performed on 29 children weighing less than 25 kg between July 2016 and October 2019 at the Ibn Sina university hospital. Results: Seven females and twenty-two males, median bodyweight 14 kg (9 - 22). A single apheresis was sufficient to obtain at least 3 × 106/kg body weight (BW) of CD34+ cells in 82.8% of the cases. The LVL was performed in 22 aphereses. A median number of 5.9 × 106/ kg CD34 cells were collected per apheresis. A total of 60 PBSC samples were cryopreserved and 46 samples were infused. The mean cell viability percentage decreased from 94.75 ± 1.14% before freezing to 70.84 ± 8.6% after thawing (p < 0.001). No correlation was found between post-thaw viability and storage time (r = -0.233; p = 0.234) or number of total nucleated cells (r = 0.344; p = 0.073). Conclusion: Leukapheresis is safe and feasible in small pediatric patients if the appropriate measures are used. Cryopreservation poses numerous challenges, especially a decrease in cell viability after thawing.


Assuntos
Neuroblastoma , Células-Tronco , Remoção de Componentes Sanguíneos , Criopreservação , Criança , Leucaférese
5.
São Paulo; s.n; s.n; 2022. 74 p. tab, graf, ilus.
Tese em Português | LILACS | ID: biblio-1378473

RESUMO

O neuroblastoma é um tumor sólido muito comum em crianças. O estágio mais avançado da doença é altamente agressivo e invasivo, além de pouco responsivo à terapia, que é limitada por mecanismos de resistência e reincidência relacionados à metástase. Muitos estudos tem sido feitos para identificar mecanismos de invasão e quimioresistência de células tumorais, afim de aumentar a sobrevida dos pacientes com câncer. Nesse trabalho, nós estudamos o efeito dos macrófagos, as células imunes mais abundantes no microambiente tumoral, os TAMs (do inglês tumor-associated macrophage) e do receptor P2X7, um purinoreceptor acionado por ATP, nesses processos. Os TAMs respondem e atuam de acordo com a miríade de fatores que encontram, podendo gerar populações heterogêneas e com funções distintas, tanto antitumorais, como pró-tumorais. Altos níveis de ATP extracelular são encontrados no microambiente tumoral, podendo então ativar o receptor P2X7. Este receptor tem sido relacionado tanto a funções inflamatórias como funções na resolução da inflamação de macrófagos. Além disso, o receptor P2X7 está envolvido em uma variedade de eventos celulares, incluindo a secreção de mediadores pró-inflamatórios, a proliferação celular e a apoptose de células tumorais. Primeiramente, foi avaliado o papel do receptor P2X7 na polarização de macrófagos da derivados medula óssea de camundongos wild-type e nocaute para o P2X7 na presença e ausência de fatores secretados por células de neuroblastoma, e então foi estudada a influência desses diferentes macrófagos polarizados em eventos celulares de grande relevância clínica para o neuroblastoma: a invasividade e quimiorresistência. Os resultados demonstraram que, apesar do reconhecido envolvimento do receptor P2X7 na inflamação, a ausência deste receptor não atenua a expressão de marcadores característicos do fenótipo inflamatório, M1. O aumento da expressão do receptor P2Y2, também envolvido na inflamação, nessas células, sugere um mecanismo genético de compensação para não atenuação da inflamação em macrófagos que não expressam o receptor P2X7. Contudo, a ausência do receptor P2X7 levou a alterações no fenótipo alternativo, M2, de modo que a expressão de Tnf, marcador de M2, não foi reprimido. TAMs noucates para P2X7 tiveram a expressão de arg1, marcador de M2, suprimida, reforçando a importância do receptor P2X7 no estabelecimento de fenótipos ativados alternativamente. Nossos dados também sugerem que ausência do receptor P2X7 em TAMs permite a aquisição de um fenótipo capaz de tornar as células de neuroblastoma que expressam P2X7 mais invasivas e mais quimioresistentes à vincristina. Por outro lado, TAMs, independentemente da presença ou ausência do receptor P2X7, induziram a proliferação e quimioresistência das células de neuroblastoma silenciadas para o receptor P2X7, o que nos leva a concluir que o receptor P2X7 em TAMs é desfavorável à progressão de tumores expressando P2X7


Neuroblastoma is a highly common childhood solid tumor. The most advanced stage of the disease is highly aggressive and invasive, besides from being poorly responsive to therapies, which are limited by resistance and recurrence mechanisms related to metastasis. Several studies attempt to identify invasion and resistance mechanisms of the tumor cells in order to increase overall survival of the patients. On the present work, we investigated the effect of macrophages, the most abundant immune cells on the tumor microenvironment, called TAMs (tumor-associated macrophages), and of the P2X7 receptor, an ATP-gated purinoceptor, on these processes. TAMs and cancer cells crosstalk, and behave accordingly to a miriad of factors present at the TME, generating heterogeneous populations with distinct functionalities, either pro- or antitumor. High extracellular levels of ATP are found in the TME, being able to activate the P2X7 receptor. This receptor mediates both pro- and anti-inflammatory functions in macrophages. In addition, it is involved in several cellular events, including the secretion of pro-inflammatory mediators, cell proliferation and tumor cell apoptosis. At first, we evaluated the role of the P2X7 receptor on the polarization of bone marrow-derived macrophages (BMDM), either wild-type or knockout for the P2X7 receptor, in presence or absence or factors secreted by neuroblastoma cells. Next, we investigated the influence of the polarized macrophages in highly relevant cellular events for neuroblastoma, such as invasiveness and chemoresistance. Our results showed that, despite the known involvement of P2X7 receptor on inflammation, its absence did not decrease the expression if inflammatory markers of M1 macrophage populations. An increase in the expression of the P2Y2 receptor, also involved in inflammation, on these cells suggest a genetic compensation mechanism for preventing attenuation of inflammation when P2X7 is lacking. However, P2X7 receptor absence did compromise the M2 phenotype, driving the expression of Tnf. TAMs knockout for the P2X7 receptor were not able to express arg1, also an M2 marker, reinforcing a role of the P2X7 receptor on establishing alternative macrophage phenotypes. Our data also suggest that TAMs lacking the P2X7 receptor acquire a phenotype capable of turning P2X7R-expressing neuroblastoma cells more invasive and chemoresistant to vincristine. On the other hand, TAMs, independently on the presence of the P2X7 receptor, induced proliferation and resistance of neuroblastoma cells silenced for P2X7 receptor expression, leading us to the conclusion that the P2X7 receptor in TAMs is unfavorable for the progression of P2X7R-expressing tumors


Assuntos
Animais , Masculino , Feminino , Camundongos , Receptores Purinérgicos P2X7/análise , Receptores Purinérgicos P2Y2/análise , Macrófagos Associados a Tumor/patologia , Macrófagos/efeitos dos fármacos , Neuroblastoma/patologia , Apoio ao Desenvolvimento de Recursos Humanos/classificação , Medula Óssea , Células/química , Inflamação
6.
Braz. J. Pharm. Sci. (Online) ; 58: e19238, 2022. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1374561

RESUMO

Abstract The aim of this work is to study three cultivars of artichoke (Cynara cardunculus var. scolymus): Gauchito, Guri and Oro Verde in terms of their in vitro chemoprevention and anti-inflammatory properties. These cultivars show good productive performance. The phenolic composition of their fresh leaves and edible bracts was analyzed by high performance liquid chromatography and high resolution mass spectrometry (HPLC-HRMS), showing mainly caffeoylquinic acids and flavonoids. Caffeoylquinic acids were quantified and the highest content was found in Gauchito cultivar. In this cultivar, the content of dicaffeoylquinic acids in fresh bracts was six times higher than that in fresh leaves (10064.5 ± 378.3 mg/kg versus 1451.0 ± 209.3 mg/kg respectively). Luteolin flavonoids were detected in leaves. The extracts from fresh bracts and leaves were assessed in their in vitro bioactivity against human neuroblastoma cells (SH-SY5Y). Inhibition of SH-SY5Y cells proliferation by Gauchito and Guri leaf extracts (8 µg/mL) was higher than 50 %. The leaf extracts of the same cultivars showed an inhibitory effect on human interferon IFN-I, decreasing its activity 50% at 40 µg/mL. Interestingly, the bract extracts did not show in vitro bioactivity at these concentrations, nor did the pure compounds chlorogenic acid, cynarin, apigenin and luteolin (at 2 µg/mL). These results suggest that Gauchito and Guri leaf extracts have potential for human neuroblastoma chemoprevention and treatment of inflammatory processes.


Assuntos
Folhas de Planta/classificação , Quimioprevenção , Cynara scolymus/metabolismo , Anti-Inflamatórios/farmacologia , Espectrometria de Massas/métodos , Extratos Vegetais/análise , Cromatografia Líquida de Alta Pressão/métodos , Compostos Fenólicos , Neuroblastoma/patologia
7.
Arch. argent. pediatr ; 119(6): e626-e630, dic. 2021. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1353052

RESUMO

El neuroblastoma es un tumor derivado de la cresta neural. Se trata del tumor maligno más frecuente en recién nacidos y existe una amplia variabilidad en su forma de presentación. En algunos casos, el diagnóstico se realiza mediante un hallazgo ecográfico, mientras que en otros se manifiesta como enfermedad metastásica agresiva. Se presenta el caso de una paciente prematura con diagnóstico posnatal de neuroblastoma congénito metastásico con manifestaciones clínicas graves y evolución atípica. Asimismo, se destaca una lesión renal grave que requirió terapia dialítica.


Neuroblastoma is a neural crest-derived tumor and the most common malignant tumor in neonates. Its clinical presentation can be quite variable. In some cases, it presents as an aggressive metastatic disease whilst in others it is a finding in scans performed for other reasons. The following report presents the case of a premature newborn, diagnosed postnatally with metastatic congenital neuroblastoma, with an atypical and torpid course, including severe renal injury and dialysis requirement.


Assuntos
Humanos , Feminino , Recém-Nascido , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neuroblastoma , Rim
8.
Rev. argent. neurocir ; 35(3): 275-275, sept. 2021. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1426936

RESUMO

Metodología y descripción: se reporta el caso de una paciente de 31 años de edad, primigesta, que consulta a las 20 semanas por hallazgo ecográfico de tumoración orofaríngea fetalAutopsia y resultados: se obtuvo un feto femenino de 950 grs con teratoma maduro de 10x6x5 cm que protruye a través de la boca. Discusión: en la actualidad, se aplica el término epignatus a cualquier teratoma de la cavidad orofaríngea sin especificar el sitio de origen. La pesquisa es por ecografía(3) pero es necesaria una resonancia obstétrica para descartar algunos diagnósticos diferenciales (meningo encefalocele, neuroblastoma, glioma nasal, teratoma cervical) y malformaciones en otras localizaciones que puedan asociarse(2). Pueden tener un crecimiento unidireccional o bidireccional. La mayoría de las publicaciones de epignatus con tumor intracraneano se limitan a reportes de casos. Conclusión: actualmente, el tratamiento quirúrgico agresivo del epignatus con extensión intracraneana no está recomendado. Creemos que correlacionando los estudios pre-natales con los hallazgos post-natales, se podría establecer una clasificación que determine la viabilidad fetal y los casos que serían candidatos a beneficiarse con un tratamiento quirúrgico.


Assuntos
Neoplasias , Autopsia , Anormalidades Congênitas , Feto , Neuroblastoma
9.
Rev. Assoc. Méd. Rio Gd. do Sul ; 65(2): 01022105, Abr. - Jun. 2021.
Artigo em Português | LILACS | ID: biblio-1367815

RESUMO

RESUMO Neoplasias na infância são incomuns, mas ainda são a principal doença relacionada a morte em crianças. Os tumores sólidos representam cerca de 30% de todas as neoplasias malignas pediátricas, sendo o tumor de Wilms e o neuroblastoma duas das malignidades abdominais mais comuns. O neuroblastoma acomete principalmente a faixa etária entre 2 meses e 2 anos, sendo ligeiramente mais comum em meninos caucasianos. O neuroblastoma é a neoplasia maligna sólida extracraniana pediátrica mais comum e, de todas as malignidades, a segunda - 10% de todas as neoplasias. Apresenta associação com as Síndromes de Klippel-Feil, alcoólica fetal, fenil- -hidantoínicas, doença de Hirschsprung e, também, com a de Beckwith-Wiedemann. Quanto mais jovem o paciente ao diagnóstico, maior sua chance de sobrevivência. Os exames de imagem - ultrassonografia, tomografia computadorizada e ressonância magnética - apresentam alta acurácia. Os exames de imagem, em conjunto com a clínica do paciente e dados epidemiológicos, permitem o diagnóstico dos tumores renais pediátricos, mas o exame anatomopatológico ainda é indispensável. PALAVRAS-CHAVE: Neuroblastoma, ultrassonografia, tomografia computadorizada por raios X


ABSTRACT Although uncommon, childhood neoplasms are still the main death-related illness in children. Solid tumors represent about 30% of all pediatric malignancies, with Wilms' tumor and neuroblastoma being two of the most common abdominal malignancies. Neuroblastoma mainly affects the age group between 2 months and 2 years, being slightly more common in Caucasian boys. Neuroblastoma is the most common pediatric extracranial solid malignancy and, of all malignancies, the second ­ 10% of all neoplasms. It is associated with Klippel-Feil, fetal alcoholic, phenytoin/hydantoin and Beckwith-Wiedemann's syndromes, as well as with Hirschsprung's disease. The younger the patients at diagnosis, the greater their chance of survival. Imaging exams ­ ultrasound, computed tomography and magnetic resonance ­ are highly accurate. Imaging exams, together with the patient's clinic and epidemiological data, allow the diagnosis of pediatric kidney tumors, but the anatomopathological exam is still indispensable. KEYWORDS: Neuroblastoma, ultrasonography, X-ray computed tomography


Assuntos
Humanos , Tomografia Computadorizada por Raios X , Ultrassonografia , Neuroblastoma
10.
Rev. chil. pediatr ; 91(5): 767-772, oct. 2020. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1144277

RESUMO

INTRODUCCIÓN: La forma clínica de presentación más común del neuroblastoma es el de una masa abdominal, pero puede presentarse con sintomatología menos habitual, como es la crisis adrenérgica por liberación de catecolaminas. OBJETIVO: Describir una forma de presentación inusual de neuroblastoma y el amplio diagnóstico diferencial que existe en un lactante con síntomas adrenérgicos. CASO CLÍNICO: Lactante femenina de 7 semanas de vida, consultó por historia de tres semanas de sudoración e irritabilidad a lo que se asoció fiebre de 24 h de evolución y dificultad respiratoria. Al ingreso presentaba mal esta do general, irritabilidad, sudoración, enrojecimiento facial, taquipnea y palidez cutánea, taquicardia sinusal extrema e hipertensión arterial (HTA), interpretadas como sintomatología adrenérgica. Se completó el estudio con una ecografía abdominal y resonancia magnética que mostraron una gran masa retroperitoneal compatible con neuroblastoma. Las catecolaminas en sangre y en orina mostraron altos niveles de dopamina, adrenalina y noradrenalina, probablemente de origen tumoral. Se inició tratamiento antihipertensivo con fármacos alfa bloqueantes con buen control de la tensión arterial. Se resecó quirúrgicamente el tumor sin incidencias y con una adecuada recuperación posterior. La paciente presentó evolución favorable a tres años de seguimiento. CONCLUSIONES: en un lactante con sintomatología adrenérgica como irritabilidad, enrojecimiento, sudoración asociada a HTA, se debe descartar patología cardiaca, metabólica (hipoglucemia), intoxicaciones y/o patología suprarrenal. Dentro de esta última, el neuroblastoma es la primera posibilidad diagnóstica, por ser uno de los principales tumores en la infancia y aunque esta presentación no es habitual puede producir estos síntomas.


INTRODUCTION: The most common clinical presentation of neuroblastoma is an abdominal mass, but it can present with uncommon symptoms, such as adrenergic storm due to catecholamine release. OBJECTIVE: To describe an unusual presentation of neuroblastoma and the wide differential diagnosis that exists in an infant with adrenergic symptoms. CLINICAL CASE: A 7-week old female infant was evaluated due to a 3-week history of sweating and irritability associated with a 24-hour fever and respiratory distress. At admission, she presented poor general condition, irritability, sweating, facial redness, tachypnea and skin paleness, extreme sinus tachycardia, and high blood pressure (HBP), interpreted as adrenergic symptoms. The study was completed with abdominal ultrasound and magnetic reso nance imaging that showed a large retroperitoneal mass compatible with neuroblastoma. Plasma and urinary catecholamines tests showed high levels of dopamine, adrenaline, and noradrenaline, probably of tumor origin. We started antihypertensive treatment with alpha-blocker drugs, showing a good blood pressure control. The tumor was surgically resected without incidents and adequate subsequent recovery. The patient presented a favorable evolution after three years of follow-up. CONCLUSIONS: In an infant with adrenergic symptoms such as irritability, redness, sweating associated with HBP, it should be ruled out pathology heart or metabolic (hypoglycemia) pathology, intoxications, and/or adrenal pathology. Within this last one, neuroblastoma is the first diagnostic possibility, since it is one of the main tumors in childhood and, although this presentation is not usual, it can produce these symptoms.


Assuntos
Humanos , Feminino , Lactente , Neoplasias Retroperitoneais/diagnóstico , Sudorese , Taquicardia/etiologia , Catecolaminas/urina , Rubor/etiologia , Hipertensão/etiologia , Neuroblastoma/diagnóstico , Neoplasias Retroperitoneais/complicações , Neoplasias Retroperitoneais/urina , Taquicardia/diagnóstico , Humor Irritável , Biomarcadores Tumorais/urina , Diagnóstico Diferencial , Hipertensão/diagnóstico , Neuroblastoma/complicações , Neuroblastoma/urina
11.
Rev. pediatr. electrón ; 17(3): 49-56, oct. 2020. ilus
Artigo em Espanhol | LILACS | ID: biblio-1367347

RESUMO

El neuroblastoma es uno de los tumores sólidos extracraneales más comunes en la edad pediátrica, y se origina en células precursoras del sistema nervioso simpático. La ubicación cervical corresponde a un 2-5% del total de los neuroblastomas y puede tener distintas manifestaciones clínicas, tales como masa cervical, disnea, estridor, síndrome de Horner o disfagia. Esta entidad debe ser considerada dentro del diagnóstico diferencial de una masa cervical pediátrica, especialmente ante la presencia de masas sólidas, laterales o paramedianas, palpables o no al examen físico. El tratamiento específico del neuroblastoma depende de la clasificación de riesgo del paciente, pudiendo ser expectante en casos específicos, exclusivamente quirúrgico, o bien requerir complementarse con otras terapias. En este artículo se presentan 2 casos clínicos de pacientes pediátricos con neuroblastoma cervical tratados de forma exclusiva y exitosa con cirugía, y una revisión del tema.


Neuroblastoma is one of the commonest extracranial solid tumors at pediatric age, originating from sympathetic nervous system precursor cells. Cervical position stands for 2-5% of all neuroblastomas, with variable clinical expression that includes cervical mass, dyspnea, stridor, Horner syndrome and dysphagia. This condition must be considered in the differential diagnosis of a pediatric cervical mass, specially in those solid, lateral/paramedian masses that could be palpable or not at physical examination. The specific treatment in neuroblastoma depends on patient´s risk group, including conservative follow-up in selected cases, surgery alone, or complementary perioperative therapy with chemotherapy and others. In this article, the group report two cases of cervical neuroblastoma exclusively treated with surgery with good results, and a literature review.


Assuntos
Humanos , Masculino , Recém-Nascido , Lactente , Neoplasias de Cabeça e Pescoço/diagnóstico , Neuroblastoma/cirurgia , Neuroblastoma/diagnóstico , Síndrome de Horner , Diagnóstico Diferencial , Obstrução das Vias Respiratórias/etiologia , Neoplasias de Cabeça e Pescoço/cirurgia , Neoplasias de Cabeça e Pescoço/complicações , Neuroblastoma/complicações
12.
Rev. chil. pediatr ; 91(3): 379-384, jun. 2020. tab
Artigo em Espanhol | LILACS | ID: biblio-1126175

RESUMO

Resumen: Introducción: El tratamiento del neuroblastoma en estadios avanzados incluye quimioterapia, cirugía y terapia con I131-Metayodo benzilguanidina (I131-MIBG). La disfunción tiroidea se reporta entre 12 y 85% a pesar de la protección tiroidea. Objetivo: Identificar la frecuencia de disfunción tiroidea en casos de neu roblastoma tratados con I131-MIBG. Pacientes y Método: Estudio transversal. Se incluyeron todos los casos con diagnóstico de neuroblastoma que recibieron I131-MIBG en el periodo de 2002-2015, a los cuales se les realizó antropometría completa, perfil de tiroides: hormona estimulante de tiroides (TSH), Triyodotironina total y libre (T3t y T3l), tiroxina total y libre (T4t, T4l), y anticuerpos antitiroglobulina y antiperoxidasa. Resultados: Se identificaron un total de 27 pacientes; once fallecieron (40%). De los 16 casos sobrevivientes, 9 (56%) presentaron disfunción tiroidea: 2 (13%) casos con hipotiroidismo subclínico y 7 (44%) casos con hipotiroidismo clínico (3 casos por retraso en el desa rrollo psicomotor y 4 por desaceleración del crecimiento). Los pacientes presentaron manifestaciones clínicas a los 16,1 meses (1,2-66,3 meses) de recibir el radiofármaco a una dosis acumulada de 142 mCi (96-391.5 mCi). No se logró evidenciar diferencias en la edad al diagnóstico, la edad al inicio del tratamiento con el I131-MIBG, la dosis acumulada del I131-MIBG y el tiempo trascurrido entre la dosis y el perfil tiroideo entre los casos con o sin disfunción tiroidea. Conclusiones: El 56% de los pacientes con neuroblastoma presentaron disfunción tiroidea. La mayoría de los casos con hipotiroidismo fue ron referidos cuando los datos de disfunción tiroidea eran clínicamente evidentes. Se propone en esta poblacion realizar perfil tiroideo semestral y valoración anual por un endocrinólogo pediatra durante los primeros 5 años posteriores al diagnóstico oncológico.


Abstract: Introduction: The treatment of advanced neuroblastoma includes chemotherapy, surgery, and radiotherapy with 131-I-Metaiodobenzylguanidine (131-I-MIBG). Despite strategies to protect thyroid function, its dysfunction is reported between 12 and 85%. Objective: To identify the frequency of thyroid dys function in cases of neuroblastoma treated with 131-I-MIBG. Patients and Method: Cross-sectional study. We included all the cases with neuroblastoma treated with 131-I-MIBG between 2002 and 2015, with complete somatometry, and complete thyroid profile (TSH, free and total T3 and T4, and anti-thyroglobulin and antiperoxidase antibodies). Results: 27 patients were identified out of which eleven died (40%). Out of the 16 surviving cases, 9 (56%) presented thyroid dysfunction: 2 (13%) cases with subclinical hypothyroidism and 7 (44%) cases with clinical hypothyroidism (3 cases due to psychomotor developmental delay and 4 due to growth deceleration). The patients presented cli nical manifestations at 16.1 months (1.2-66.3 months) after receiving the radiopharmaceutical at acumulative dose of 142 mCi (96-391.5 mCi). No differences were found in the age at diagnosis, age at the start of treatment with 131-I-MIBG, the cumulative dose of 131-I-MIBG, and the time elapsed between the dose and the thyroid profile among the cases with or without thyroid dysfunction. Con clusions: 56% of patients with neuroblastoma had thyroid dysfunction. Most of the cases with hypothyroidism were referred when thyroid dysfunction was clinically evident. A thyroid profile should be performed every 6 months, along with an annual endocrinological evaluation during the next 5 years in these patients.


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Compostos Radiofarmacêuticos/efeitos adversos , 3-Iodobenzilguanidina/efeitos adversos , Hipotireoidismo/etiologia , Radioisótopos do Iodo/efeitos adversos , Neuroblastoma/radioterapia , Doenças da Glândula Tireoide , Estudos Transversais , Estudos Retrospectivos , Fatores de Risco , Compostos Radiofarmacêuticos/uso terapêutico , 3-Iodobenzilguanidina/uso terapêutico , Hipotireoidismo/diagnóstico , Hipotireoidismo/epidemiologia , Radioisótopos do Iodo/uso terapêutico
13.
Braz. j. med. biol. res ; 53(11): e10067, 2020. graf
Artigo em Inglês | LILACS, Coleciona SUS | ID: biblio-1132493

RESUMO

RU486 (mifepristone), a glucocorticoid and progesterone receptor antagonist, has been reported to exert antiproliferative effects on tumor cells. Experiments were performed to analyze the effects of RU486 on the proliferation of the human neuroblastoma, both in vitro and in vivo, using the human neuroblastoma SK-N-SH cell line. The exposure in vitro of SK-N-SH cells to RU486 revealed a dose-dependent inhibition of 3H-thymidine incorporation due to a rapid but persistent inhibition of MAPKinase activity and ERK phosphorylation. A significant decrease of SK-N-SH cell number was evident after 3, 6, and 9 days of treatment (up to 40% inhibition), without evident cell death. The inhibitory effect exerted by RU486 was not reversed by the treatment of the cells with dexamethasone or progesterone. Moreover, RU486 induced a shift in SK-N-SH cell phenotypes, with an almost complete disappearance of the neuronal-like and a prevalence of the epithelial-like cell subtypes. Finally, the treatment with RU486 of nude mice carrying a SK-N-SH cell xenograft induced a strong inhibition (up to 80%) of tumor growth. These results indicated a clear effect of RU486 on the growth of SK-N-SH neuroblastoma cells that does not seem to be mediated through the classical steroid receptors. RU486 acted mainly on the more aggressive component of the SK-N-SH cell line and its effect in vivo was achieved at a concentration already used to inhibit oocyte implantation.


Assuntos
Humanos , Animais , Coelhos , Neuroblastoma/tratamento farmacológico , Progesterona , Mifepristona/farmacologia , Glucocorticoides , Camundongos Nus
14.
Rev Assoc Med Bras (1992) ; 66(7): 898-903, 2020. tab, graf
Artigo em Inglês | Sec. Est. Saúde SP, LILACS | ID: biblio-1136307

RESUMO

SUMMARY OBJECTIVE Long noncoding RNA neuroblastoma-associated transcript 1 (NBAT1) has been reported to be involved in cancer progression. However, the clinical significance of NBAT1 in non-small cell lung cancer (NSCLC) is still unclear. Our present research aimed to explore whether NBAT1 serves as a biomarker for NSCLC prognosis. METHODS The expression of NBAT1 was examined by RT-PCR in tissue samples of 162 NSCLC patients and was compared with the adjacent non-tumor lung specimens. Then the association between NBAT1 expression and clinical-pathological parameters was further evaluated. Survival analysis was performed using the Kaplan-Meier method. The prognostic significance of NBAT1 expression in NSCLC patients was explored by the use of univariate and multivariate analyses. RESULTS NBAT1 expression was prominently decreased in NSCLC tissues compared with matched normal lung specimens (p < 0.01). Moreover, survival analyses indicated that patients with low expression displayed dramatically decreased 5-year overall survival (p = 0.008). CONCLUSIONS NBAT1 expression might contribute to tumor progression and poor prognosis of NSCLC and might be a new therapeutic target in NSCLC.


RESUMO OBJETIVO Há relatos de que o NBAT1 está associado à progressão do câncer. Contudo, o significado clínico do NBAT1 no câncer de pulmão de células não pequenas (NSCLC) ainda não está claro. O objetivo da nossa pesquisa foi explorar se NBAT1 serve como biomarcador para o prognóstico de NSCLC. MÉTODOS A expressão de NBAT1 foi examinada por RT-PCR em amostras de tecido de 162 pacientes com NSCLC e comparada a amostras adjacentes não tumorais de pulmão. Em seguida, a associação entre a expressão do NBAT1 e os parâmetros clínico-patológicos foi avaliada. A análise de sobrevivência foi realizada utilizando o método Kaplan-Meier. A significância prognóstica da expressão do NBAT1 em pacientes com NSCLC foi explorada através de análises univariadas e multivariadas. RESULTADOS A expressão do NBAT1 foi claramente diminuída nos tecidos de NSCLC em comparação aos espécimes normais dos pulmões (p<0,01). Além disso, as análises de sobrevivência indicaram que pacientes com baixa expressão apresentavam uma diminuição drástica da sobrevivência global em cinco anos (p=0,008). CONCLUSÃO A expressão do NBAT1 pode contribuir para a progressão tumoral e um prognóstico negativo do NSCLC e pode ser um novo alvo de terapia no NSCLC.


Assuntos
Humanos , Carcinoma Pulmonar de Células não Pequenas/genética , RNA Longo não Codificante/metabolismo , Neoplasias Pulmonares/genética , Neuroblastoma , Prognóstico , Biomarcadores Tumorais , Regulação Neoplásica da Expressão Gênica , Estimativa de Kaplan-Meier
15.
Autops. Case Rep ; 10(4): e2020181, 2020. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1131852

RESUMO

Neuroblastoma (NB) is a solid tumor of the sympathetic nervous system, most commonly found in childhood, standing for 7% of all pediatric malignancies. The incidence in adults is markedly smaller: 1 case per 10 million adults per year. We report the case of a previously healthy 27-year-old woman who started with lumbar pain, asthenia, and abdominal distension over the last month. A chest and abdomen tomography scan showed a huge mass in the upper left hemithorax and marked hepatomegaly. The diagnosis was confirmed by hepatic and lung biopsies. On day 4, after admission, the patient started chemotherapy. On the following days, she had severe vaginal bleeding, epistaxis, worsening of the hepatic function markers, refractory shock, and multiple organ dysfunction. She died on the twelfth day of admission. We also present a review of adult cases of NB reported in the past 5 years.


Assuntos
Humanos , Feminino , Adulto , Síndrome de Lise Tumoral/patologia , Neuroblastoma/patologia , Evolução Fatal
16.
J. venom. anim. toxins incl. trop. dis ; 26: e20190073, 2020. graf, ilus
Artigo em Inglês | LILACS, VETINDEX | ID: biblio-1135150

RESUMO

Neuroblastoma is a pediatric tumor with a mortality rate of 40% in the most aggressive cases. Tumor microenvironment components as immune cells contribute to the tumor progression; thereby, the modulation of immune cells to a pro-inflammatory and antitumoral profile could potentialize the immunotherapy, a suggested approach for high-risk patients. Preview studies showed the antitumoral potential of BJcuL, a C- type lectin isolated from Bothrops jararacussu venom. It was able to induce immunomodulatory responses, promoting the rolling and adhesion of leukocytes and the activation of neutrophils. Methods: SK-N-SH cells were incubated with conditioned media (CM) obtained during the treatment of neutrophils with BJcuL and fMLP, a bacteria-derived peptide highly effective for activating neutrophil functions. Then we evaluated the effect of the same stimulation on the co-cultivation of neutrophils and SK-N-SH cells. Tumor cells were tested for viability, migration, and invasion potential. Results: In the viability assay, only neutrophils treated with BJcuL (24 h) and cultivated with SK-N-SH were cytotoxic. Migration of tumor cells decreased when incubated directly (p < 0.001) or indirectly (p < 0.005) with untreated neutrophils. When invasion potential was evaluated, neutrophils incubated with BJcuL reduced the total number of colonies of SK-N-SH cells following co-cultivation for 24 h (p < 0.005). Treatment with CM resulted in decreased anchorage-free survival following 24 h of treatment (p < 0.001). Conclusion: Data demonstrated that SK-N-SH cells maintain their migratory potential in the face of neutrophil modulation by BJcuL, but their invasive capacity was significantly reduced.(AU)


Assuntos
Animais , Peptídeos , Bothrops , Venenos de Crotalídeos/isolamento & purificação , Lectinas Tipo C/isolamento & purificação , Neuroblastoma , Neutrófilos , Técnicas In Vitro
18.
Arch. argent. pediatr ; 117(6): 651-654, dic. 2019. tab, ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1046714

RESUMO

El síndrome de opsoclonus mioclonus es un trastorno poco frecuente en pediatría. El diagnóstico es clínico y se caracteriza por la presencia de, al menos, tres de los siguientes: opsoclonus, mioclonías, ataxia, irritabilidad y trastornos del sueño. En más del 50 % de los casos, se asocia con la presencia de neuroblastoma. Es un trastorno de origen inmunitario y su tratamiento es a base de inmunosupresores, inmunomoduladores y resección tumoral en los casos secundarios a neuroblastoma. Entre el 70 % y el 80 % de los casos pueden tener secuelas neurológicas, dependiendo de la causa, la gravedad inicial de los síntomas y la velocidad de instauración del tratamiento.Se presenta el caso de un varón de 2 años con diagnóstico de síndrome de opsoclonus mioclonus secundario a un neuroblastoma suprarrenal izquierdo, en el que se realizó la resección tumoral y el tratamiento con corticoides, inmunoglobulina y rituximab.


Opsoclonus-myoclonus syndrome is a rare disorder among pediatric patients.The diagnosis is clinical and is characterized by the presence of at least three of the following: opsoclonus, myoclonus, ataxia, irritability and sleep disorders. In over 50 % of cases it is associated with the presence of Neuroblastoma. It is a disorder of immune origin and its treatment is based on immunosuppressants, immunomodulators and tumor resection in cases secondary to Neuroblastoma. Up to 70 % to 80 % of cases may present neurological sequelae, depending on the cause, the initial severity of symptoms and the delay of proper treatment. We present the case of a 2-year-old male with diagnosis of opsoclonus-myoclonus syndrome secondary to a left adrenal Neuroblastoma. Tumor resection and treatment with corticosteroids, immunoglobulin and rituximab were performed.


Assuntos
Humanos , Masculino , Pré-Escolar , Síndrome de Opsoclonia-Mioclonia , Neuroblastoma/cirurgia , Neuroblastoma/tratamento farmacológico , Pediatria , Neoplasias Abdominais
19.
Medicina (B.Aires) ; 79(4): 280-283, ago. 2019. graf, tab
Artigo em Inglês | LILACS | ID: biblio-1040522

RESUMO

The purpose of the study was to evaluate the outcome of patients under 18 months diagnosed with neuroblastoma. Between April 2006 and December 2013, 45 consecutive patients followed in Hospital de Pediatría Garrahan, were retrospectively reviewed. With a median age of 9.3 months (1-18 months) N-myc amplification was detected in 5 out of 38 patients, 1p deletion (del1p) in 4 patients, and 11q aberration in one patient. With a median follow-up of 53 (range: 6-109 months), at 24 months the event free survival (EFS) of all patients was 83% (SE 6%) and overall survival (OS) of 88% (SE 5%). Significant difference was found in OS and EFS between patients with stages L1, L2 and Ms vs. stage M (p = 0.01 and p = 0.01 respectively). EFS for each stage: L1 85% (SE 7%), L2 100%, MS 100%, vs. M 55% (SE 16%). OS: L1 90% (SE 6%), L2 100%, MS 100%, vs. M 66% (SE 15%). OS and EFS results are similar to those reported in international studies. However, better identification of biological prognostic factors will warr ant accurate staging and consequently an appropriate treatment.


El objetivo del trabajo fue evaluar las características y evolución de pacientes menores de 18 meses de edad, con diagnóstico de neuroblastoma. Se realizó un análisis descriptivo, retrospectivo entre abril/2006 y diciembre/2013, de 45 pacientes diagnosticados en forma consecutiva. La edad media fue 9.3 meses (1-18 meses). La amplificación del gen N-myc fue detectada en 5 pacientes, deleción del cromosoma 1p (del1p) en 4, y aberración de 11q en uno. Con una media de seguimiento de 53 meses (6-109 meses), la supervivencia libre de eventos (SLE) de todos los pacientes, a 24 meses fue 83% (ES 6%) y la supervivencia global (SG) de 88% (ES 5%). Se encontró diferencia significativa en la SG y SLE entre los pacientes con estadios L1, L2 y Ms, y aquellos con estadio M (p = 0.01). La SLE para cada estadio fue: L1 85% (ES 7%), L2 100%, MS 100%, M 55% (ES 16%). SG para cada estadio: L1 90% (ES 6%), L2 100%, MS 100%, y M 66% (ES 15%). Aunque los resultados de SG y SLE son similares a los publicados en estudios internacionales, una mejor identificación de los factores pronósticos biológicos permitirá una estadificación precisa y, en consecuencia, un tratamiento adecuado.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Neuroblastoma/diagnóstico , Neuroblastoma/tratamento farmacológico , Antineoplásicos/administração & dosagem , Prognóstico , Estudos Retrospectivos , Seguimentos , Protocolos Antineoplásicos , Estimativa de Kaplan-Meier , Estadiamento de Neoplasias , Neuroblastoma/mortalidade
20.
Rev. chil. pediatr ; 90(3): 321-327, jun. 2019. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1013840

RESUMO

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.


Abstract: Introduction: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. Objective: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. Case report: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. Conclusions: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.


Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Lactente , Sequestro Broncopulmonar/diagnóstico , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neuroblastoma/diagnóstico por imagem , Ultrassonografia Pré-Natal , Ultrassonografia , Laparoscopia/métodos , Neoplasias das Glândulas Suprarrenais/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos , Diagnóstico Diferencial , Neuroblastoma/cirurgia
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