Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 820
Filter
1.
Arch. argent. pediatr ; 119(6): e626-e630, dic. 2021. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1353052

ABSTRACT

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.


Subject(s)
Humans , Female , Infant, Newborn , Adrenal Gland Neoplasms/diagnosis , Neuroblastoma , Kidney
2.
Rev. Assoc. Méd. Rio Gd. do Sul ; 65(2): 01022105, Abr. - Jun. 2021.
Article in Portuguese | LILACS | ID: biblio-1367815

ABSTRACT

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


Subject(s)
Humans , Tomography, X-Ray Computed , Ultrasonography , Neuroblastoma
3.
Article in Chinese | WPRIM | ID: wpr-879903

ABSTRACT

OBJECTIVE@#To study the efficacy and safety of intensity-modulated radiotherapy (IMRT) in children with high-risk neuroblastoma (NB).@*METHODS@#A retrospective analysis was performed on the medical data of 24 children with high-risk NB who were diagnosed and treated with IMRT in the Department of Hematology and Oncology, Hunan Provincial People's Hospital, from April 2018 to December 2020. The medical data included age, radiotherapy dose, times of radiotherapy, laboratory examination results, adverse reactions, and survival.@*RESULTS@#All 24 children (14 boys and 10 girls) received IMRT, with a mean age of (65±23) months and a median age of 59 months. The primary tumor was located in the abdomen in 23 children and 1 child had primary tumor in the mediastinum. The median age was 41.5 months at the time of radiotherapy. The radiation dose of radiotherapy ranged from 14.4 to 36.0 Gy, with a mean dose of (22±3) Gy and a daily dose of 1.8-2.0 Gy. The radiotherapy was performed for a total number of 8-20 times, with a mean number of 11.9 times. Among these children, 6 received radiotherapy for the residual or metastatic lesion. Of all the 23 children, 3 experienced cough, 2 experienced diarrhea, and 1 experienced vomiting during radiotherapy. At 2 weeks after radiotherapy, serum creatinine ranged from 2.3 to 70.1 μmol/L and alanine aminotransferase ranged from 9.1 to 65.3 μ/L. Ten children experienced grade Ⅲ bone marrow suppression and 2 experienced grade Ⅳ bone marrow suppression 1 to 2 weeks after radiotherapy. Four children experienced grade Ⅲ bone marrow suppression and 1 experienced grade Ⅳ bone marrow suppression 3 to 4 weeks after radiotherapy. During a median follow-up time of 13.5 months, 23 children (96%) achieved stable disease and 1 died. Up to the follow-up date, second malignant tumor or abnormal organ function was not observed.@*CONCLUSIONS@#IMRT can improve the local control rate of NB. IMRT appears to be safe in the treatment of children with NB.


Subject(s)
Child , Child, Preschool , Female , Humans , Male , Neuroblastoma/radiotherapy , Radiotherapy Dosage , Radiotherapy, Intensity-Modulated/adverse effects , Retrospective Studies
4.
Rev. chil. pediatr ; 91(5): 767-772, oct. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1144277

ABSTRACT

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.


Subject(s)
Humans , Female , Infant , Retroperitoneal Neoplasms/diagnosis , Sweating , Tachycardia/etiology , Catecholamines/urine , Flushing/etiology , Hypertension/etiology , Neuroblastoma/diagnosis , Retroperitoneal Neoplasms/complications , Retroperitoneal Neoplasms/urine , Tachycardia/diagnosis , Irritable Mood , Biomarkers, Tumor/urine , Diagnosis, Differential , Hypertension/diagnosis , Neuroblastoma/complications , Neuroblastoma/urine
5.
Rev. chil. pediatr ; 91(3): 379-384, jun. 2020. tab
Article in Spanish | LILACS | ID: biblio-1126175

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Radiopharmaceuticals/adverse effects , 3-Iodobenzylguanidine/adverse effects , Hypothyroidism/etiology , Iodine Radioisotopes/adverse effects , Neuroblastoma/radiotherapy , Thyroid Diseases , Cross-Sectional Studies , Retrospective Studies , Risk Factors , Radiopharmaceuticals/therapeutic use , 3-Iodobenzylguanidine/therapeutic use , Hypothyroidism/diagnosis , Hypothyroidism/epidemiology , Iodine Radioisotopes/therapeutic use
6.
Autops. Case Rep ; 10(4): e2020181, 2020. tab, graf
Article in English | LILACS | ID: biblio-1131852

ABSTRACT

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.


Subject(s)
Humans , Female , Adult , Tumor Lysis Syndrome/pathology , Neuroblastoma/pathology , Fatal Outcome
7.
Braz. j. med. biol. res ; 53(11): e10067, 2020. graf
Article in English | ColecionaSUS, LILACS, ColecionaSUS | ID: biblio-1132493

ABSTRACT

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.


Subject(s)
Humans , Animals , Rabbits , Neuroblastoma/drug therapy , Progesterone , Mifepristone/pharmacology , Glucocorticoids , Mice, Nude
8.
Rev Assoc Med Bras (1992) ; 66(7): 898-903, 2020. tab, graf
Article in English | SES-SP, LILACS, SES-SP | ID: biblio-1136307

ABSTRACT

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.


Subject(s)
Humans , Carcinoma, Non-Small-Cell Lung/genetics , RNA, Long Noncoding/metabolism , Lung Neoplasms/genetics , Neuroblastoma , Prognosis , Biomarkers, Tumor , Gene Expression Regulation, Neoplastic , Kaplan-Meier Estimate
9.
J. venom. anim. toxins incl. trop. dis ; 26: e20190073, 2020. graf, ilus
Article in English | LILACS, VETINDEX | ID: biblio-1135150

ABSTRACT

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)


Subject(s)
Animals , Peptides , Bothrops , Crotalid Venoms/isolation & purification , Lectins, C-Type/isolation & purification , Neuroblastoma , Neutrophils , In Vitro Techniques
10.
Chinese Medical Journal ; (24): 334-343, 2020.
Article in English | WPRIM | ID: wpr-877961

ABSTRACT

BACKGROUND@#High agglomeration of myeloid-derived suppressor cells (MDSCs) in neuroblastoma (NB) impeded therapeutic effects. This study aimed to investigate the role and mechanism of targeted inhibition of MDSCs by low-dose doxorubicin (DOX) to enhance immune efficacy in NB.@*METHODS@#Bagg albino (BALB/c) mice were used as tumor-bearing mouse models by injecting Neuro-2a cells, and MDSCs were eliminated by DOX or dopamine (DA) administration. Tumor-bearing mice were randomly divided into 2.5 mg/kg DOX, 5.0 mg/kg DOX, 50.0 mg/kg DA, and control groups (n = 20). The optimal drug and its concentration for MDSC inhibition were selected according to tumor inhibition. NB antigen-specific cytotoxic T cells (CTLs) were prepared. Tumor-bearing mice were randomly divided into DOX, CTL, anti-ganglioside (GD2), DOX+CTL, DOX+anti-GD2, and control groups. Following low-dose DOX administration, immunotherapy was applied. The levels of human leukocyte antigen (HLA)-I, CD8, interleukin (IL)-2 and interferon (IFN)-γ in peripheral blood, CTLs, T-helper 1 (Thl)/Th2 cytokines, perforin, granzyme and tumor growth were compared among the groups. The Wilcoxon two-sample test and repeated-measures analysis of variance were used to analyze results.@*RESULTS@#The slowest tumor growth (F = 6.095, P = 0.018) and strongest MDSC inhibition (F = 14.632, P = 0.001) were observed in 2.5 mg/kg DOX group. Proliferation of T cells was increased (F = 448.721, P < 0.001) and then decreased (F = 2.047, P = 0.186). After low-dose DOX administration, HLA-I (F = 222.489), CD8 (F = 271.686), Thl/Th2 cytokines, CD4+ and CD8+ lymphocytes, granzyme (F = 2376.475) and perforin (F = 488.531) in tumor, IL-2 (F = 62.951) and IFN-γ (F = 240.709) in peripheral blood of each immunotherapy group were all higher compared with the control group (all of P values < 0.05). The most significant increases in the aforementioned indexes and the most notable tumor growth inhibition were observed in DOX+anti-GD2 and DOX+CTL groups.@*CONCLUSIONS@#Low-dose DOX can be used as a potent immunomodulatory agent that selectively impairs MDSC-induced immunosuppression, thereby fostering immune efficacy in NB.


Subject(s)
Animals , Doxorubicin/therapeutic use , Mice , Mice, Inbred C57BL , Myeloid-Derived Suppressor Cells , Neuroblastoma/drug therapy , Tumor Microenvironment
11.
Article in Chinese | WPRIM | ID: wpr-828913

ABSTRACT

OBJECTIVE@#To investigate the inhibitory effect of ketogenic diet (KD) on growth of neuroblastoma in mice.@*METHODS@#BALB/c-nu mouse models bearing neuroblastoma xenografts were established by subcutaneous injection of human neuroblastoma cell line (SH-SY5Y). When the tumor volume reached 250 mm3, the mice were randomized into SD group with standard diet and PBS treatment, KD group with ketogenic diet and PBS treatment, and CP+KD group with ketogenic diet and cyclophosphamide (60 mg·kg·day) treatment, =8. The tumor volume, body weight, blood glucose, ketone body (β-Hydroxybutyrate) levels, and hepatic steatosis in the mice were assessed. The expressions of caspase-3 and caspase-8 were detected by Western blotting, and Ki67 expresison was detected using immunohistochemistry (IHC). Transmission electron microscopy (TEM) was employed for the autophagosomes, and the autophagic protein Beclin1, LC3A/B and P62 were detected by IHC and Western blotting.@*RESULTS@#On day 28 post tumor cell injection, the mice in KD and CP+KD groups could prolong the overall survival rates than that in SD group ( < 0.001). On day 22 post the injection, the tumor volume in KD group was smaller than that in SD group ( < 0.05); on 16, 19, and 22 day post the injection, the tumor volume in CP+KD group was smaller than that in SD group ( < 0.01). The mice in SD group showed greater body weight on day 19 and higher blood glucose level on day 13 post the injection than those in the other two groups ( < 0.05). Blood ketone level and hepatic steatosis score were higher and glucose ketone index (GKI) was lower in KD and CP+KD groups than those in SD group (all < 0.05). The expressions of Ki67 and apoptotic proteins were detected in the tumor tissues of all groups. TEM revealed more autophagosomes in the tumor tissues of KD group than that of SD group. P62 expression was lowered ( < 0.01) and Beclin1 and LC3A/B expressions were up-regulated in the tumor tissues of KD group ( < 0.05), which is consisitent with IHC.@*CONCLUSIONS@#KD has a strong anti-tumor effect in the xenograft mouse model possibly by regulating cell autophagy.


Subject(s)
3-Hydroxybutyric Acid , Animals , Blood Glucose , Cell Line, Tumor , Diet, Ketogenic , Humans , Mice , Mice, Inbred BALB C , Neuroblastoma
12.
Article in Chinese | WPRIM | ID: wpr-879775

ABSTRACT

OBJECTIVE@#To study the clinical features of neuroblastoma (NB) and the factors influencing survival rate.@*METHODS@#A total of 44 children with NB who were admitted from April 2016 to February 2020 were enrolled as research subjects. A retrospective analysis was performed on their medical data and follow-up data.@*RESULTS@#The common clinical symptoms of these 44 children were fever (10/44, 23%), mass (9/44, 20%), abdominal pain (8/44, 18%), cough (7/44, 16%), pale complexion (3/44, 7%), claudication (2/44, 5%), and abnormal activity (2/44, 5%). According to the INSS stage, 2 children (4%) had stage I NB, 5 children (11%) had stage II NB, 5 children (11%) had stage III NB, and 32 children (73%) had stage IV NB. The mean follow-up time was (15.3±1.5) months, with a recurrence rate of 20% and an overall survival rate of 82%. Among the 44 children, 29 (66%) achieved event-free survival and 7 (16%) had survival with tumor. The univariate analysis showed that a pathological type of NB and an increase in serum neuron-specific enolase (NSE) decreased the overall survival rate of children with NB (P<0.05).@*CONCLUSIONS@#The clinical symptoms of children with NB are not specific at the first visit. Fever, abdominal pain, and mass are common symptoms, and there is a high proportion of children in the advanced stage. The pathological type of NB and an increase in serum NSE may be associated with a reduction in the overall survival rate of children with NB.


Subject(s)
Child , Humans , Infant , Infant, Newborn , Neoplasm Recurrence, Local , Neoplasm Staging , Neuroblastoma , Phosphopyruvate Hydratase , Retrospective Studies , Survival Rate
14.
Arch. argent. pediatr ; 117(6): 651-654, dic. 2019. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1046714

ABSTRACT

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.


Subject(s)
Humans , Male , Child, Preschool , Opsoclonus-Myoclonus Syndrome , Neuroblastoma/surgery , Neuroblastoma/drug therapy , Pediatrics , Abdominal Neoplasms
15.
Medicina (B.Aires) ; 79(4): 280-283, ago. 2019. graf, tab
Article in English | LILACS | ID: biblio-1040522

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Neuroblastoma/diagnosis , Neuroblastoma/drug therapy , Antineoplastic Agents/administration & dosage , Prognosis , Retrospective Studies , Follow-Up Studies , Antineoplastic Protocols , Kaplan-Meier Estimate , Neoplasm Staging , Neuroblastoma/mortality
16.
Rev. chil. pediatr ; 90(3): 321-327, jun. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1013840

ABSTRACT

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.


Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Infant , Bronchopulmonary Sequestration/diagnosis , Adrenal Gland Neoplasms/diagnostic imaging , Neuroblastoma/diagnostic imaging , Ultrasonography, Prenatal , Ultrasonography , Laparoscopy/methods , Adrenal Gland Neoplasms/surgery , Minimally Invasive Surgical Procedures , Diagnosis, Differential , Neuroblastoma/surgery
17.
Arch. argent. pediatr ; 117(2): 163-166, abr. 2019. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1001175

ABSTRACT

El neuroblastoma congénito es el tumor sólido maligno más frecuente en el período neonatal. La forma de presentación suele ser por diagnóstico prenatal o por una masa abdominal. Su estadificación permite clasificarlo en grupos de riesgo con pronóstico y tratamiento diferentes. En el período neonatal, se caracteriza por la alta tasa de regresión espontánea y el buen pronóstico (supervivencia libre de enfermedad a los 5 años superior al 90 %). Se presenta un caso clínico de neuroblastoma congénito cuya forma de presentación, shock e hipertensión, solo estaba descrita en otra ocasión antes. El tratamiento antihipertensivo, junto con la quimioterapia sistémica, produjo el control clínico y la mejoría del paciente.


Congenital neuroblastoma is the most frequent malignant solid tumor in the neonatal period. The clinical presentation is usually either by prenatal diagnosis or by palpation of an abdominal mass. Staging allows classifying it according to risk groups with a different prognosis and treatment. In the neonatal period, it is characterized by a high rate of spontaneous regression and good prognosis (disease-free survival at 5 years greater than 90 %). We present a clinical case of congenital neuroblastoma whose presentation, shock and hypertension, was only described on a previous occasion. Antihypertensive treatment along with systemic chemotherapy produced clinical control and patient improvement.


Subject(s)
Humans , Male , Infant, Newborn , Shock , Hypertension , Neonatology , Neuroblastoma
18.
Rev. bras. parasitol. vet ; 28(1): 126-133, Jan.-Mar. 2019. tab, graf
Article in English | LILACS | ID: biblio-990809

ABSTRACT

Abstract The antitumor properties of ticks salivary gland extracts or recombinant proteins have been reported recently, but little is known about the antitumor properties of the secreted components of saliva. The goal of this study was to investigate the in vitro effect of the saliva of the hard tick Amblyomma sculptum on neuroblastoma cell lines. SK-N-SK, SH-SY5Y, Be(2)-M17, IMR-32, and CHLA-20 cells were susceptible to saliva, with 80% reduction in their viability compared to untreated controls, as demonstrated by the methylene blue assay. Further investigation using CHLA-20 revealed apoptosis, with approximately 30% of annexin-V positive cells, and G0/G1-phase accumulation (>60%) after treatment with saliva. Mitochondrial membrane potential (Δψm) was slightly, but significantly (p < 0.05), reduced and the actin cytoskeleton was disarranged, as indicated by fluorescent microscopy. The viability of human fibroblast (HFF-1 cells) used as a non-tumoral control decreased by approximately 40%. However, no alterations in cell cycle progression, morphology, and Δψm were observed in these cells. The present work provides new perspectives for the characterization of the molecules present in saliva and their antitumor properties.


Resumo As propriedades antitumorais de extratos de glândulas salivares de carrapatos ou proteínas recombinantes foram relatadas recentemente, mas pouco se sabe sobre as propriedades antitumorais dos componentes secretados da saliva. O objetivo deste estudo foi investigar o efeito in vitro da saliva bruta do carrapato duro Amblyomma sculptum sobre as linhagens celulares de neuroblastoma. Células SK-N-SK, SH-SY5Y, Be(2)-M17, IMR-32 e CHLA-20 foram suscetíveis à saliva, com redução de 80% na sua viabilidade em comparação com controles não tratados, como demonstrado pelo ensaio de Azul de Metileno. Investigações posteriores utilizando CHLA-20 revelaram apoptose, com aproximadamente 30% de células positivas para anexina-V, e G0/G1 (> 60%) após tratamento com saliva. O potencial de membrana mitocondrial (Δψm) foi reduzido significativamente (p <0,05), e o citoesqueleto de actina foi desestruturado, como indicado pela microscopia de fluorescência. A viabilidade do fibroblasto humano (células HFF-1), usado como controle não tumoral, diminuiu em aproximadamente 40%. No entanto, não foram observadas alterações na progressão do ciclo celular, morfologia e Δψm nestas células. O presente trabalho fornece novas perspectivas para a caracterização das moléculas presentes na saliva e suas propriedades antitumorais.


Subject(s)
Animals , Saliva/chemistry , Biological Products/pharmacology , Cytoskeleton/drug effects , Ixodidae/chemistry , Arthropod Proteins/pharmacology , Neuroblastoma/pathology , Antineoplastic Agents/pharmacology , Biological Products/isolation & purification , Cell Survival/drug effects , Apoptosis/drug effects , Cell Line, Tumor , Arthropod Proteins/isolation & purification , Antineoplastic Agents/isolation & purification
19.
Säo Paulo med. j ; 137(1): 104-106, Jan.-Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-1004749

ABSTRACT

ABSTRACT CONTEXT: Neuroblastoma is the most common extracranial malignant solid tumor that occurs during childhood. It arises from primitive cells and is seen in the adrenal medulla and sympathetic ganglia of the sympathetic nervous system. CASE REPORT: We present a rare case of a 40-year-old man who was diagnosed with the onset of neuroblastoma arising in the mediastinum. He was treated by means of surgical resection in the superior mediastinum after neoadjuvant chemotherapy. The patient's surgical outcome was satisfactory. CONCLUSION: There are still no standard treatment guidelines for adult neuroblastoma patients. Although they have a poor prognosis, the main treatment option should be complete surgery at an early stage. This situation may become clarified through biological and genetic studies in the future.


Subject(s)
Humans , Male , Adult , Mediastinal Neoplasms/surgery , Neuroblastoma/surgery , Tomography, X-Ray Computed , Treatment Outcome , Chemoradiotherapy, Adjuvant
20.
Article in Chinese | WPRIM | ID: wpr-772513

ABSTRACT

In this paper, the classification and location of neuroblastoma in NMR images are realized by using Deep Neural Network(CNN) algorithm as the core technology. The module is integrated to realize the development of computer-aided diagnostic software. It is used to make up for the gap in the field of intelligent identification and accurate positioning of neuroblastoma in the current nuclear magnetic resonance detection technology, effectively reduce the work intensity of doctors reading films, and further promote the clinical application and technical development of nuclear magnetic resonance detection technology in the diagnosis of neuroblastoma.


Subject(s)
Algorithms , Deep Learning , Humans , Magnetic Resonance Imaging , Neural Networks, Computer , Neuroblastoma , Diagnostic Imaging
SELECTION OF CITATIONS
SEARCH DETAIL