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Background: Moringa peregrina is widely used in the traditional medicine of the Arabian Peninsula to treat various ailments, because it has many pharmacologically active components with several therapeutic effects. Objective: This study aimed to investigate the inhibitory effect of Moringaperegrina seed ethanolic extract (MPSE) against key enzymes involved in human pathologies, such as angiogenesis (thymidine phosphorylase), diabetes (α-glucosidase), and idiopathic intracranial hypertension (carbonic anhydrase). In addition, the anticancer properties were tested against the SH-SY5Y (human neuroblastoma). Results: MPSE extract significantly inhibited α-glucosidase, thymidine phosphorylase, and carbonic anhydrase with half-maximal inhibitory concentrations (IC50) values of 303.1 ± 1.3, 471.30 ± 0.3, and 271.30 ± 5.1 µg/mL, respectively. Furthermore, the antiproliferative effect of the MPSE was observed on the SH-SY5Y cancer cell line with IC50 values of 55.1 µg/mL. Conclusions: MPSE has interesting inhibitory capacities against key enzymes and human neuroblastoma cancer cell line.
Antecedentes: La Moringa peregrina se utiliza ampliamente en la medicina tradicional de la Península Arábiga para tratar diversas dolencias, ya que posee numerosos componentes farmacológicamente activos con varios efectos terapéuticos. Objetivo: Este estudio tenía como objetivo investigar el efecto inhibidor del extracto etanólico de semillas de Moringaperegrina (MPSE) frente a enzimas clave implicadas en patologías humanas, como la angiogénesis (timidina fosforilasa), la diabetes (α-glucosidasa) y la hipertensión intracraneal idiopática (anhidrasa carbónica). Además, se comprobaron las propiedades anticancerígenas frente al SH-SY5Y (neuroblastoma humano). Resultados: El extracto de MPSE inhibió significativamente la α-glucosidasa, la timidina fosforilasa y la anhidrasa carbónica con concentraciones inhibitorias semimáximas (IC50) de 303,1 ± 1,3, 471,30 ± 0,3 y 271,30 ± 5,1 µg/mL, respectivamente. Además, se observó el efecto antiproliferativo del MPSE en la línea celular del cáncer SH-SY5Y con valores de IC50 de 55,1 µg/mL. Conclusiones: MPSE posee interesantes capacidades inhibitorias frente a enzimas clave y línea celular de neuroblastoma canceroso humano.
Subject(s)
Humans , Anticarcinogenic Agents , Moringa , Enzyme Inhibitors , alpha-GlucosidasesABSTRACT
Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.
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Background: Peripheral neuroblastic tumors arise from the sympathoadrenal lineage of the neural crest. They have been classified according to the International Neuroblastoma Pathology Committee (INPC) into Four categories according to International Neuroblastoma Pathology Committee (INPC): a) Neuroblastoma (NB) b) Ganglioneuroblastoma (GNB), nodular c) Ganglioneuroblastoma, intermixed, and d) Ganglioneuroma (GN). Because of the rarity of extra-adrenal peripheral neuroblastic tumors, limited information is available regarding the chemotherapy of NB and GNB. A few case reports or case series with a small number of patients have been documented in the literature. Aim: To describe the clinicopathological characteristics of extra-adrenal peripheral neuroblastic tumors. Materials and Methods: Clinical, histopathological, and immunohistochemistry (IHC) findings of 18 cases were retrieved. Immunohistochemistry at the time of diagnosis was performed using Ventana Benchmark XT. The mean value was calculated using the Microsoft Office Excel 2019 software. Results: The posterior mediastinum was the most commonly affected extra-adrenal site in our study. Neuroblastoma consisted of eight cases (six in children, two in adults), of which four cases were poorly differentiated and the other four cases were differentiating. Two cases had favorable histology. The bone marrow and cervical lymph node metastasis were documented. Of the four GNB cases, one patient developed bone metastasis. All patients of NB and GNB received combination chemotherapy. One out of six GN patients presented with a large retroperitoneal mass encasing the aorta and renal vessels, mimicking a sarcoma. Conclusion: Extra-adrenal peripheral neuroblastic tumors do not pose any diagnostic issue in adequate tissue sampling. In limited material, immunohistochemistry is needed. The chemotherapy regimen has not been standardized due to rarity. Further molecular testing and targeted therapy may be of help in the future.
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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.
Subject(s)
Magnetic Resonance Imaging , Neuroblastoma , Secondary Care , InfantABSTRACT
Neuroblastoma is one of the most common tumors in children. Cases where an isolated soft-tissue metastasis mass is the initial symptom are rare, with only four such cases reported to date. We describe the imaging findings of ten cases of neuroblastoma patients in our hospital with superficial soft tissue mass (SSTM) as the primary symptom. The main ultrasound finding of SSTM was hypoechoic masses or scattered speck-like hyperechoic masses. However, when this type of SSTM is caused by soft tissue metastasis, the location is often atypical, and ultrasound findings are difficult to distinguish from other benign diseases. Therefore, this research should remind clinicians to recognize atypical presentations of this common childhood malignant tumor. Radiologists should also consider the possibility of neuroblastoma when finding this type of SSTM with atypical ultrasound features.
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INTRODUCTION@#In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor.@*METHODS@#A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors.@*RESULTS@#There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%.@*CONCLUSION@#Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.
Subject(s)
Child , Humans , Male , Infant , Child, Preschool , Adolescent , Disease-Free Survival , Neuroblastoma/pathology , Hematopoietic Stem Cell Transplantation/methods , Treatment Outcome , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Asia, Southeastern/epidemiology , Combined Modality TherapyABSTRACT
High-risk neuroblastoma (NB) is highly aggressive and has poor prognosis. Treatment of NB mainly includes comprehensive therapies, of which radiotherapy serves as a part of consolidation therapy. For patients who receive complete resection of the primary lesion, usually an irradiation dose of 21-23.4 Gy is given; for patients with incomplete resection, further study focused on radiation dose is necessary. Recurrence is most commonly observed in the bone lesions involved at presentation. Currently, the principle of irradiation to the metastatic sites is to treat lesions where metaio-dobenzylguanidine (MIBG) uptake remains positive after induction chemotherapy, or those become negative uptake but still at high risk of recurrence. On the premise of lacking of MIBG imaging, positron emission tomography CT (PET-CT) may assist in screening for metastatic sites requiring irradiation. The late side effects of radiotherapy are mainly mild musculoskeletal abnormalities. No significant increase is observed in the incidence of second primary tumor during short-term follow-up.
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Objective:To explore the clinical features, risks of recurrence and prognosis of neuroblastoma (NB) with intraspinal extension in children, and to perform a long-term follow-up to monitor their health problems.Methods:Clinical data of 22 children with NB with intraspinal extension treated in Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2007 to December 2019 were retrospectively analyzed, including gender, age at diagnosis, clinical stage, and risks of recurrence.They were divided into non-recurrent group and recurrent group, and the survival was analyzed by Kaplan-Meier method.Results:(1)Fifteen(68.2%) children had motor nervous symptoms as the initial symptoms, including lower limb pain, weakness, and limited mobility, and 10(45.5%) showed moderate-to-severe nervous compression.(2)Neuronal enolase(NSE) (<200 μg/L), lactate dehydrogenase(LDH) (≤500 U/L) and vanillylmandelic acid(VMA) were in the normal range in most of children in the non-recurrence group, and a single site was involved.(3)Tumor lesions were not completely resected in the majority of children in the recurrent group, and their NSE (≥200 μg/L), LDH (>500 U/L) and VMA (more than 3 times higher) were abnormally higher, or suffered multisite pyramidal metastasis.(4)The median survival time (MST) was 119.4 months for children in the non-recurrent group, while it was only 25.3 months for those in the recurrent group.The 3-year overall survival (OS) rate was (95.5±6.4)% for the non-recurrent group, compared to only (20.0±17.9)% for children in the recurrent group ( χ2=9.387, P=0.002). Likewise, the 3-year event-free survival (EFS) rate for children in the non-recurrent group was (94.1±5.7)%, whereas it was only (20.0±17.9)% for children in the recurrent group( χ2=29.700, P<0.001). (5)Eleven of 22 children had long-term health problems, especially motor nerve function defects and scoliosis. Conclusions:Motor neurological, and moderate-to-severe neurological compression predominates are the main symptoms of NB children with intraspinal extension at the initial diagnosis.Patients who exhibit intradural tumor remnants, higher NSE, LDH, or VMA levels, and intradural extension with multiple sites of cone metastases are prone to recurrence.Once relapsed, the prognosis is extremely poor.The prognosis of NB with intraspinal extension in children is related to the severity of the initial neurological impairment, the duration of tumor compression, and the treatment regimen.Early diagnosis and intervention may reduce the risk of long-term health problems in children.
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Objective:To analyze the clinical characteristics and prognostic factors of high-risk neuroblastoma (HR-NB) patients with skeletal metastasis.Methods:The clinical features of 336 newly treated HR-NB patients with skeletal metastases admitted to the Department of Medical Oncology of Beijing Children′s Hospital, Capital Medical University from January 2007 to December 2018 were analyzed retrospectively.Kaplan-Meier method was used for the survival analysis, and Log- Rank test was used for univariate prognosis analysis.The Cox model was used to analyze the multifactorial prognostic analysis. Results:A total of 336 HR-NB patients were recruited, involving 188 males and 148 females with the median age of onset of at 43 (4-148) months.Skeletal metastases affected the viscerocranium (89 cases, 26.5%), neurocranium (193 cases, 57.4%), vertebrae (298 cases, 88.7%), sternum and ribs (183 cases, 54.5%), pelvis (270 cases, 80.4%), upper limbs (182 cases, 54.2%) and lower limbs (240 cases, 71.4%). The 5-year event-free survival (EFS) rate and overall survival (OS) rate were (30.4±2.7)% and (41.3±2.9)%, respectively.Univariate analysis showed a significantly lower 5-year OS rate in skeletal metastatic HR-NB patients with poor prognostic classification, the morphology of neuroblastoma (stroma-poor) and ganglioneuroblastoma (intermixed), high index of mitosis-karyorrhexis index, lactate dehydrogenase≥587 U/L, serum ferritin≥92 μg/L, MYCN amplification and 1p loss of heterozygosity, and metastases in the viscerocranium, neurocranium, vertebrae, sternum and ribs, pelvis, upper limbs and lower limbs (all P<0.05). The 5-year OS rate of HR-NB patients with all 7 regions of skeletal metastases was only (14.2±5.9)%, which was significantly lower than that in patients with a single region metastasis or multi-region metastases[(66.0±10.2)% vs.(43.6±3.4)%, χ2=45.722, P<0.05]. Cox multifactorial analysis showed that MYCN amplification ( HR=4.165, 95% CI: 2.356-7.363) and the viscerocranium metastasis ( HR=2.560, 95% CI: 1.519-4.315) were the independent risk factors affecting the prognosis of HR-NB patients with skeletal metastases (all P<0.05). Conclusions:The prognosis is extremely poor in HR-NB patients with multiple skeletal metastases at the initial diagnosis.The amplification of MYCN and the viscerocranium metastasis are the poor prognostic factors for HR-NB patients with skeletal metastases.
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Objective:To explore the protective effect of zonisamide (ZNS) on oxygen-glucose deprivation (OGD) cell model of traumatic brain injury (TBI), and its underlying mechanism.Methods:Human neuroblastoma cells (SH-SY5Y) were cultured in vitro and divided into the control group, OGD group, and drug administration group (OGD+ZNS group) according to the random number table method. The OGD method was used to establish a TBI cell model. After modeling, the cell activity, the release of lactate dehydrogenase (LDH), and β-galactosidase staining were detected to evaluate cell function and senescence. Additionally, mitochondrial morphology and potential membrane changes were observed using Mito Tracker Red and JC-1 mitochondrial membrane potential staining. ATP concentration was measured, and protein was extracted from SH-SY5Y cells and then subjected to Western blot analysis to detect endoplasmic reticulum stress-related markers, including glucose-regulated protein 78 (GRP78), C/EBP homologous protein (CHOP), protein disulfide isomerase (PDI), and β-actin.Results:The OGD group had a significantly lower cell survival rate compared to the control group ( P<0.01), while the OGD+ZNS group had a significant higher cell survival rate than the OGD group ( P<0.01). The LDH release rate was significantly higher in the OGD group than in the control group ( P<0.01), while the OGD+ZNS group had a significant lower LDH release rate compared to the OGD group ( P<0.01). Moreover, the cell staining results indicated that compared to the control and OGD+ZNS groups, the cells in the OGD group exhibited significant damage and senescence with darker staining while the mitochondrial staining results demonstrated a significant reduction in mitochondrial linear junctions and decreased mitochondrial activity in the OGD group compared to the control and OGD+ZNS groups. Compared to the control and OGD+ZNS groups, the OGD group exhibited a significant reduction in mitochondrial staining red fluorescence, a significant increase in green fluorescence, and a significant decrease in mitochondrial membrane potential. The OGD group demonstrated a significant decrease in ATP concentration compared to the control group ( P<0.01), whereas the OGD+ZNS group exhibited a significant higher ATP concentration compared to the OGD group ( P<0.01). Western blot analysis revealed significant upregulation of GRP78, CHOP, and PDI in the OGD group compared to the control group (all P<0.05), while in the OGD+ZNS group, the expression levels of these proteins were significantly downregulated compared to the OGD group (all P<0.05). Conclusions:Zonisamide can protect OGD TBI cell model by preserving mitochondrial activity and inhibiting endoplasmic reticulum stress.
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Neuroblastoma(NB)is the most common extracranial solid tumor in children.It is notable for highly heterogeneous and associated with tumor histologic classification and differentiation status, with ganglioneuroma representing fully mature and differentiated NB.Differentiation therapy reduces the adverse reactions caused by treatment without affecting normal cells and tissues by inducing the redifferentiation of NB cells, and has a good development prospect in the maintenance treatment of high-risk NB patients.Therefore, studying the key molecules and signaling pathways affecting NB differentiation is significant to further clarify the pathogenesis and improve the prognosis of neuroblastoma.This article reviews the important molecules related to NB cell differentiation, signaling pathways and the research progress of differentiation induction therapy.
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Objective To explore the technology frontiers for neuroblastoma treatment from the perspective of patent citation network. Methods Through patent analysis for neuroblastoma treatment, highly cited patents and highly cited papers in the citation network were taken as the research objects. The title and abstract of the citing patents were analyzed by text clustering to identify the technology frontiers. Through social network analysis, the core patents were identified from the indices of degree centrality, betweenness centrality, closeness centrality, and eigenvector centrality. Results A total of 6240 patent applications for neuroblastoma treatment were found, including 71304 patent citations and 88698 journal-article citations. Four technology frontiers were identified based on patent citation network, namely, drug target, drug design, tumor-indication expansion, and gene-expression regulation. Three technology frontiers were identified based on journal-article citation network. They were drug target, drug design, and tumor-indication expansion. Conclusion The development of technology for neuroblastoma treatment continues to be active. Drug target and drug design are the most important technology frontiers. This study could provide certain reference for neuroblastoma treatment from the perspective of information science.
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While neuroblastoma accounts for 15% of childhood tumor-related deaths, treatments against neuroblastoma remain scarce and mainly consist of cytotoxic chemotherapeutic drugs. Currently, maintenance therapy of differentiation induction is the standard of care for neuroblastoma patients in clinical, especially high-risk patients. However, differentiation therapy is not used as a first-line treatment for neuroblastoma due to low efficacy, unclear mechanism, and few drug options. Through compound library screening, we accidently found the potential differentiation-inducing effect of AKT inhibitor Hu7691. The protein kinase B (AKT) pathway is an important signaling pathway for regulating tumorigenesis and neural differentiation, yet the relation between the AKT pathway and neuroblastoma differentiation remains unclear. Here, we reveal the anti-proliferation and neurogenesis effect of Hu7691 on multiple neuroblastoma cell lines. Further evidence including neurites outgrowth, cell cycle arrest, and differentiation mRNA marker clarified the differentiation-inducing effect of Hu7691. Meanwhile, with the introduction of other AKT inhibitors, it is now clear that multiple AKT inhibitors can induce neuroblastoma differentiation. Furthermore, silencing AKT was found to have the effect of inducing neuroblastoma differentiation. Finally, confirmation of the therapeutic effects of Hu7691 is dependent on inducing differentiation in vivo, suggesting that Hu7691 is a potential molecule against neuroblastoma. Through this study, we not only define the key role of AKT in the progression of neuroblastoma differentiation but also provide potential drugs and key targets for the application of differentiation therapies for neuroblastoma clinically.
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OBJECTIVES@#To investigate the effectiveness of high-dose chemotherapy combined with autologous hematopoietic stem cell transplantation (ASCT) in the treatment of children with high-risk neuroblastoma (NB).@*METHODS@#A retrospective analysis was performed on 29 children with high-risk NB who were admitted to Shanghai Children's Hospital and were treated with high-dose chemotherapy combined with ASCT from January 2013 to December 2021, and their clinical features and prognosis were analyzed.@*RESULTS@#Among the 29 children treated by high-dose chemotherapy combined with ASCT, there were 18 boys (62%) and 11 girls (38%), with a median age of onset of 36 (27, 59) months. According to the International Neuroblastoma Staging System, 6 children (21%) had stage III NB and 23 children (79%) had stage IV NB, and the common metastatic sites at initial diagnosis were bone in 22 children (76%), bone marrow in 21 children (72%), and intracalvarium in 4 children (14%). All 29 children achieved reconstruction of hematopoietic function after ASCT. After being followed up for a median time of 25 (17, 45) months, 21 children (72%) had continuous complete remission and 8 (28%) experienced recurrence. The 3-year overall survival rate and event-free survival rate were 68.9%±16.1% and 61.4%±14.4%, respectively. Presence of bone marrow metastasis, neuron-specific enolase ≥370 ng/mL and positive bone marrow immunophenotyping might reduce the 3-year event-free survival rate (P<0.05).@*CONCLUSIONS@#Children with high-risk NB who have bone marrow metastasis at initial diagnosis tend to have a poor prognosis. ASCT combined with high-dose chemotherapy can effectively improve the prognosis of children with NB with a favorable safety profile.
Subject(s)
Child, Preschool , Female , Humans , Male , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow Neoplasms/drug therapy , China , Combined Modality Therapy , Disease-Free Survival , Hematopoietic Stem Cell Transplantation , Neuroblastoma/pathology , Prognosis , Retrospective Studies , Stem Cell Transplantation , Transplantation, AutologousABSTRACT
El neuroblastoma es un tumor embrionario que surge de los neuroblastos (células simpáticas pluripotentes) y es la lesión sólida maligna más común de la infancia. Estos tumores pueden aparecer en cualquier sitio del sistema nervioso simpático fetal, pero la glándula suprarrenal está afectada en el 90% de los casos durante el periodo prenatal. Los neuroblastomas quísticos comprenden alrededor del 50% de todos los casos diagnosticados en fetos y su frecuencia es aún mayor en el periodo posnatal. La ecografía puede utilizarse para determinar el tamaño, localización y características ecográficas del tumor. La resonancia magnética fetal puede ser útil para la estadificación y la evaluación de las metástasis. El manejo del embarazo es expectante y rara vez está indicada la interrupción del embarazo. Se presenta un caso de diagnóstico prenatal de neuroblastoma quístico suprarrenal fetal.
Neuroblastoma is an embryonal tumor arising from neuroblasts (pluripotent sympathetic cells) and is the most common malignant solid lesion in childhood. These tumors can arise anywhere in the fetal sympathetic nervous system, but the adrenal gland is affected in 90% of cases during the prenatal period. Cystic neuroblastomas comprise about 50% of all cases diagnosed in fetuses and their frequency is even higher in the postnatal period. Ultrasonography can be used to determine the size, location, and sonographic features of the tumor. Fetal MRI can be useful for staging and evaluation of metastases. Management of pregnancy is expectant and termination of pregnancy is rarely indicated. A case of prenatal diagnosis of fetal adrenal cystic neuroblastoma is presented.
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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 , LeukapheresisABSTRACT
Embryonal tumors are a heterogenous group of neoplasms mostly defined by recurrent genetic driver events. They have been, previously, broadly classified as either medulloblastoma or supratentorial primitive neuroectodermal tumors (PNETs). However, the application of DNA methylation/gene expression profiling in large series of neoplasms histologically defined as PNET, revealed tumors, which showed genetic events associated with glial tumors. These findings led to the definitive removal of the term “PNET” in the 2016 World Health Organization (WHO) classification of CNS tumors. Moreover, further studies on a large scale of methylation profiling have allowed the identification of new molecular-defined entities and have largely influenced the 5th edition of the WHO classification of CNS tumors (WHO CNS5) for both medulloblastomas and other CNS embryonal tumors. The importance of molecular characteristics in CNS embryonal tumors is well represented by the identification of different molecular groups and subgroups in medulloblastoma. So, in the CNS5, the emerged group 3 and group 4 belong to the classification, and the four molecular and morphologic types are now combined into a unique section. Among other embryonal tumors, two new recognized entities are introduced in CNS5: CNS neuroblastoma, FOXR2-activated, and CNS tumor with BCOR internal tandem duplication (ITD). Embryonal tumor with multilayered rosettes (ETMR), already present in the previous classification now has a revised nomenclature as a result of the new DICER1 alteration, additional to the formerly known C19MC. Regarding atypical teratoid/rhabdoid tumor (AT/RT), three molecular subgroups are recognized in CNS5. The combination of histopathological and molecular features reflects the complexity of all these tumors and gives critical information in terms of prognosis and therapy. This encourages the use of a layered diagnostic report with the integrated diagnosis at the top, succeeded by layers including the histological, molecular, and other essential details.
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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 , NeoplasmsABSTRACT
@#Opsoclonus-myoclonus-ataxia syndrome(OMAS) is a rare neurological disorder. This disease is often associated with neuroblastoma(NB). OMAS and neuroblastoma in children in China are treated separately by neurology, medical and surgical oncology. In this group of children, NB usually has a good prognosis, while OMAS is prone to sequelae, but the lack of standardized evaluation and follow-up is not conducive to the diagnosis and treatment of the disease. In this study, experts from multidisciplinary fields worked together to develop recommendations for the diagnosis, treatment and follow-up of NB associated OMAS in children, hoping to improve the prognosis of children through standardized management of this group of children.
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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.