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1.
Hematol., Transfus. Cell Ther. (Impr.) ; 44(4): 535-541, Oct.-dec. 2022. tab, ilus
Article in English | LILACS | ID: biblio-1421540

ABSTRACT

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.


Subject(s)
Neuroblastoma , Stem Cells , Blood Component Removal , Cryopreservation , Child , Leukapheresis
2.
Alerta (San Salvador) ; 5(1): 12-16, ene. 28, 2022. ilus
Article in Spanish | LILACS, BISSAL | ID: biblio-1354268

ABSTRACT

El neuroblastoma olfativo, también conocido como estesioneuroblastoma, es un tumor derivado de la capa basal del epitelio olfativo. Se presenta como una masa de tejido blando en la porción superior de la cavidad nasal que involucra las células de aire etmoides anteriores y medias en un lado y se extiende a través de la placa cribiforme en la fosa craneal anterior, es poco frecuente y representa menos del 3 % de las neoplasias intranasales. De acuerdo con la evolución epidemiológica, su distribución por edades es bimodal con un pico en pacientes adultos en la segunda década de la vida y otro en los quinta y sexta, sin existir predilección por género reconocida


Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a tumor derived from the basal layer of the olfactory epithelium. It presents as a soft tissue mass in the upper portion of the nasal cavity that involves the anterior and middle ethmoid air cells on one side and extends through the cribriform plate in the anterior cranial fossa, it is rare and represents less 3% of intranasal neoplasms. According to the epidemiological evolution, its age distribution is bimodal with a peak in adult patients in the second decade of life and another in the fifth and sixth, with no recognized gender predilection


Subject(s)
Olfactory Mucosa , Esthesioneuroblastoma, Olfactory , Epithelium , Nasal Cavity , Patients , Air , Neoplasms
3.
Article in Chinese | WPRIM | ID: wpr-954067

ABSTRACT

Neuroblastoma is the most common extracranial solid tumor for infants and young children.About 50% of patients have extensive metastasis before diagnosis, and the neuroblastoma can metastasize to bone marrow, bone, lymph node, orbit, liver and skin.Bone marrow is the most common site of neuroblastoma metastasis and recurrence.Once neuroblastoma metastasize or relapse, their survival rate will reduce significantly.The mechanism of neuroblastoma bone marrow metastasis has not been elucidated.The drug resistance of tumor cells, the interaction of bone marrow microenvironment, and the regulation of cell signaling pathways may play important roles in regulating tumor cell bone marrow metastasis.This review summarizes the research progress of bone marrow metastasis in neuroblastoma, which helps us to better understand the mechanism of interaction between neuroblastoma and the bone marrow microenvironment, and to provide new ideas for the diagnosis and treatment of patients.

4.
Article in Chinese | WPRIM | ID: wpr-934399

ABSTRACT

Objective:To retrospectively analyze the bone marrow smears of neuroblastoma and rhabdomyosarcoma in children and summarize the morphological characteristics of the two types of tumors invading the bone marrow to provide reference for the identification and differential diagnosis.Method:A total of 908 bone marrow specimens were collected from the outpatient and inpatient children who were diagnosed as Neuroblastoma and Rhabdomyosarcoma by pathological tissue or lymph node biopsy in Sun Yat-sen University Cancer Center from January 2013 to July 2020. Of which, 231 cases of tumor bone marrow metastasis were detected. Bone marrow smears were observed and analyzed, classified and summarized according to the morphological characteristics of tumor cells.Result:A total of 231 cases of bone marrow metastases were detected, including two types of tumors, 217 cases Neuroblastoma, with an invasion rate of 34.23%; 14 cases Rhabdomyosarcoma, with an invasion rate of 5.11%. The tumor cells of neuroblastoma were arranged in a pseudo-chrysanthemum or wall-like arrangement and most of them were surrounded by nerve fibers. According to cell size, they could be divided into large cell type and small cell type. Rhabdomyosarcoma cells were mainly medium in size, with vacuoles in the nucleus, and double, triple and multinucleated cells can be seen. The cytoplasm was gauze-like, with bead-like vacuoles at the edges. According to the morphological characteristics of neuroblastoma and rhabdomyosarcoma, they can be differentiated from acute leukemia.Conclusion:Among two malignant solid tumors in children, Neuroblastoma had a higher bone marrow invasion rate, while Rhabdomyosarcoma had a lower bone marrow invasion rate. Rhabdomyosarcoma can be initially divided into embryonal type and acinar type according to whether tumor cells were fused or not.

5.
Chinese Journal of Neurology ; (12): 223-228, 2022.
Article in Chinese | WPRIM | ID: wpr-933785

ABSTRACT

Objective:To summarize the clinical features of childhood opsoclonus-myoclonus syndrome (OMS), evaluate severity degree and prognosis using OMS Symptom Severity Standard Rating Scale.Methods:The clinical features,diagnosis, therapeutic regimen and follow-up of 9 children with OMS in Department of Neurology and Oncology Department of Children's Hospital of Fudan University between 2011 and 2019 were retrospectively reviewed. Severity degree and prognosis were evaluated using OMS Symptom Severity Standard Rating Scale.Results:Among the 9 children with OMS, 4 were males and 5 were females. The onset age ranged from 14 months to 5 years, with a median of 17 months. Main symptoms were opsoclonus, myoclonus, ataxia, motor function regression, behavior and mood changes and sleep disorders. Eghit patients were combined with tumor. Seven of them with neuroblastoma were confirmed by surgical pathology, the other one showed spontaneous regression and symptom remission without treatment. Eight patients received hormone combined with intravenous immunoglobulin, of which 3 cases received adrenocorticotropic hormone, while 5 cases received methylprednisolone pulse therapy, with prednisone sequential therapy, decreasing dose gradually. Rituximab was administrated in 3 patients whose annual recurrent time was≥2. Nine patients were divided into 3 groups according to OMS Symptom Severity Standard Rating Scale, 1 of mild degree, 3 of moderate degree, 5 of severe degree. There was no recurrence and sequelae in the mild-degree patient after 3 years follow-up. There was no recurrence and sequelae in 2 of the moderate-degree patients after 1-2 years follow-up. Different degrees of motor, cognitive sequelae were found in 1 of moderate-degree patients and all of severe-degree patients after 1-8 years follow-up.Conclusions:Childhood OMS patients have early onset age. Bad symptom severity before treatment and multiphase course are associated with poor prognosis. OMS Symptom Severity Standard Rating Scale is suitable for estimations of long-term prognosis.

6.
Article in Chinese | WPRIM | ID: wpr-940635

ABSTRACT

ObjectiveTo explore the possible mechanism of dried fruiting bodies of Fomes officinalis (FOA) against Alzheimer's disease (AD) based on network pharmacology and experimental verification. MethodThe effective components of FOA were retrieved from a Bioinformatics Analysis Tool for Molecular mechANism of Traditional Chinese Medicine (BATMAN-TCM) and previous reports. The targets of the components were searched from PharmMapper and TargetNet, and the targets related to AD from Gene Expression Omnibus (GEO), DrugBank, among other databases. Thereby, the common targets of FOA and AD were obtained, and the protein-protein interaction (PPI) network and component-target network were established based on STRING and Cytoscape 3.7.1, followed by the topology analysis of the networks, and Gene Ontology (GO) term enrichment and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis of the common targets. The results were verified by the molecular docking and the in vitro cell experiment. ResultA total of 24 candidate components and 242 predicted targets of FOA, and 96 common targets of FOA and AD were screened out. The key components included [2-(1-carboxyhexadecylamino)-2-aminosuccinic acid], 3-keto-dehydrosulfurenic acid, and eburicoic acid, and the active targets were albumin (ALB), acetylcholinesterase (AChE), estrogen receptor 1 (ESR1), cysteine aspartate-specific protease-3 (Caspase-3), and beta-secretase1 (BACE1). The common targets were involved in 392 GO terms, and the key terms were the β-amyloid metabolic process and cholinesterase activity. A total of 77 KEGG pathways were obtained, which mainly included estrogen signaling pathway, cholinergic synapse, and AD. The results of molecular docking showed that 7 components of FOA had high binding affinity to amyloid precursor protein (APP), BACE1, AChE, and Caspase-3. The cell survival rate rose (P<0.01) and the mRNA and protein expression of APP, BACE1, AChE, and Caspase-3 reduced in FOA groups in a dose-dependent manner compared with those in the model group (P<0.05). ConclusionThis study reveals for the first time that FOA has multi-component, multi-target, and multi-pathway characteristics in the treatment of AD, which serves as a reference for further explaining the mechanism of FOA against AD.

7.
Article in Chinese | WPRIM | ID: wpr-940344

ABSTRACT

ObjectiveTo study the inhibitory effect of Banxia Houputang (BHT) on lipopolysaccharide (LPS)-induced inflammation of microglia (BV2) cells and the neuroprotective effect on human neuroblastoma (SH-SY5Y) cells. MethodAfter the neuroinflammatory model was constructed by LPS inducing BV2 cells, model group (LPS 100 µg·L-1), administration groups (LPS+1 g·L-1 BHT, LPS+2 g·L-1 BHT, LPS+5 g·L-1 BHT, LPS+10 g·L-1 BHT), and blank group were given DEME medium at the same volume. In addition, neuronal apoptosis model was established by co-culture of LPS-induced BV2 cell inflammation medium and SH-SY5Y cells (LPS-DMEM) and was administrated according to the above grouping. Cell viability was detected by Cell Counting Kit-8 (CCK-8) assay. The content of nitric oxide (NO) and that of tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β), and interleukin-6 (IL-6) were determined by Griess aasay and enzyme-linked immunosorbent assay (ELISA), respectively. The mRNA levels of TNF-α, IL-1β, interleukin-4 (IL-4), nitric oxide synthase (iNOS), and interleukin-10 (IL-10) were measured by real-time polymerase chain reaction (Real-rime PCR). Western blot was used to detect the expression levels of signal transducer and activator of transcription 3 (STAT3), Janus kinase 2 (JAK2) and nuclear factor kappa-B (NF-κB p65), protein kinase B (Akt), inhibitor of nuclear factor κB α (IκBα), B-cell lymphoma-2 (Bcl-2), and Bcl-2 associated X protein (Bax). ResultCompared with blank group, LPS increased the NO release, levels of TNF-α, IL-1β, IL-6, and iNOS and protein expression of Akt, NF-κB p65, IκBα, JAK2 and STAT3, decreased the content of IL-4 and IL-10 in BV2 cells, and induced apoptosis of co-cultured SH-SY5Y cells (P<0.01). Compared with model group, BHT reduced the content of NO, TNF-α, IL-1β, and iNOS (P<0.01) and protein expression of Akt, NF-κB p65, IκBα, JAK2 and STAT3 (P<0.01), elevated the content of IL-4 and IL-10 (P<0.01), and inhibited the apoptosis of SH-SY5Y cells induced by LPS-DMEM (P<0.01). ConclusionThis experiment reveals that BHT inhibited LPS-induced inflammation in BV2 cells by regulating Akt/NF-κB/JAK2/STAT3 signaling pathway and showed neuroprotective effects on SH-SY5Y cells.

8.
São Paulo; s.n; s.n; 2022. 74 p. tab, graf, ilus.
Thesis in Portuguese | LILACS | ID: biblio-1378473

ABSTRACT

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


Subject(s)
Animals , Male , Female , Mice , Receptors, Purinergic P2X7/analysis , Receptors, Purinergic P2Y2/analysis , Tumor-Associated Macrophages/pathology , Macrophages/drug effects , Neuroblastoma/pathology , Training Support/classification , Bone Marrow , Cells/chemistry , Inflammation
9.
Article in Chinese | WPRIM | ID: wpr-933302

ABSTRACT

Objective:To evaluate the role of has_circ_0008039 and miR-484 in oxygen-glucose deprivation/reoxygenation (OGD/R) injury in SK-N-SH cells and the relationship with Fis1.Methods:SK-N-SH cells were cultured in vitro to logarithmic growth stage and divided into 5 groups ( n=25 each) according to the random number table method: control group (group C), OGD/R group, has_circ_0008039 siRNA group (group S), hsa_circ_0008039 over-expression group (group E) and has_circ_0008039 siRNA plus miR-484 inhibitor group (group S+ I). Cells were cultured in normal condition in group C. In S, E and S+ I groups, after the cells were transfected with hsa_circ_0008039 siRNA, has_circ_0008039 over-expression vector, hsa_circ_0008039 siRNA and miR-484 inhibitor, the cells were subjected to oxygen-glucose deprivation for 12 h followed by 24 h restoration of O 2-glucose supply to develop the OGD/R model.At 24 h of restoration of O 2-glucose supply, the cell viability and amount of lactic dehydrogenase (LDH) released were measured using CCK-8 assay, the expression of hsa_circ_0008039, miR-484 and Fis1 mRNA was detected using real-time polymerase chain reaction, and the expression of Fis1 was detected by Western blot.A dual-fluorescein experimental report was used to verify the targeting relationship between hsa_circ_0008039 and miR-484. Results:Compared with group C, the cell viability was significantly decreased, and the amount of LDH released was increased in the other 4 groups, the expression of hsa_circ_0008039 and Fis1 was significantly up-regulated, and the expression of miR-484 was down-regulated in OGD/R and E groups, the expression of hsa_circ_0008039 and Fis1 was significantly down-regulated, and miR-484 was up-regulated in group S, and the expression of hsa_circ_0008039 and miR-484 was significantly down-regulated, and the expression of Fis1 was up-regulated in group S+ I ( P<0.05). Compared with group OGD/R, the cell viability was significantly decreased, and the amount of LDH released was increased in E and S+ I groups, the cell viability was significantly increased, and the amount of LDH released was decreased in group S, the expression of hsa_circ_0008039 and Fis1 was significantly up-regulated, and the expression of miR-484 was down-regulated in group E, the expression of hsa_circ_0008039 and Fis1 was significantly down-regulated, and the expression of miR-484 was up-regulated in group S, and the expression of hsa_circ_0008039 and miR-484 was significantly down-regulated, and the expression of Fis1 was up-regulated in group S+ I ( P<0.05). Compared with group S, the cell viability was significantly decreased, the amount of LDH released was increased, the expression of miR-484 was down-regulated, and the expression of Fis1 was up-regulated in group S+ I ( P<0.01). The dual-fluorescein experimental report verified that miR-484 was the target of hsa_circ_0008039 which binded to miR-484 specifically. Conclusions:has_circ_0008039 is involved in OGD/R injury in SK-N-SH cells by targetedly binding to miR-484, which is associated with up-regulation of Fis1 expression.

10.
Article in Chinese | WPRIM | ID: wpr-931644

ABSTRACT

Objective:To investigate the clinical characteristics of infantile neuroblastoma (NB) and the factors that affect prognosis.Methods:We retrospectively analyzed the clinical data collected from 44 cases of NB who received treatment in The Fourth Affiliated Hospital of China Medical University from March 2008 to March 2017 to summarize the clinical characteristics of NB and the factors that affect prognosis.Results:Among the 44 cases, 21 were male and 23 were female, with a median age of 5.5 months (range, 0 days-12 months). Four cases had stage I NB, seven cases stage II NB, five cases stage III NB, 15 cases stage IV NB, and 13 cases stage IVs NB. The tumors were located in the adrenal glands and retroperitoneum ( n = 26, 59.1%), posterior mediastinum ( n = 15, 34.1%), pelvis ( n = 2, 4.5%), and neck ( n = 1, 2.3%). The median follow-up time was 90 months (range, 2-144 months). The 3-year and 5-year overall survival rates were 93.2% and 90.9%, respectively. Among 35 cases who survived more than 5 years, 30 cases survived healthily, 5 cases survived with tumor, and 4 cases died. Bone marrow metastasis, bone metastasis, and the extent of tumor resection greatly affect the prognosis of NB ( χ2 = 6.92, 12.19, 4.70, all P < 0.05). Conclusion:The overall prognosis of NB is good in infants. NB mainly occurs in the abdomen. The survival rate of infants with stage IVs NB is lower than that of infants with stages I, II, and III NB. The prognosis of NB occurring in the abdomen is poorer than that occurring in other regions. Bone marrow metastasis, bone metastasis, and the extent of tumor resection are adverse factors affecting the prognosis.

11.
Article in Chinese | WPRIM | ID: wpr-930511

ABSTRACT

Objective:To summarize the clinical features of neuroblastoma (NB) with bone metastasis in infants and the prognostic factors.Methods:A retrospective analysis was performed on 32 patients aged ≤12 months who were enrolled in Beijing Children′s Hospital, Capital Medical University from January 2010 to December 2019 and had imaging findings suggesting signs of distant bone metastasis.The control group was included NB children, aged ≤12 months, who were admitted to Beijing Children′s Hospital, Capital Medical University during the same period, without signs of distant bone destruction.The clinical manifestations and auxiliary examinations of infants with bone metastasis were summarized, and the efficacy evaluation and survival analysis of infants with regular treatment and follow-up were conducted until December 31, 2020. Kaplan- Meier survival analysis was used for prognostic analysis, and Log Rank test was used for univariate prognostic analysis. Results:There were 32 NB infants with bone metastases, involving 12 males (37.5%) and 20 females (62.5%), accounting for 16.0% (32/200 cases) of infants diagnosed with NB du-ring the same period.The median age of onset was 9 (4.5-12.0) months.The main primary site included the retroperitoneal and adrenal region in 24 cases(75.0%) and mediastinum in 3 cases (9.4%). Among the 32 cases, 14 cases (43.8%) had simple bone metastasis, 19 cases (59.4%) had distant lymph nodes, 18 cases (56.3%) had bone marrow, and 3 cases (9.4%) had intracranial and meningeal metastasis.Bone metastasis mainly occurred in the skull, with 11 cases of single bone metastases and the remaining with 2 or more bone metastases.Compared with 168 NB infants without bone metastasis, the prognosis of those with bone metastasis was significantly worse [3-year overall survival(OS) rate 97.6% vs.82.7%, P=0.001]. Univariate analysis showed that the prognosis of NB children with bone marrow metastasis, meningeal and intracranial metastasis, MYCN gene amplification, and high-risk group was poor (all P<0.05). Two patients returned to the local hospital for treatment after diagnosis.A total of 30 children were recruited for efficacy evaluation and prognostic analysis.Twenty-nine children underwent surgery, of which 6 cases received surgery before chemotherapy and 23 cases received surgery after chemotherapy.One case received chemotherapy only.The mean course of chemotherapy was 6.2 (4-13) times.One case was treated with radiotherapy, 1 case was treated with Metaiodobenzylguanidine (MIBG) therapy, and 1 case was treated with stem cell transplantation.A total of 18 cases (62.1%) event-free survived, and 12 cases (40.0%) had a mean event at 7 (1.5-32.0) months.Among them, 7 cases survived and 5 cases died (16.7%). The expected 3-year event-free survival rate and OS rate were 57.1% and 82.7%, respectively. Conclusions:The most common sites of infant NB metastasis are bone and bone marrow, and the most common sites of bone metastasis are skull.Infants with bone metastasis had a worse prognosis than those without bone metastasis, and infants with bone and bone marrow metastasis had a worse prognosis than infants with single bone metastasis.

12.
Article in Chinese | WPRIM | ID: wpr-930422

ABSTRACT

Objective:To explore the clinical significance of the MYCN gene, PHOX2B gene and plasma cell-free DNA (cfDNA) in risk stratification and predicting the prognosis of high-risk neuroblastoma (NB). Methods:This was a prospective study involving 94 high-risk NB children admitted to Beijing Children′s Hospital, Capital Medical University from August 2017 to December 2018.Relative levels of MYCN and PHOX2B and cfDNA at diagnosis, and 4 and 6 cycles of chemotherapy were detected, and their differences were compared by the Chi- square test.Kaplan-Meier survival analysis was performed to explore their prognostic potential in high-risk NB. Results:Among the 94 high-risk NB children, 14 cases (14.9%) had MYCN amplification, 76 cases (80.8%) had positive expression of PHOX2B and 56 cases (59.6%) had cfDNA level higher than 100 μg/L.The proportion of high lactate dehydrogenase (LDH, ≥1 500 U/L) level in the MYCN gene amplification group (6/14 cases) was higher than that in the normal group (9/80 cases) ( P=0.009). The proportion of multi-site metastasis (54/76 cases) and high neuron specific enolase (NSE) level (NSE≥370 μg/L, 37/76 cases) in PHOX2B positive group were significantly higher than those in the negative group (5/14 cases, 2/14 cases) ( P=0.015, 0.020). The proportion of high LDH and high NSE in high cfDNA concentration (≥229.6 μg/L)group (13/37 cases, 28/37 cases) were significantly higher than those in low cfDNA concentration group (2/48 cases, 10/48 cases) (all P<0.001). With the decreased tumor burden during the treatment, the copy number of PHOX2B gene and cfDNA level were significantly lower than those at the initial diagnosis [0 (0-719.6) copies vs.1 723.5 (0-186 000.0) copies; 19.0 (1.1-225.5) μg/L vs.200.6 (8.0-5 247.4) μg/L, all P<0.001]. The 2-year event-free survival (EFS) rate of the MYCN gene amplification group was significantly lower than that of the normal group[(33.3±13.1)% vs.(58.5±7.1)%, P=0.020]. The 2-year EFS rate of PHOX2B positive group was significantly lower than that of the negative group[(47.9±7.1)% vs.(79.1±11.1)%, P=0.043]. EFS rate in high cfDNA concentration group was significantly lower than that in cfDNA low concentration group[(38.6±9.8)% vs.( 71.7±8.2)%, P=0.001]. After 6 cycles of chemotherapy, EFS rate in the PHOX2B positive group was significantly lower than that in the negative group [(16.7±14.4)% vs.( 60.6±6.6)%, P=0.014]; which was significantly lower in the Metaiodobenzylguanidine (MIBG) positive group than that of the negative group[(35.2±11.7)% vs.(65.8±7.1)%, P=0.037]. The MYCN gene and cfDNA concentration were not correlated with the prognosis of high-risk NB.Survival analysis of the combination of PHOX2B and MYCN gene ( PHOX2B+ /MIBG + , PHOX2B+ or MIBG + , PHOX2B-/MIBG -) showed a significant difference in the survival among three groups[0 vs.(53.6±1.2)% vs.(65.5±7.4)%, P=0.003]. Conclusions:The MYCN and PHOX2B gene and cfDNA concentration are of significance in risk stratification and predicting the prognosis of high-risk NB.Compared with the MYCN gene and cfDNA concentration, the PHOX2B gene is more suitable for monitoring the curative effect of chemotherapy on high-risk NB.A combined analysis of PHOX2B gene and MIBG before treatment can be more accurate in evaluating the treatment effect and residual lesions.

13.
Article in Chinese | WPRIM | ID: wpr-928885

ABSTRACT

With the advent of the era of artificial intelligence, as an emerging technology, radiomics can extract a large amount of quantitative information describing the physiological condition and phenotypic characteristics of tumors with high throughput from the massive data of CT, MRI and other imaging tomography, and analyze these high-dimensional imaging omics features containing disease pathophysiological information can be used to accurately determine tumor differentiation, staging, and predict tumor behavior, which has broad application prospects. This article aims to introduce the technical principles of radiomics and its abdominal tumor application status, and to prospect its application prospects in pediatric abdominal neuroblastoma.


Subject(s)
Abdominal Neoplasms/diagnostic imaging , Artificial Intelligence , Child , Humans , Magnetic Resonance Imaging , Precision Medicine , Tomography, X-Ray Computed
14.
Article in English | WPRIM | ID: wpr-928610

ABSTRACT

Neuroblastoma (NB) is the most common extracranial solid tumor in children and has the features of high recurrence rate and low survival rate, and therefore, early diagnosis, treatment response evaluation, and recurrence monitoring are of great significance for NB patients. Liquid biopsy refers to the detection of cells and nucleic acids in fluid specimens, mainly blood. It is noninvasive and can overcome tumor heterogeneity, thus making it possible to achieve the early diagnosis and dynamic detection of NB. This review introduces the latest advances in clinical research on the application of liquid biopsy in NB.


Subject(s)
Child , Humans , Liquid Biopsy , Neuroblastoma/diagnosis
15.
Article in Chinese | WPRIM | ID: wpr-939659

ABSTRACT

OBJECTIVES@#To study the early clinical efficacy of combined therapy of stage 4 neuroblastoma.@*METHODS@#A retrospective analysis was performed on the medical data and follow-up data of 14 children with stage 4 neuroblastoma who were diagnosed in Hong Kong University-Shenzhen Hospital from January 2016 to June 2021.@*RESULTS@#The median age of onset was 3 years and 7.5 months in these 14 children. Among these children, 9 had positive results of bone marrow biopsy, 4 had N-Myc gene amplification, 13 had an increase in neuron-specific enolase, and 7 had an increase in vanilmandelic acid in urine. Based on the results of pathological examination, differentiated type was observed in 6 children, undifferentiated type in one child, mixed type, in one child and poorly differentiated type in 6 children. Of all the children, 10 received chemotherapy with the N7 regimen (including 2 children receiving arsenic trioxide in addition) and 4 received chemotherapy with the Rapid COJEC regimen. Thirteen children underwent surgery, 14 received hematopoietic stem cell transplantation, and 10 received radiotherapy. A total of 8 children received Ch14.18/CHO immunotherapy, among whom 1 child discontinued due to anaphylactic shock during immunotherapy, and the other 7 children completed Ch14.18/CHO treatment without serious adverse events, among whom 1 child was treated with Lu177 Dotatate 3 times after recurrence and is still undergoing chemotherapy at present. The median follow-up time was 45 months for all the 14 children. Four children experienced recurrence within 2 years, and the 2-year overall survival rate was 100%; 4 children experienced recurrence within 3 years, and 7 achieved disease-free survival within 3 years.@*CONCLUSIONS@#Multidisciplinary combined therapy is recommended for children with stage 4 neuroblastoma and can help them achieve better survival and prognosis.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Child , Child, Preschool , Combined Modality Therapy , Humans , Infant , Neuroblastoma/drug therapy , Positron-Emission Tomography , Radionuclide Imaging , Retrospective Studies , Treatment Outcome
16.
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
17.
Article in Chinese | WPRIM | ID: wpr-908029

ABSTRACT

Objective:To explore the application value of 3D printing technology in the precise resection of pediatric retroperitoneal neuroblastoma(NB).Methods:Eleven patients with retroperitoneal NB admitted to the Department of Pediatric Surgery of Henan Provincial People′s Hospital from March 2017 to December 2019 were selected in this study, and all of them received preoperative plain and enhanced computed tomography (CT) scanning of whole abdomen.The original data were imported into the mimics 20 software for processing.The tumor and important peripheral blood vessels were reconstructed with such commands as multi-layer edition, threshold adjustment, regional growth and 3D edition to output the standardized 3D printing stl format file and print out the tumor model by 3D printer.Based on the 3D reconstruction results, the precise surgical planning was performed to determine the optimal operative scheme.The data of all cases were analyzed retrospectively.The parents of these children were investigated by questionnaire and the results were statistically analyzed.Results:All 11 patients successfully underwent the operation under the planning of 3D printing technology.Nine of them had complete tumor resection and 2 cases had palliative resection.The operation time ranged from 140 min to 750 min, with an average of 210 min; the bleeding volume ranged from 100 mL to 1 500 mL, with an average of 240 mL; the postoperative pathological reports revealed that 9 children had NB and 2 cases had ganglioneuroblastoma.There was no case of perioperative death.There were 2 cases of postoperative complications, including 1 case of severe diarrhea with chylous leakage, which was relieved after the treatment of parenteral nutrition and somatostatin, and 1 case of left lower extremity weight-bearing difficulty after left iliac artery anastomosis, which was improved after rehabilitation physical therapy.The follow-up time ranged from 1 month to 30 months.There were 2 cases recurred in the original operation area.One patient received reoperation after chemotherapy; the other continued chemotherapy in progress.As for the 2 cases of palliative resection, they received chemotherapy and survived with tumor.After using 3D model to communicate with children′s parents, their awareness rate of disease, operation mode and postoperative complications were significantly higher than that of CT.Conclusions:3D printing technology can accurately evaluate the structural relationship between retroperitoneal NB and important peripheral blood vessels in children, which contributes to improving the parents′ cognition of the operation mode, and is conducive to selecting the optimal operation scheme and achieving precise tumor resection.

18.
Article in Chinese | WPRIM | ID: wpr-908027

ABSTRACT

Objective:To summarize the clinical characteristics of single-center children with low and intermediate-risk neuroblastoma (NB), report the long-term follow-up results of the growth and survival quality, and provide a basis for further clinical research.Methods:Clinical characteristics, including the sex, age, stage, risk of disease, and metastatic site of 370 newly treated children with low and intermediate-risk NB admitted to Hematology Oncology Center, Beijing Children′s Hospital from March 2007 to June 2019 were retrospectively analyzed.Kaplan-Meier method was used for survival analysis.WHO Anthro Plus was used for calculating Z score.Results:A total of 370 eligible children with low and intermediate-risk NB were included, with the mean age at onset of 16.8 months (1-191 months). Among them, 148 cases (40%) were younger than 12 months old.Mediastinal region was the most common primary site of NB (47.8%, 177 cases), followed by retroperitoneum/adrenal gland (41.4%, 153 cases). The median follow-up time of 370 patients was 31 months (0.3-157.0 months), the 5-years event free survival (EFS) and 5-year overall survival (OS) were 86.2% and 96.9%, respectively.Thirty-seven cases had growth and deve-lopment problems, of which 22 cases had stunted growth, 6 cases had low body mass, 9 cases had wasting, and 7.3%(27/370 cases) had scoliosis.5.5% of them had heart damage and 5.0%(18/357 cases) had kidney damage, involving 12 cases related to the primary tumor and 6 cases were surgically related.30.2%(95/315 cases) of them had hair changed after chemotherapy, and curly hair was the most common change.Compared with before treatment, 14.9% of the children had a personality change, with an impatient being the most common.Conclusions:The 5-year overall survival rate of the single-center large sample of low and intermediate-risk NB was high, mediastinal was the most common primary site of tumor, and the long-term quality of life is good, but there were still treatment-related side effects, and further clinical monitoring and long-term follow-up were needed.

19.
Article in Chinese | WPRIM | ID: wpr-907918

ABSTRACT

Objective:To summarize the causes of death and severe complication in the early diagnosis of children with neuroblastoma (NB), and to analyze the relative factors of early death of children with NB, so as to raise awareness and reduce early mortality by early detection and early intervention.Methods:Patients with newly diagnosed NB in the Hematology Oncology Center of Beijing Children′s Hospital from April 2007 to December 2017 were included consecutively, and those died within 1 month after diagnosis were retrospectively analyzed.The general data of patients, immediate causes of death, complications, time elapsed between death and diagnosis, whether to receive chemotherapy and other information were collected.Results:A total of 654 cases were included for diagnosis, treatment and follow-up, 31 cases of which died in early stage, accounting for 4.7% of the total.The major complication were pulmonary infection in 18 cases (58.1%) and bone marrow suppression after chemotherapy in 17 cases (54.8%), tumor rupture hemorrhage in 16 cases (51.6%), multiple organ failure in 8 cases (25.8%). Risk factor analysis of the 31 early death cases with NB was conducted.Single factor analysis: there were statistical differences between early death group and non-early death group in risk grouping ( P=0.006 6), bone marrow invasion ( P=0.020 7), site of primary tumor ( P=0.016 7), age ( P=0.003 3), lactate dehydrogenase (LDH) level ( P<0.000 1), neuron-specific enolase (NSE) level ( P<0.000 1), serum ferritin level ( P=0.016 0), D dimer level ( P<0.000 1), fibrinogen level ( P=0.002 7), diameter of tumor ( P<0.000 1), hemoglobin ( P<0.000 1), platelet level ( P<0.000 1), serum albumin level ( P<0.000 1). Multiple-factor analysis: age younger than 30 months, OR=2.824 (95% CI: 1.084-7.359), LDH level greater than 1 004 IU/L, OR=6.991 (95% CI: 2.135-22.887), albumin level less than 36 g/L, OR= 65.237 (95% CI: 2.024-13.545), hemoglobin level less than 92 g/L, OR=5.358 (95% CI: 2.024-13.545), platelet level less than 192×10 9/L, OR=3.554 (95% CI: 1.267-9.965). Conclusions:Strengthening vital signs detection after admission, identifying severe life-threatening complications such as rupture of tumors as early as possible, implementing symptomatic interventions such as appropriate sedation and active transfusion of blood products as early as possible after invasive operation, and transferring to intensive care unit for respiratory support when necessary are important means to avoid early death.

20.
Article in Chinese | WPRIM | ID: wpr-907910

ABSTRACT

Objective:To summarize the clinical characteristics, treatment response and long-term postoperative complications in children with neuroblastoma (NB) in the pelvic and sacral regions as the primary site.Methods:The clinical characteristics of 16 NB children (8 males and 8 females) with primary pelvic and sacral admitted to the Department of Hematology Oncology Center in Beijing Children′s Hospital, Capital Medical University from March 2007 to June 2019 were analyzed retrospectively with respect to the age at first diagnosis, primary tumor site, tumor size, clinical stage, risk grouping, and other clinical characteristics.The clinical characteristics of the patients who were followed up for regular treatment were analyzed, and the postoperative complications of the patients were summarized, and the Kaplan-Meier method was used for survival analysis.Results:The median age at diagnosis of these 16 children was 23.0 months (5.7-102.0 months), of which 6 cases (37.5%) were younger than 12 months old.All these children received chemotherapy, with a median of 6 (1-8) courses of chemotherapy.Fifteen children received surgical resection of the pelvic tumor, with complete resection in 12 cases (80%). The surgical approach was mainly transabdominal (86.7%, 13/16 cases). The median follow-up time of these children was 33.5 (8-136) months.The patella was absent in 3 patients (18.8%) after the operation, and no permanent neurological damage occurred in all patients.Five-year overall survival (OS) rate was 100%.Conclusions:A single-center summary showed a high survival rate for NB patients in the pelvic and sacral regions.Complete tumor resections combined with chemotherapy could be effective measures and rare cases occurred permanent postoperative neurological complications.

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