Haematological and renal toxicity of radiation therapy in neuroblastoma paediatric patients

Purpose The objective of this study was to evaluate the renal and hematologic toxicity in paediatric patients with adrenal high-risk neuroblastoma who have received radiation therapy (RT) as part of radical treatment. Material and methods Pediatric patients diagnosed with high-risk adrenal neuroblastoma who received RT as part of the definitive treatment between January 2004 and May 2020 in a single institution were selected. Complete blood counts (CBC) and creatinine clearance (CrCl) pre-RT and post-RT were compared through the Wilcoxon signed-rank test and correlated with survival analysis by Cox regression. Results Forty-two children with a median age of 3 years at diagnosis and 2.8 years of follow-up were selected. A significant and acute decrease in lymphocytes was found (p = 0.002) 1 month from RT. Patients with a drop higher than 50% of the previous value experimented a significant reduction in overall survival (55 vs 10%; p = 0.031). At the end of the follow-up, a significant increase in all blood counts was observed. With respect to renal function, an acute and significant decrease in CrCl was observed tin patients younger than 4 years who received RT (p = 0.013). However, it was not clinically relevant. Conclusion Our data suggest that acute lymphopenia occurs after RT and could be associated with a poorer prognosis. Other blood counts are reduced after RT and all of them are in physiological range at the end of follow-up. Our cohort presented excellent renal outcomes without any case of chronic renal dysfunction (AU)

Síndrome opsoclono-mioclono: características clínicas, aspectos terapéuticos y factores pronósticos en una cohorte pediátrica española / Opsoclonus-myoclonus syndrome: clinical characteristics, therapeutic considerations, and prognostic factors in a Spanish paediatric cohort

Introducción: El síndrome opsoclono-mioclono-ataxia es un raro trastorno de inicio pediátrico; de base neuroinflamatoria y origen paraneoplásico, parainfeccioso o idiopático. Actualmente no hay biomarcadores, siendo el diagnóstico clínico. El pronóstico cognitivo parece estar relacionado con el inicio temprano de la terapia inmunomoduladora.MétodoSe describen las características epidemiológicas, clínicas, terapéuticas y pronósticas a largo plazo de una cohorte de 20 pacientes españoles.ResultadosLa edad media de debut fue de 21 meses (2-59 meses). La ataxia y el opsoclonus fueron los síntomas de inicio más frecuentes y predominantes en la evolución. El tiempo medio desde los primeros síntomas hasta el diagnóstico fue de 1,1 mes. Un tumor de extirpe neuroblástica fue detectado en el 45%, realizándose resección quirúrgica en siete y quimioterapia en dos pacientes. En el estudio de líquido cefalorraquídeo se constató pleocitosis en cuatro (25%), con negatividad de anticuerpos antineuronales y bandas oligoclonales en todos los casos estudiados. En el 100% se emplearon fármacos inmunomoduladores. En nueve pacientes el tratamiento combinado inmunomodulador se inició desde el momento del diagnóstico, y en cinco el tiempo medio de implementación fue de 2,2 meses. A largo plazo, seis de 10 pacientes con seguimiento superior a cinco años presentaban secuelas cognitivas leves o moderadas; cuatro pacientes presentaron recaídas, generalmente coincidiendo con el descenso de la corticoterapia.ConclusionesEl inicio precoz de la inmunoterapia, así como de la triple terapia en los casos que lo precisaron, se relacionó con una menor frecuencia de afectación cognitiva a los dos años del debut. (AU)

Introduction: Opsoclonus-myoclonus-ataxia syndrome is a rare neuroinflammatory disorder with onset during childhood; aetiology may be paraneoplastic, para-infectious, or idiopathic. No biomarkers have yet been identified, and diagnosis is clinical. Better cognitive prognosis appears to be related to early onset of immunomodulatory therapy.MethodsWe describe the epidemiological, clinical, therapeutic, and long-term prognostic characteristics of a cohort of 20 Spanish patients.ResultsThe mean age of onset was 21 months (range, 2-59). Ataxia and opsoclonus were the most frequent symptoms both at disease onset and throughout disease progression. The mean time from onset to diagnosis was 1.1 months. Neuroblast lineage tumours were detected in 45% of patients; these were treated with surgical resection in 7 cases and chemotherapy in 2. Cerebrospinal fluid analysis revealed pleocytosis in 4 cases (25%) and neither antineuronal antibodies nor oligoclonal bands were detected in any patient. Immunomodulatory drugs were used in all cases. Nine patients started combined immunomodulatory treatment at the time of diagnosis, and 5 patients after a mean of 2.2 months. In the long term, 6 of the 10 patients followed up for more than 5 years presented mild or moderate cognitive sequelae. Four patients presented relapses, generally coinciding with the decrease of corticosteroid doses.ConclusionsEarly initiation of immunotherapy, as well as triple combination therapy, where needed, was associated with a lower frequency of cognitive impairment 2 years after onset. (AU)

FTO inhibits oxidative stress by mediating m6A demethylation of Nrf2 to alleviate cerebral ischemia/reperfusion injury

Current therapies are of limited efficacy in cerebral ischemia/reperfusion (I/R) injury. Based on the important role of oxidative stress in cerebral I/R injury, this study aimed to explore how the N6-adenosine methylation (m6A) demethylase FTO affects oxidative stress. Middle cerebral artery occlusion/reperfusion (MCAO/R)-induced rat model and oxygen and glucose deprivation/re-oxygenation (OGD/R)-induced SH-SY5Y cells were established as in vivo and in vitro model, respectively. The neurological score of rats was measured, and the volume of cerebral infarction was measured by TTC staining. The levels of FTO, nuclear factor-erythroid 2-related factor (Nrf2), and the activity of m6A demethylase FTO were detected. The m6A methylation level of Nrf2 mRNA was detected by MeRIP experiment. Flow cytometry and MTT assay were used to detect apoptosis and proliferation in vitro. TUNEL assay was used to detect apoptosis in brain tissues. FTO and Nrf2 expressions were decreased in the MCAO/R rat brain tissues and OGD/R SH-SY5Y cells, while the m6A methylation level of Nrf2 mRNA was significantly increased. Overexpression of FTO upregulated Nrf2 expression by decreasing the m6A methylation level of Nrf2 mRNA. m6A binding protein YT521-B homology (YTH) domain family protein 2 (YTHDF2) promoted the degradation of Nrf2 by promoting the m6A methylation level of Nrf2 mRNA. Furthermore, SH-SY5Y cell apoptosis was increased and cell viability was decreased after the addition of methyltransferases METTL 3/14, thus blocking FTO to protect SH-SY5Y cells from oxidative stress injury. In vivo, overexpression of FTO decreased the area of cerebral ischemia infarction and the extent of cell apoptosis. In conclusion, FTO increases Nrf2 expression by mediating m6A demethylation of Nrf2 mRNA, thereby inhibiting oxidative stress response and ultimately alleviating cerebral I/R injury. (AU)

Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada / Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation

Introducción: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. Caso clínico. Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. Conclusión. El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.(AU)

Background: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. Observation. A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. Conclusion. Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.(AU)

Ganglioneuroblastoma cervical gigante como causa de trastornos del sueño en niños / Giant cervical ganglioneuroblastoma as a cause of sleep disorders in children

El ganglioneuroblastoma es un tumor derivado de los neuroblastos, generalmente originado a partir de estructuras simpáticas, típicamente localizado en la glándula suprarrenal. En este artículo presentamos un caso excepcional de una paciente de 6 años con un ganglioneuroblastoma cervical que desarrolló trastornos del sueño derivados de la compresión de la vía aérea desde su primer año de vida. (AU)

Ganglioneuroblastoma is a tumor derived from neuroblasts, generally related to sympathetic structures, which is usually located in the adrenal gland. In this article, we present a rare case of a patient with cervical ganglioneuroblastoma, who developed sleep disorders since the first year of life due to compression of the airway. (AU)

Minimally invasive surgery for pediatric dumbbell neuroblastoma: systematic literature review and report of a single-stage neurosurgical and thoracoscopic approach

IntroductionTraditional surgical strategies for dumbbell neuroblastoma entail, among others, high risk of spinal deformity. Less invasive procedures might reduce these sequelae, however, there is small evidence comparing different strategies. Indications of minimally invasive surgery in neuroblastoma are still developing. Our aim is to identify and analyze different surgical approaches described in the recent literature and to suggest a minimally invasive option.MethodsA systematic review of the literature was conducted in PubMed (Jan 2000–Dec 2021) to identify reports describing surgical resection of dumbbell neuroblastoma in children, according to the PRISMA guidelines. Only full-text articles were included.Results7 articles met the inclusion criteria which, added to the present case, represent a total of 43 patients. All were retrospective studies, most of them small series. Tumor location was mostly thoracic. Most of combined approaches were performed in two stages. Spinal deformity after surgery was reported in 3 patients. Minimally invasive approach was described in only one paper, with no reported cases of its use in a single-stage combined surgery. We also report, to our knowledge, the first single-stage posterior neurosurgical approach combined with thoracoscopy for resection of a dumbbell neuroblastoma in an infant.ConclusionSurgical resection of dumbbell neuroblastomas is challenging. There is no consensus on best surgical approach. Dumbbell tumors should not be considered a contraindication for minimally invasive surgery. A single stage and minimally invasive strategy is proposed.

Síndrome de Horner pediátrico. A propósito de una serie de 14 casos en un hospital terciario / Paediatric Horner syndrome. A case series of 14 patients in a tertiary hospital

Antecedentes El síndrome de Horner (SH) se caracteriza por la triada de ptosis palpebral, miosis y anhidrosis facial. Debido a su amplia variedad de causas puede ocurrir en cualquier edad, siendo infrecuente en pediatría. La etiología y estudio diagnóstico del SH pediátrico (SHP) es motivo de controversia. ObjetivoDescribir las características clínicas de una serie de 14 niños diagnosticados de SH, incidiendo en la etiología del SH y en la evolución clínica que presentaron. Métodos Estudio observacional retrospectivo de pacientes menores de 14 años diagnosticados de SHP en nuestro centro entre el 01 de enero del 2009 y el 30 de abril del 2020. En función de la edad al diagnóstico, los casos se dividieron en congénitos (antes de los cinco meses) y adquiridos. Resultados Se reclutaron 14 pacientes, con una mediana de edad al diagnóstico de 8,5 meses. La causa más frecuente de SHP fue tumoral (6/14), siendo la neoplasia más representativa el neuroblastoma (4/14). De los casos adquiridos (8/14), la causa más frecuente fue iatrogénica (5/8), secundario a cirugía cérvico-torácica. La etiología principal del SH congénito (6/14) fue el neuroblastoma (4/6), siendo la primera manifestación clínica de la enfermedad en el 50% de los pacientes (2/4). Conclusiones El SH puede ser el primer signo de una enfermedad subyacente grave, como es el neuroblastoma. Por este motivo, es necesario realizar un adecuado estudio de extensión en todos los pacientes pediátricos diagnosticados de SH sin una causa clara atribuible. Es fundamental un examen riguroso para un diagnóstico precoz de estos pacientes (AU)

Background Horner syndrome (HS) is characterised by the triad of upper eyelid ptosis, miosis, and facial anhidrosis. Due to its wide variety of causes, it can occur at any age, and is uncommon in paediatrics. The aetiology and diagnostic approach of paediatric HS (PHS) is controversial. ObjectiveThe purpose of this study is to describe the clinical characteristics of a 14 case series, focusing on the aetiology of HS and the clinical evolution the patients presented. Methods A retrospective observational study was conducted on patients under 14 years-old (enrolled between 1 st January 2009 and 30th April 2020). Depending on the age at diagnosis (before or after the first 5 months of life), the study cases were divided into two groups: congenital or acquired. Results Fourteen patients, with a mean age of 8.5 months, were enrolled. The most frequent cause of PHS were tumours (6/14), with the most representative neoplasm being neuroblastoma (4/14). Of the acquired cases (8/14), the most frequent cause was iatrogenic (5/8), mainly secondary to cervical or thoracic surgery. The main origin of congenital HS (6/14) was neuroblastoma (4/6), being the first manifestation of the disease in 50% of patients (2/4). Conclusion HS may be the first sign of a major underlying disease, such as neuroblastoma. For this reason, children presenting with HS of unknown origin require imaging studies to exclude a life threatening disease. A thorough examination is essential for early diagnosis of these patients. (AU)

Distribution of segmental chromosomal alterations in neuroblastoma

Background Neuroblastoma (NB) is a heterogeneous tumor with extremely diverse prognosis according to clinical and genetic factors such as specific combinations of chromosomal imbalances. Methods Molecular karyotyping data from a national neuroblastic tumor database of 155 NB samples were analyzed and related to clinical data. Results Segmental chromosomal alterations (SCA) were detected in 102 NB, whereas 45 only displayed numerical alterations. Incidence of SCA was higher in stage M (92%) and MYCN amplified (MNA) NB (96%). Presence of SCA was associated with older age, especially 1q gain and 3p deletion. 96% of the deaths were observed in the SCA group and 85% of the relapsed NB contained SCA. The alteration most commonly associated with a higher number of other segmental rearrangements was 11q deletion, followed by 4p deletion. Whole-chromosome 19 gain was associated with lower stages, absence of SCA and better outcome. Conclusions SCA are not randomly distributed and are concentrated on recurrent chromosomes. The most frequently affected chromosomes identify prognostic factors in specific risk groups. SCA are associated with older age and MNA. We have identified a small subset of patients with better outcome that share whole-chromosome 19 numeric gain, suggesting its use as a prognostic biomarker in NB (AU)

Tumores neuroblásticos en el adulto / Neuroblastic tumors in adults

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Cáncer en los primeros 18 meses de vida / Cancer in the first 18 months of life

INTRODUCCIÓN: La enfermedad oncohematológica continúa siendo la primera causa de mortalidad no traumática en la infancia y una importante causa de morbilidad. El paciente menor de 18 meses presenta particularidades clínicas, diagnósticas y terapéuticas que es interesante conocer por todo pediatra, con el fin de lograr una mayor supervivencia y una menor comorbilidad a lo largo de su vida. MATERIAL Y MÉTODOS: Estudio descriptivo retrospectivo de variables clínicas, diagnósticas y terapéuticas en pacientes menores de 18 meses diagnosticados de enfermedad oncohematológica que reciben quimioterapia en una unidad de oncología pediátrica entre enero 2007 y agosto 2019. RESULTADOS: Setenta y dos pacientes fueron diagnosticados de 76 neoplasias que precisaron quimioterapia. La neoplasia de mayor incidencia fue la leucemia (21 pacientes), seguida del neuroblastoma (15 pacientes) y los tumores del sistema nervioso central (12 pacientes). La presentación con «síntomas amenazantes para la vida» tuvo lugar en el 20,8% de los afectados, especialmente en tumores de estirpe neural (13/15). El 18% de pacientes no presentaron síntomas al inicio. El 51% de los diagnósticos totales tuvieron lugar en «estadios avanzados». Concretamente en el caso de los tumores sólidos, el 23,6% de los inicios presentaron metástasis. Se aislaron importantes porcentajes de alteraciones genéticas implicadas en la etiopatogenia de las diferentes neoplasias. CONCLUSIONES: El cáncer en la primera etapa de la vida supone un reto diagnóstico y terapéutico por su diversidad fenotípica, su carga genética y sus dificultades terapéuticas. El conocimiento de sus particularidades es fundamental para un abordaje precoz y eficaz

INTRODUCTION: Oncological-haematological disease continues to be the first cause of non-traumatic mortality in childhood, as well as a significant cause of morbidity. The patient less than 18-months-old has special clinical, diagnostic, and therapeutic features that all paediatricians are interested in determining, with the aim of achieving greater survival and a lower morbidity throughout the lives of their patients. MATERIAL AND METHODS: A retrospective, descriptive study was carried out using the clinical, diagnostic, and therapeutic variables in patients less than 18-months-old diagnosed with an oncological-haematological that received chemotherapy in a Paediatric Oncology Unit between January 2007 and August 2019. RESULTS: A total of 72 patients were diagnosed with 76 cancers that required chemotherapy. The most common cancer was leukaemia (21 patients), followed by neuroblastoma (15 patients), and tumours of the central nervous system (12 patients). The presentation of "life-threatening symptoms" was seen in 20.8% of cases, particularly in tumours of neural origin (13/15). Although 18% of patients showed no symptoms on diagnosis, just over half (51%) of the diagnoses took place in the "advanced stages". Particularly in the case of solid tumours in which 23.6% were diagnosed with metastases. A significant percentage of genetic alterations implicated in the aetiopathogenesis of the different cancers were found. CONCLUSIONS: Cancer in the first stages of life is a diagnostic and therapeutic challenge due to its phenotypical diversity, its genetic load, and its therapeutic difficulties. Knowledge of its particular features is essential for its early and effective approach

Cáncer en el Hospital 12 de Octubre de Madrid. Evolución en la supervivencia. Años 1999-2016 / Childhood cancer in a tertiary care hospital in Madrid. Evolution of survival. Years 1999-2016

INTRODUCCIÓN: El cáncer es la primera causa de muerte por enfermedad en niños. Se detallan algunos aspectos epidemiológicos del cáncer infantil obtenidos del Registro de Tumores de un hospital de tercer nivel de Madrid, con el fin de aportar información útil para el manejo del cáncer en este grupo de pacientes. MATERIAL Y MÉTODOS: Análisis descriptivo y retrospectivo de los datos del Registro de Tumores de un hospital de tercer nivel (periodo 1999-2016), con el objetivo de analizar la incidencia (global y por categorías diagnósticas) y la supervivencia (global, por grupos diagnósticos y por cohortes de años de diagnóstico) del cáncer infantil. RESULTADOS: Entre 1999 y 2016 se registraron 769 tumores infantiles, 431 en niños y 338 en niñas. Las neoplasias más frecuentes fueron los tumores del sistema nervioso central (32,5%), las leucemias, los síndromes mielodisplásicos y síndromes mieloproliferativos (19%), los linfomas (15%) y los neuroblastomas (7,5%). La supervivencia global a los 5 años fue del 78%. La supervivencia a los 5 años para estas categorías diagnósticas fue del 74% (67-81%) para los tumores del sistema nervioso central; del 80% (72-88%) para las leucemias, síndromes mielodisplásicos y síndromes mieloproliferativos; del 87% (80-95%) para los linfomas y neoplasias reticuloendoteliales; y del 68% (53-84%) para los neuroblastomas y otros tumores de células nerviosas periféricas. La comparativa entre dos cohortes de años de diagnóstico (1999-2004 vs. 2005-2010) revela un incremento de la supervivencia en la cohorte más reciente, que solo es estadísticamente significativo en los tumores del sistema nervioso central. CONCLUSIONES: Nuestros resultados son similares a los del Registro Español de Tumores Infantiles. La información aportada por los Registros de Tumores es necesaria para un mayor conocimiento del cáncer y para garantizar la calidad asistencial de los enfermos oncológicos

INTRODUCTION: Cancer is the leading cause of death from disease in children. Some epidemiological aspects of childhood cancer obtained from the Tumour Registry of a tertiary care hospital in Madrid are detailed, in order to provide useful information for the management of cancer in this group of patients. MATERIAL AND METHODS: Descriptive and retrospective analysis of the data from the Hospital's Tumour Registry (period 1999-2016), with the aim of analysing the incidence (overall, and by diagnostic categories) and survival (overall, by diagnostic groups and cohorts of years of diagnosis) of childhood cancer. RESULTS: A total of 769 childhood tumours were registered between 1999 and 2016, 431 in boys and 338 in girls. The most common neoplasms were central nervous system tumours (32.5%), leukaemias, myelodysplastic syndromes and myeloproliferative syndromes (19%); lymphomas (15%), and neuroblastomas (7.5%). Overall 5-year survival was 78%. Five-year survival of these diagnostic categories was 74% (67-81%) for central nervous system tumours; 80% (72-88%) for leukaemias, myelodysplastic syndromes and myeloproliferative syndromes; 87% (80-95%) for lymphomas and reticuloendothelial neoplasms; and 68% (53-84%) for neuroblastomas and other peripheral nerve cells tumours. The comparison between two diagnostic cohorts (1999-2004 vs 2005-2010) showed an increase in survival in the most recent cohort, which was only statistically significant in central nervous system tumours. CONCLUSIONS: These results are similar to those of the Spanish Register of Childhood Tumours. The information provided by the Tumour Registries is necessary for greater knowledge of cancer and to ensure the quality of care for cancer patients

Linfocitos infiltrantes de tumor en sangre periférica en un modelo de neuroblastoma / Peripheral blood tumor-infiltrating lymphocytes in a neuroblastoma model

Objetivo: Comprobar la existencia de linfocitos T que incluyen linfocitos infiltrantes de tumor (TILs) en la sangre periférica (SP) de un modelo preclínico de neuroblastoma. Material y métodos: Utilizamos un modelo en ratones inmunodeficientes y otro en inmunocompetentes mediante inyección de suspensiones de la línea tumoral NB36769 con mutación de MYCN (TH-MYCN+). Se realizaron análisis por citometría de flujo (bazo, SP y tumor) y secuenciación del TCR-b en el ADN de muestras pareadas de tumor y SP. Resultados: En los ratones inmunodeficientes el componente principal en SP fue CD4: 83,1% (control) y 86,1% (tumor), siendo PD-1+ el 0,4 y el 0,3%. En el bazo obtuvimos un mayor porcentaje de linfocitos T PD-1+ que en SP, siendo similar en el control (6,5%) y en el ratón con tumor (6,2%), en subpoblación CD4+ exclusivamente. En los ratones inmunocompetentes observamos que la proporción de los 10 clones más frecuentes en los tumores constituía el 11,09% ± 2,83% del repertorio del TCR, mientras en SP representaba el 1,59% ± 0,59% (p = 0,024). Estos resultados sugieren un enriquecimiento de clonotipos dentro del tumor. De los 10 clones más frecuentes en las muestras tumorales, localizamos 9 también en la SP en dos ratones y 6 en el tercero. Además, encontramos secuencias compartidas por TILs de animales diferentes. Conclusiones: Nuestros resultados de inmunofenotipo y clonalidad apuntan a la existencia de linfocitos en SP que podrían contener TILs en un modelo experimental de neuroblastoma

Objective: To detect tumor-infiltrating lymphocytes (TILs) in the peripheral blood (PB) of a preclinical neuroblastoma model. Materials and methods: Two types of preclinical models - immuno-deficient mice and immunocompetent mice - were generated by injecting a cell suspension of neuroblastoma cell line NB36769 with MYCN gene (TH-MYCN+) overexpression. Spleen, tumor, and peripheral blood were studied using flow cytometry to detect PD-1+ T-cells. TCR-b immunose-quencing was performed in matched samples (tumor and peripheral blood). Results: Most PB T-cells of immunodeficient mice were CD4 (control: 83.1%; tumor: 86.1%), with a small proportion of PD-1+ T-cells (control: 0.4%; tumor: 0.3%). However, the percentage of PD-1+T-cells in the spleen was higher (control: 6.5%; tumor: 6.2%), and it was expressed in the CD4+ subset only. Regarding the TCR repertoire of immunocompetent mice, the propor-tion of the 10 most frequent sequences was significantly higher in tumors (11.09% ± 2.83%) than in the peripheral blood (1.59% ± 0.59%) (p = 0.024). These findings are suggestive of clonotype enrichment within the tumor. 9 out of the 10 most frequent tumor clones were identified in the matched peripheral blood sample in 2 mice, and 6 out of 10 in one mouse. In ad-dition, TILs with shared sequences from different animals were found. Conclusions: Our results in terms of immunophenotype and clon-ality suggest the presence of PB T-cells which could include TILs in a preclinical neuroblastoma model

Linfangioma quístico mediastínico en un lactante: estudio isotópico con 123I-MIBG en el diagnóstico diferencial con neuroblastoma / Mediastinal cystic lymphangioma in an infant: 123I-MIBG scintigraphy in the differential diagnosis of neuroblastoma

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Terapia inmunosupresora en síndrome de opsoclonus-mioclonus ataxia asociado a un neuroblastoma paravertebral / Immunosuppressive therapy in opsoclonus-myoclonus ataxia syndrome associated with paravertebral neuroblastoma

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Diferenciación benigna del neuroblastoma tratado como causa de un falso positivo mediante SPECT/TC con 123I-MIBG. Utilidad de la PET/TC con 18F-FDG / Benign differentiation of treated neuroblastoma as a cause of false positive by 123I-MIBG SPECT/CT. Usefulness of 18F-FDG PET/CT

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Síndrome de arlequín secundario a neuroblastoma / Harlequin syndrome secondary to a neuroblastoma

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El hemangioma mediastínico y la importancia del GLUT-1 / Mediastinal haemangioma and the role of GLUT1

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Oncología quirúrgica pediátrica / Pediatric surgical oncology

Las neoplasias suponen la primera causa de muerte en niños mayores de 1 año en países desarrollados. la cirugía pediátrica juega un importante papel terapéutico en el cáncer infantil a distintos niveles: extirpación tumoral, implantación de accesos vasculares, manejo de metástasis y complicaciones. La oncología pediátrica es una especialidad muy dinámica con actualizaciones y cambios en protocolos constantemente. el objetivo de este artículo es la revisión actualizada de los tumores sólidos más frecuentes y con características específicas en relación con la cirugía pediátrica: neuroblastoma, tumor de Wilms, hepatoblastoma, tumores ováricos, testiculares y de partes blandas. El neuroblastoma es el tumor sólido extracraneal más común en la infancia. tanto el neuroblastoma como el tumor de Wilms suelen presentarse como una masa abdominal asintomática. la combinación del grupo de riesgo, edad, factores biológicos y resultados histológicos permite asignar a cada paciente un estadio de riesgo con valor pronóstico, y establecer una estrategia terapéutica específica en cada caso de neuroblastoma. el nefroblastoma o tumor de Wilms es el tumor renal maligno más frecuente en niños. en nuestro medio el nuevo protocolo establecido por la Siop se conoce como UMBrella que se basa en la quimioterapia preoperatoria con el objetivo de reducir masa tumoral seguido de cirugía. En los casos de hepatoblastoma es importante la clasificación preteXt (Pretreatment Extent of Disease) porque define la extensión de parénquima hepático afectada y enfermedad extrahepática dando información sobre la resecabilidad del tumor y su respuesta a la quimioterapia. Ante una masa de tejido blando debe plantearse en primer lugar que se trate de procesos reactivos y tumores benignos, los tumores malignos de partes blandas son raros. dentro de estos, el rabdomiosarcoma se caracteriza por su buena respuesta a quimioterapia, por lo que está en desuso la cirugía con resecciones agresivas o mutilantes. Respecto a los tumores gonadales en la infancia, a diferencia de los adultos, predominan los germinales, en concreto el teratoma. la cirugia tiende a ser conservadora para respetar la fertilidad futura. en las últimas décadas, los avances en tratamientos oncológicos han logrado un aumento de la supervivencia en la mayoría de tumores infantiles, en parte gracias al enfoque multidisciplinar necesario desde el diagnóstico de cada caso

Malignant neoplasms constitute the first cause of death in children over 1 year of age in developed countries. Pediatric surgery plays an important therapeutic role in childhood cancer on different sides: removing tumors, placing vascular access devices, metastasis management and complications. Pediatric oncology is a dinamyc specialty with constant updates and changes in protocols. the principal aim of this report is an updated review of more common solid tumors and their specific surgical aspects: neuroblastoma, Wilms' tumor, hepatoblastoma, ovarian tumor, testicular tumor and rhabdomyosarcoma. Neuroblastoma is the most common extracranial solid tumor in chidhood. Wilms' tumor and neuroblastoma usually appears as an asyntomatic abdominal mass. the risk group, age, biological factors and histologic analysis allow to assign a risk stage to each patient, that has prognostic value and determines the specific treatment for each case of neuroblastoma. Nephroblastoma or Wilms' tumor is the most frequent malignant kidney tumor in children. UMBrella is the new protocol of Siop based on preoperative chemotherapy to reduce the size of the tumor and surgery after. The classification PRETEXT (Pretreatment Extent of Disease) is important in cases of hepatoblastoma because defines the extent of hepatic infiltrated parenchyma, so we can know about its resectability and the response to chemotherapy. A soft-tissue mass is probably a reactive process or a benign tumor, since malignant soft-tissue tumors are rare. rhabdomyosarcoma has a good response to chemotherapy, so wide resections and radical surgery are not current techniques. With regard to gonadal tumors in childhood, unlike adults, germ tumors predominate, in particular teratoma. Sparing surgery is the current treatment to preserve future fertility. over the last several decades, the advances in cancer treatment have achieved an increased survival in most of childhood tumors, thanks to multidisciplinary approach from diagnosis

Paracentesis como tratamiento descompresivo abdominal en neuroblastoma MS con hepatomegalia masiva / No disponible

El neuroblastoma MS (o 4S según la nomenclatura clásica) con hepatomegalia masiva supone un mínimo porcentaje de los casos de neuroblastoma. Es más frecuente en lactantes de menos de 4-6 semanas de vida, y conlleva, al contrario que la norma del NB MS, mal pronóstico dadas las complicaciones que puede tener. En caso de síndrome compartimental abdominal se aconseja inicio rápido de tratamiento quimioterápico, asociando o no radioterapia para intentar reducir el tamaño del hígado, y en caso de ser necesario, laparotomía descompresiva. Presentamos el caso de una paciente con NB MS, hepatomegalia masiva y síntomas amenazantes para la vida, en la que la actitud quirúrgica que consiguió aliviar el síndrome de compresión intraabdominal consistió únicamente en paracentesis evacuadora

Neuroblastoma MS with massive hepatomegaly is a small percentage of cases of neuroblastoma. It is more common in infants less than 4-6 weeks of life, and involves, in contrast to the standard of the NB MS, poor prognosis given the complications that can have. In the case of abdominal compartment syndrome it is recommended a quick start of chemotherapy, associating or not radiation therapy, to try to reduce the size of the liver, and if necessary, decompressive laparotomy. We present the case of a patient with NB MS, massive hepatomegaly and threatening symptoms for life, in which the surgical attitude that got relieve intra-abdominal compression syndrome consisted just in an evacuating paracentesis