Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma.

Very rare tumors (VRTs) account for up to 11% of childhood cancers. Dedicated national groups and registries only exist in some European countries. Pleuropulmonary blastoma (PPB) is a very rare intrathoracic pediatric tumor with a potentially severe prognosis. Due to its rarity, it sometimes goes unrecognized. We investigated PPB diagnostic capability and possible correlations between diagnostic performance and VRT-dedicated activities. The number of cases of PPB registered between 2000 and 2014 at pediatric oncology centers in Europe was compared with the number of expected cases. Data sources included VRT registries, population-based cancer registries, and hospital registries. Data were obtained for 25 countries, grouped into 4 geographical regions. The expected cases were 111, and the observed cases were 129. The observed-to-expected ratio was 1.86 for Northern Europe, 1.33 for Southern Europe, 1.22 for Central Europe, and 0.65 for Eastern Europe. More cases than expected were registered in all countries with an official VRT registry.Conclusion: The number of cases observed is consistent with expectations, but disparities exist across Europe. Difficulties in diagnosing PPB emerged in most Eastern countries. The incidence rate of PPB may be underestimated. The creation of VRT-dedicated groups and a European Registry for VRTs could help to reduce inequalities.What is Known:• Very rare pediatric tumors are often not recognized, despite representing almost 11% of childhood cancers .• Pleuropulmonary blastoma is a rare pediatric tumor with a poor prognosis.What is New:• The ability to diagnose and register pleuropulmonary blastoma varies in Europe.Registries dedicated to very rare pediatric tumors improve the diagnostic rates.• The incidence rate of pleuropulmonary blastoma may currently be underestimated.

[Paracentesis as abdominal decompression therapy in neuroblastoma MS with massive hepatomegaly]. / Paracentesis como tratamiento descompresivo abdominal en neuroblastoma MS con hepatomegalia masiva.

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.

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.

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

Metastatic neuroblastoma in infants: are survival rates excellent only within the stringent framework of clinical trials?

Introduction. SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population. Materials and methods. Italian and Spanish metastatic INES patients’ data are reported. SPSS 20.0 was used for statistical analysis. Results. Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis. Conclusions. The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data (AU)

No disponible

Metastatic neuroblastoma in infants: are survival rates excellent only within the stringent framework of clinical trials?

INTRODUCTION: SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population. MATERIALS AND METHODS: Italian and Spanish metastatic INES patients' data are reported. SPSS 20.0 was used for statistical analysis. RESULTS: Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis. CONCLUSIONS: The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data.

Mastocitosis cutánea difusa. Presentación de 3 casos y revisión de su manejo terapéutico / Diffuse cutaneous mastocytosis. Presentation of 3 cases and therapeutic management review

No disponible

Exoftalmos unilateral de corta evolución. Una forma de debut de un neuroblastoma / Unilateral exophthalmos with short evolution. One way to debut a neuroblastoma

El neuroblastoma (NB) es el tumor extracraneal sólido más frecuente en la infancia. Representa el 7% de los cánceres pediátricos. Se origina de la cresta neural, y puede aparecer en cualquiera de los sitios anatómicos a lo largo de la cadena ganglionar simpática, así como en la glándula suprarrenal. El 90% ocurre en menores de 5 años. Presenta un amplio espectro de comportamiento clínico. Los estudios muestran metástasis orbitarias en el 10-20% de los casos. La proptosis y equimosis periorbitaria son consideradas dos de los signos clásicos del neuroblastoma en niños. Presentamos el caso de una paciente de 4 años de edad con un neuroblastoma metastásico que se manifestó como una proptosis de corta evolución al diagnóstico, sin otro tipo de sintomatología ni hallazgos en la exploración física. Tras las pruebas complementarias, se la diagnosticó de neuroblastoma estadio IV de la INSS (The International Neuroblastoma Staying System). No se localizó el tumor primario. Se realizó tratamiento con quimioterapia de inducción, terapia de acondicionamiento y trasplante autólogo de progenitores hematopoyéticos, entrando en remisión completa. Posteriormente inmunoterapia, manteniendo enfermedad residual negativa. Queremos destacar la importancia de un diagnóstico precoz en esta patología de cara a la supervivencia del paciente, y que siempre debe considerarse el diagnóstico de NB ante un exoftalmos en un paciente previamente sano. El tratamiento de los pacientes con neuroblastoma de alto riesgo es multimodal, habiendo mejorado el pronóstico el uso combinado de inmunoterapia, ácido 13 cis-retinoico y trasplante autólogo de médula ósea

Neuroblastoma (NB) is an extracranial solid brain tumor found most frequently during childhood. It represents 7% of the pediatric cancers. It originates in the neural crest and can appear in any part of the anatomy along the ganglionic sympathetic chain, as well as in the adrenal gland. 90% of cases occur en children under the age of 5. It presents a broad spectrum of behavior. Studies have shown orbital metastases in 10-20% of cases. Proptosis and ecchymosis periorbitary are considered two classic signs of neuroblastoma in children. We are presenting a case of a 4 year old female patient with metastatic neuroblastoma that manifested like a proptosis of short evolution to its diagnosis, without any other type of symtomatololgy or findings in the physical exploration. After complementary tests she was diagnosed with stage IV neuroblastoma in the INSS (The International Neuroblastoma Staying System). The primary tumor was not located. Complete remission was obtained using induction chemotherapy, conditioning therapy and autologous hematopoietic progenitors transplant. After immunotherapy negative residual disease was maintained. We would like to highlight the importance of early diagnosis in this pathology facing the patient’s survival and must always consider the NB diagnosis when an exophthalmos presents in a previously healthy patient. Treatment in patients with high risk neuroblastoma is multimodal, having improved the prognosis by using a combination of immunotherapy, 13-cis-retinoic acid and the autologous bone marrow transplant

First Report of Cymbidium mosaic virus and Odontoglossum ringspot virus in Orchids in Mexico.

In 2010, a survey for viral diseases in commercial, orchid-producing greenhouses was carried out in Morelos, Mexico. Many symptomatic plants were observed. The most common leaf symptoms were yellow mottle, yellow streaks, and chlorotic and necrotic ringspots. Leaf samples were collected from eight symptomatic plants from the following genera: Encyclia, Oncidium, Shomburghia, Brassia, Guarianthe, Cattleya, Epidendrum, Vanilla, Xilobium, Laelia, and Brassocattleya. Samples were tested using double-antibody sandwich (DAS)-ELISA (Agdia, Elkhart, IN) with antiserum for Cymbidium mosaic virus (CymMV), Odontoglossum ringspot virus (ORSV), Cymbidium ringspot mosaic virus, and Tobacco mosaic virus (TMV) and a general antiserum for potyviruses. At least one plant from each genus was positive to CymMV and ORSV as individual or mixed infections. Encyclia and Laelia plants were the most frequently found with mixed infections by both viruses. All genera were negative for TMV and potyviruses. Total RNA extracts were obtained from all ELISA-positive samples by a modified silica capture protocol (2). Reverse transcription (RT)-PCR was carried out with general polymerase (RdRp) gene primers corresponding to the Potexvirus group (3) and specific primers for the coat protein gene (CP) of CymMV and ORSV (1). The PCR amplification from a positive sample of each genus was resolved in agarose gels. Amplification products of the expected size were obtained for CymMV and ORSV. Five CymMV RdRp gene clones from five different plants of Laelia (GenBank Accession Nos. HQ393958, HQ393959, HQ393960, HQ393961, and HQ393962), two CP gene clones of CP gene of CymMV from two different plants of Oncidium (GenBank Accession Nos. HQ393956 and HQ393957), and three CP clones of CP of ORSV from three different plants of Encyclia (GenBank Accession Nos. HQ393953, HQ393954, and HQ393955) were sequenced. The nucleotide sequences of the Mexican orchid CymMV isolates were 96 to 97% identical to CymMV sequences in the GenBank, while those of ORSV were 99 to 100% identical to deposited ORSV sequences. To our knowledge, this is the first report of CymMV and ORSV in orchids in Mexico, which are two of the most important quarantine virus in orchids in Mexico. References: (1) P. Ajjikuttira et al. J. Gen. Virol. 86:1543, 2005. (2) J. R. Thompson et al. J. Virol. Methods 111:85, 2003. (3) R. A. A. van der Vlugt and M. Berendsen. Eur. J. Plant Pathol. 108:367, 2002.

Tumores vasculares en la infancia / Infantile vascular tumors

La utilización en el pasado de una terminología imprecisa para designar a los tumores vasculares infantiles ha contribuido durante años a diagnósticos incorrectos y, como consecuencia, a tratamientos inadecuados. En la infancia pueden presentarse diferentes tipos de tumores vasculares, como los hemangiomas infantiles, que son con diferencia los más frecuentes, y otros mucho más raros, como los hemangiomas congénitos (rápidamente involutivo y no involutivo), el hemangioendotelioma kaposiforme, el angioblastoma o angioma en penacho, o el granuloma piógeno. Su correcto conocimiento y diagnóstico, siempre en el contexto de un equipo multidisciplinario, es imprescindible para reducir errores diagnósticos, exámenes complementarios y pruebas invasivas innecesarias, y así, si fuera preciso, recibir el tratamiento más indicado y efectivo en cada caso. En el presente artículo revisamos la evolución histórica en cuanto a la nomenclatura y clasificación de las lesiones vasculares, las diferentes características clinicopatológicas de cada uno de los tumores vasculares, los exámenes complementarios indicados para llegar a un correcto diagnóstico, su diagnóstico diferencial y los distintos tipos de tratamiento que existen con sus indicaciones más reconocidas, en el momento actual, para los diferentes tumores vasculares y situaciones clínicas concretas (AU)

The use in the past of an imprecise terminology to designate vascular tumors has contributed to its incorrect diagnosis, and as a consequence, to inadequate treatment. In childhood, different types of vascular tumors may be present. Hemangiomas of infancy are by far the most frequent, and other less common types are congenital hemangiomas (rapidly involuting or RICH and non-involuting or NICH), kaposiform hemangioendothelioma, angioblastoma or tufted angioma and pyogenic granuloma. The correct knowledge and diagnosis, always in a multidisciplinary setting, is required to reduce incorrect diagnosis, unnecessary complementary examinations and invasive tests, and for the patient to receive the most effective and precise treatment in each case. This article reviews the historical evolution, nomenclature and classification of vascular lesions, the different clinical and pathological characteristics of each vascular tumor, the complementary examinations required correct diagnosis, the differential diagnosis, as well as highlighting the treatment options currently available for different vascular tumors and related clinical conditions (AU)

[Infantile vascular tumors]. / Tumores vasculares en la infancia.

The use in the past of an imprecise terminology to designate vascular tumors has contributed to its incorrect diagnosis, and as a consequence, to inadequate treatment. In childhood, different types of vascular tumors may be present. Hemangiomas of infancy are by far the most frequent, and other less common types are congenital hemangiomas (rapidly involuting or RICH and non-involuting or NICH), kaposiform hemangioendothelioma, angioblastoma or tufted angioma and pyogenic granuloma. The correct knowledge and diagnosis, always in a multidisciplinary setting, is required to reduce incorrect diagnosis, unnecessary complementary examinations and invasive tests, and for the patient to receive the most effective and precise treatment in each case. This article reviews the historical evolution, nomenclature and classification of vascular lesions, the different clinical and pathological characteristics of each vascular tumor, the complementary examinations required correct diagnosis, the differential diagnosis, as well as highlighting the treatment options currently available for different vascular tumors and related clinical conditions.

Study of the relationship between acetaminophen and asthma in Mexican children aged 6 to 7 years in 3 Mexican cities using ISAAC methodology.

OBJECTIVE: To establish the relationship between the use of acetaminophen and the frequency of asthma in Mexican children in 3 Mexican cities. METHODS: Ours was a multicenter, observational, descriptive, cross-sectional study. Patients from 6 to 7 years of age participating in Phase Three B of the ISAAC (International Study of Asthma and Allergies in Children) living in the north of Mexico City, Victoria City, and Merida were included. After adjusting for confounders, we calculated the odds ratios (OR) for the presence of wheezing ever, wheezing in the last 12 months, asthma ever, and the use of acetaminophen in the first year of life and during the last 12 months. RESULTS: The ORs for wheezing ever, wheezing in the last year, and asthma ever with respect to use of acetaminophen in the first year of life were not statistically significant (P > .05) in Mexico City, but they were significant in Victoria City (P < .05) and Merida (P < .05). The ORs (95% confidence intervals) for wheezing ever, wheezing in the last year, and asthma ever with respect to use of acetaminophen in the last year were 3.44 (2.96-4.0), 7.97 (5.89-10.78), and 6.10 (3.30-8.81) (P < .05) in Mexico City. Values forVictoria City were 1.36 (1.13-1.63), 3.80 (2.88-5.05), and 2.18(1.57-3.01) (P < .05). Those for Merida were 1.61 (1.40-1.85), 2.07 (1.73-2.48), and 1.53 (1.29-1.82) (P < .05). CONCLUSION: The use of acetaminophen is associated with the presence of wheezing and asthma in 3 different cities in Mexico.

Leaf Stripe and Stem Rot Caused by Burkholderia gladioli, a New Disease of Maize in México.

A new maize disease appeared in the State of Veracruz, Mexico during 2003-2004. Initial symptoms in the leaves were small, white-yellow, watery spots, which coalesced into dry necrotic stripes that were 0.3 wide and 8 cm long. Reddening sometimes developed on these leaves. Stems developed a rot in the crown. The flag leaf showed a rot and necrosis at the base, rolled inward, and dried out. Necrosis developed at the base of the corn ears and their growth was halted. A bacterium characterized by white colonies was consistently isolated from lesions on casamino acid peptone and glucose (CPG), King's medium B, and nutrient agar media. Ten isolates were chosen for further characterization. Pathogenicity was confirmed in the greenhouse (25 to 30°C) on 45-day-old ASGROW 7573 maize plants by injection of bacterial suspensions (107 CFU/ml) at the base of the stem. Control maize seedlings were injected with phosphate buffer. Symptoms similar to those observed in the field were observed after 3 days on all inoculated plants but were not observed on control plants. Koch's postulates were fulfilled with reisolation of the inoculated strain from the inoculated tissues and confirmation was fulfilled by phenotypic characters and 16S rDNA sequences. The white colonies on CPG were slightly convex, shiny, circular with entire margins, gram negative, lacked arginine dihydrolase, did not produce fluorescent pigment on Pseudomonas F medium, and grew aerobically. The strains were able to utilize l-arabinose, d-mannitol, and cellobiose, but unable to utilize d-maltose and l-rhamnose. Gel hydrolysis was positive but starch hydrolysis was not positive. Colonies grew at 40°C. These characteristics are the same as those described previously for Burkholderia gladioli (3). The identity of these isolates was confirmed by 16S analysis with two universal primers, FD1 and RD1, for Eubacteria (2), which generate a 1,600-bp fragment. Two primers specific for the genus Burhkolderia, RHG-F and RHG-R that generate a 500-bp fragment (1) were also used for amplification. Comparison of sequences using Clustal W and Megaline, DNAstar software showed 100% similarity among strains isolated from the three counties (GenBank Accession Nos. EU161873 to EU161878) and 99% similarity of field isolates with B. gladioli in the GenBank database. To our knowledge, this is the first report of B. gladioli causing leaf stripe and stem rot of maize in Mexico. References: (1) J. J. Lipuma et al. J. Clin. Microbiol. 37:3167, 1999. (2) L. M. Rodrigues et al. Appl. Environ. Microbiol. 69:4249, 2003. 3) N. W. Shaad et al., eds. 3rd ed. Laboratory Guide for Identification of Plant Pathogenic Bacteria. The American Phytopathological Society, St. Paul, MN, 2001.

First Report of Prunus necrotic ringspot virus in Peach in Mexico.

Peach (Prunus persica (L.) Batsch) is one of the most important fruit crops in the temperate regions of Mexico. In 2006, during a survey conducted in commercial peach orchards in Puebla, Mexico for viral diseases, many trees were observed with foliar symptoms that included yellow mottle, ringspot, line patterns, and mosaic. Samples (flowers, young shoot tips, and leaves) were collected from 120 symptomatic trees in three locations (San Martin Texmelucan, Domingo Arenas, and Tepetzala). All samples were tested using double-antibody sandwich (DAS)-ELISA kits (Agdia, Inc., Elkhart, IN) for the presence of the following viruses: Apple mosaic virus, Plum pox virus, Prune dwarf virus, and Prunus necrotic ringspot virus (PNRSV). Sap extracts from young symptomatic leaves and shoots were used to mechanically inoculate Chenopodium quinoa, C. amaranticolor, Gomphrena globosa, Nicotiana tabacum cv. Xanthi, N. glutinosa, N. clevelandii, N. benthamiana, Datura stramonium, Capsicum annuum, and Solanum lycopersicum. Plants were kept in a greenhouse with approximate temperatures of 25 to 35°C, humidity of 70%, and 12 h of light. Sap extracts were also used for dsRNA extraction and analyses (2) and RNA extraction for use in reverse transcription (RT)-PCR with the Access RT-PCR system (Promega, Madison, WI) and primers that annealed to a conserved region in the PNRSV coat protein gene (1). The expected size amplicons of approximately 450 bp were generated from all field-collected samples. The PCR products from three geographically distinct PNRSV isolates (Domingo Arenas [Accession No. DQ979004], Tepetzala [Accession No. DQ979005], and San Martin Texmelucan [Accession No. EF456771]) were directly sequenced with a Genetic Analyzer 3100 (Applied Biosystems, Foster City, CA) and their nucleotide and deduced amino acids sequences were more than 93% identical to corresponding sequences of PNRSV available in the NCBI/GenBank database. PNRSV was the only virus detected by DAS-ELISA in flowers and young shoots from 60 of the symptomatic field samples tested from the three locations. DsRNA banding patterns were obtained from 40 field-collected symptomatic samples; all showed three bands of approximately 3.6, 2.5, and 1.8 kb, the expected sizes for RNAs 1, 2, and 3 of PNRSV, respectively. DsRNAs were not detected in asymptomatic plants. PNRSV transmission by mechanical inoculation induced mosaic symptoms in N. tabacum cv. Xanthi and necrotic local lesions in G. globosa. Although G. globosa is reported to be a systemic host of PNRSV and N. tabacum is not reported to be a host, symptomatic plants were positive for PNRSV in DAS-ELISA tests. It is possible that there was an additional virus not detected in our assays that was responsible for the unexpected reactions in the host range studies. To our knowledge, this is the first report of PNRSV in peach in Mexico. References: (1) D. J. MacKenzie et al. Plant Dis. 81:222, 1997. (2) R. A. Valverde et al. Plant Dis. 74:255,1990.

Enterocolitis neutropénica en el niño con cáncer: nuestra casuistica y revisión de la literatura / Neutropenic enterocolitis in the child with cancer: our casuistics and review of the literature

Introducción: La enterocolitis neutropénica o tiflitis es una alteración de la región ileocecal con ausencia de infiltrado inflamatorio o tumoral y diferentes grados de afectación de la pared intestinal, típica de pacientes con tumores hematológicos afectos de neutropenia grave o prolongada secundaria a la quimioterapia. Objetivos: Revisar los casos de enterocolitis neutropénica diagnosticados en niños con cáncer del Hospital Universitario de Canarias (HUC) durante los últimos 6 años y medio y realizar una revisión actualizada de la literatura sobre el tema. Pacientes y métodos: Revisión retrospectiva de historias clínicas de los niños que padecieron tiflitis. Se analizaron las siguientes variables: edad, sexo, tipo de neoplasia, protocolo de quimioterapia, y al diagnóstico de la tiflitis; número de días desde el último ciclo de quimioterapia (quimioterápicos recibidos), días de neutropenia, síntomas, método diagnóstico (grosor máximo de la pared intestinal), tratamiento y evolución. Resultados: De los 41 casos de tumores malignos tratados con quimioterapia, el 7,3% (n = 3) presentaron uno o más episodios de enterocolitis neutropénica (todos afectos de leucemia aguda; dos mieloblásticas y una linfoblástica).Todos eran varones, con una edad media de 11 años y medio. La clínica predominante fue dolor abdominal en el contexto de una neutropenia febril postquimioterapia. La confirmación diagnóstica se realizó mediante ecografía abdominal en dos casos y tomografía computarizada (TC) en los otros dos (media del grosor intestinal máximo: 11,5 mm). El tratamiento instaurado en todos los casos fue conservador, logrando una recuperación clínico-radiológica en el 100% de los pacientes. Conclusión: En todo niño con cáncer en tratamiento quimioterápico, especialmente en afectos de leucemia aguda, con neutropenia febril que además refiera dolor abdominal persistente, vómitos y/o diarrea, se debe sospechar una enterocolitis neutropénica, solicitar una ecografía y/o una TC abdominal urgente para confirmarla, e instaurar lo antes posible el tratamiento recomendado, logrando, así, una reducción significativa de la morbimortalidad que conlleva esta grave complicación (AU)

Background: Neutropenic enterocolitis or thyphlitis is a lesion of the ileocecal arca with lack of inflammatory or tumoral infiltration and different degrees of affection on the bowel wall. It is typical in patients with hematologic malignancies with prolonged or severe neutropema alter intensive chemotherapy. Objectives: Revise all neutropenic enterocolitis diagnosed at Hospital Universitario de Canarias (HUC) in the last six and a half years in children with cancer, and carry out an up today literature review. Patients and methods: Retrospective review of the medical histories to the children who had thyplitis. We analyzed the following data: age, sex, cancer type and chemotherapy protocol, and when thyplitis was diagnosed; days from the last cytotoxic chemotherapy cycle (anticancer drugs used), days of neutropenia, symptoms, diagnosis method used (maximum thickness on the bowel wall), treatment and evolution. Results: Forty one cases of malignant neoplasms were treated with chemotherapy and 7,3% (n = 3) had one or more episodes of neutropenic enterocolitis (all of them with acute leukemia, two myelogenus and one lymphoblastic). All of them were male, with an average age of 11 years and 6 months. The symptom more frequently seen was abdominal pain in a child with a febrile neutropenia alter intensive chemotherapy. The diagnosis confirmation was with abdominal ultrasonography (US) in two cases, and computed tomography (CT) in the other two ones (average thickness on the bowel wall was 11,5 mm). All the children recovered without problems with medical treatment. Conclusion: Any child with cancer treated with chemotherapy, especially with acute leukaemia, and febrile neutropenia associated with persistent abdominal pain, vomiting and/or diarrhea, we must suspect neutropenic enterocolitis, ask for an urgent abdominal US and/or CT to confirm the diagnosis, and start as far as possible the recommended treatment, achieving as that a significative morbimortality reduction so common in this severe complication (AU)

[Severe cerebellar mutism after posterior fossa tumor resection]. / Mutismo cerebeloso grave tras cirugía de un tumor de fosa posterior.

Cerebellar mutism is an infrequent but important complication after posterior fossa surgery in children. Dysarthria, irritability and ataxia are among the signs and symptoms of this disorder, which are usually mild and self-limiting. However, in severe cases, there can be impairment of higher-level cognitive functions, affecting the child's future personal and social relations. This disorder has been described in many other situations and consequently pediatricians should be familiar with its symptoms, physiopathology, diagnosis, degrees of severity, treatment, and prognosis, since a multidisciplinary approach is required. We present the case of a 5-year-old boy who underwent surgery for a low-grade ependymoma in the fourth ventricle; 48 hours after surgical resection, the boy developed irritability, cranial nerve involvement and stereotyped movements in the context of active hydrocephalus. His symptoms progressively improved 6 weeks after the intervention. We review the literature on cerebellar mutism and discuss the physiopathology of this disorder, which seems to confirm that the cerebellum not only acts as a simple coordinator of motor function, but also plays an important role in higher-level cognitive functions, such as language.

Mutismo cerebeloso grave tras cirugía de un tumor de fosa posterior / Severe cerebellar mutism after posterior fossa tumor resection

El mutismo cerebeloso es una complicación infrecuente pero con gran trascendencia en los niños intervenidos de cirugía de fosa posterior. Disartria, irritabilidad y ataxia, son parte de sus síntomas y signos, que suelen ser leves y autolimitados, pero en algunos casos graves, pueden alterarse funciones cognitivas superiores, pudiendo verse comprometidas las relaciones personales y sociales futuras del niño. Está descrito en otras situaciones, por lo que es importante para el pediatra el conocimiento de sus síntomas, fisiopatología, diagnóstico, grados de severidad y enfoque terapéutico, así como su pronóstico, dado que requiere de un tratamiento multidisciplinario. Presentamos el caso clínico de un niño de 5 años intervenido de un ependimoma de bajo grado localizado en el cuarto ventrículo, que desarrolló 48 h después de la resección quirúrgica, un cuadro consistente en mutismo, irritabilidad, afectación de pares craneales y movimientos estereotipados, en el contexto de una hidrocefalia activa. La clínica del paciente mejoró progresivamente a partir de 1,5 mes tras la intervención. Revisamos la bibliografía existente sobre el mutismo cerebeloso y discutimos su fisiopatología, que parece confirmar que el cerebelo no participa únicamente como un simple coordinador de la función motora, sino que desempeña un papel relevante en funciones cognitivas superiores, como el lenguaje

Cerebellar mutism is an infrequent but important complication after posterior fossa surgery in children. Dysarthria, irritability and ataxia are among the signs and symptoms of this disorder, which are usually mild and self-limiting. However, in severe cases, there can be impairment of higher-level cognitive functions, affecting the child's future personal and social relations. This disorder has been described in many other situations and consequently pediatricians should be familiar with its symptoms, physiopathology, diagnosis, degrees of severity, treatment, and prognosis, since a multidisciplinary approach is required. We present the case of a 5-year-old boy who underwent surgery for a low-grade ependymoma in the fourth ventricle; 48 hours after surgical resection, the boy developed irritability, cranial nerve involvement and stereotyped movements in the context of active hydrocephalus. His symptoms progressively improved 6 weeks after the intervention. We review the literature on cerebellar mutism and discuss the physiopathology of this disorder, which seems to confirm that the cerebellum not only acts as a simple coordinator of motor function, but also plays an important role in higher-level cognitive functions, such as language

[Neonatal neoplasms: a single-centre experience]. / Neoplasias neonatales: experiencia de un centro.

INTRODUCTION: Malignant tumors are uncommon in the neonatal period and benign tumors may have malignant potential. OBJECTIVES: To describe the neoplasms diagnosed and treated in newborns (