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Introducción El hamartoma fibroso de la infancia (HFI) es tumor poco frecuente de tejidos blandos en lactantes y niños, caracterizado histopatólogicamente por su morfología trifásica y curso benigno. Métodos Presentamos las características histopatológicas e inmunohistoquímicas de una serie de 21 casos de HFI y revisamos sus principales diagnósticos diferenciales. Resultados Las lesiones predominaron en varones con una edad media de 3,7 años (5 meses-24 años) en localizaciones previamente descritas. Histopatológicamente, se caracterizaron por proporciones variables de tejido fibroblástico, mesenquimal y adiposo maduro. Tres casos (21%) mostraron zonas hialinizadas con artefactos en grietas, que imitaban al fibroblastoma de células gigantes. Presentaron tinción positiva para actina de músculo liso 8/13 (61%) en el componente fibroblástico, CD34 en 6/8 (75%) en el mesenquimal inmaduro y fibroblástico; y S100 en el tejido adiposo en 7/7 (100%). Conclusión Nuestros hallazgos histopatológicos son superponibles a los descritos en series amplias. No obstante, la heterogeneidad clínica obliga a conocer las variantes de esta entidad dentro de su típica histomorfología trifásica, para lograr un correcto diagnóstico y manejo terapéutico (AU)

Background Fibrous hamartoma of infancy (FHI) is a rare soft-tissue tumor usually seen in infants and young children. Histologically, the tumor is characterized by a triphasic morphology, and the clinical course is benign. Methods We described the histopathologic and immunohistochemical features in a series of 21 cases of FHI and reviewed the main entities to consider in differential diagnosis. Results Most patients in the series were male. The mean age was 3.7 years (range, 5 months–24 years), and lesions were found in locations that have been previously reported. Histopathologically, the tumors were composed of variable proportions of fibroblastic, mesenchymal, and mature adipose tissue. Three of the 13 immunohistochemically stained biopsies (14%) contained hyalinized zones with cracking artifacts, mimicking giant cell fibroblastoma. Eight of the 13 stained with smooth-muscle actin (61%) were positive in the fibroblastic component, 6 of the 8 stained with CD34 (75%) were positive in the immature mesenchymal and fibroblastic components, and all 7 stained with S100 were positive (100%) in adipose tissue. Conclusions Our histopathologic findings are consistent with those described in larger series. However, in order to reach a precise diagnosis and plan treatment, clinical heterogeneity obliges us to become familiar with variations in the characteristic triphasic histology of FHI (AU)

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In pediatric patients with sarcomas, hepatoblastomas, or other types of primary tumors, lung metastases are often found at diagnosis or during follow-up. The wide variety of primary tumors and clinical situations makes management and follow-up of these patients challenging. Chest CT is the best way to detect the dissemination of disease to the lungs. Many pulmonary nodules are nonspecific, and many might not be pathological. Others have characteristics that make them suspicious. Although there are some general features that indicate that a pulmonary nodule is likely to be a metastasis, sometimes the meaning of these features depends on the primary tumor. Furthermore, metastases can develop during the course of the disease, and the protocols for follow-up are different for different primary tumors. We review the different protocols used at our hospital for the primary tumors that most often metastasize to the lungs, including the criteria for lung metastases and the follow-up for each primary tumor.

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Introducción: Los tumores localizados en el tallo cerebral en los niños y adolescentes conllevan un mal pronóstico, especialmente aquellos infiltrantes y difusos. Con el tratamiento de radioterapia apenas llegan a más de 15 por ciento de supervivencia y no mejora la cifra con quimioterapia agregada. Objetivos: Estimar el efecto de la asociación del tratamiento radiante con el anticuerpo monoclonal Nimotuzumab en la supervivencia de niños y adolescentes con tumores del tallo cerebral. Método: Estudio clínico no aleatorizado, analítico, longitudinal y prospectivo. Se estudió una serie de 46 pacientes entre 2 y 18 años de edad que padecían de tumores del tallo cerebral, infiltrantes y difusos, desde enero de 2008 y en seguimiento hasta marzo de 2018. Todos se trataron con radioterapia, con dosis entre 54 y 59,8 cGrey, dosis diaria de 1,8 cGrey, y se irradiaban de lunes a viernes. Mientras duró el tratamiento radiante recibieron Nimotuzumab, en la dosis de 150 mg/m2 de superficie corporal, luego semanal con 8 dosis, y finalmente mensual durante uno o dos años. Resultados: Se alcanzó en la serie una supervivencia media de 18,4 meses, y una esperada de 42,9 por ciento a 2 años y 35,5 por ciento a 5 años, estabilizada hasta los 10 años. Conclusiones: La combinación de radioterapia y el anticuerpo monoclonal Nimotuzumab incrementa la supervivencia en niños y adolescentes con tumores del tallo cerebral y es bien tolerada, aun en periodos prolongados, e incluso en casos de recidiva(AU)

Introduction: Tumors localized in the brainstem of children and adolescents entail a bad prognosis, especially those that are intrinsic and diffuse. With radiotherapy treatment, patients barely get a 15 percent of survival, and the numbers don't improve with added chemotherapy. Objectives: To estimate the effect of the association of radiotherapy treatment with Nimotuzumab monoclonal antibody in the survival of children and adolescents with brainstem tumors. . Method: Non randomized, analytical, longitudinal and prospective clinical study that was authorized by the National Regulatory Authority. There was studied a group of 46 patients aged from 2 to 18 years that suffered from intrinsic and diffuse brainstem tumors, from January 2008 (and in follow up) to March 2018. All the patients were treated with radiotherapy, with doses among 54 and 59,8 Grey, daily doses of 1,8 Grey, and from Monday to Friday. While they were under radiotherapy treatment, they get Nimotuzumab, in doses of 150 mg/m2 of corporal surface; then weekly doses of 8 shots; and finally, monthly doses during one or two years. Results: In this group there was a survival mean of 18, 4 months, and an expected survival of 42, 9 percent for 2 years and 35, 5 percent for 5 years that can be stabilized to 10 years. Conclusions: Combination of radiotherapy and Nimotuzumab monoclal antibody can increase the survival from brainstem tumors in children and adolescents(AU)

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Introducción: El hamartoma fibroso de la infancia es una proliferación benigna de los tejidos blandos, de presentación infrecuente; 91 % de los casos ocurre durante el primer año de edad y afecta más frecuentemente al sexo masculino. Se caracteriza por ser una lesión subcutánea de morfología característica con patrón de crecimiento organoide trifásico. Caso clínico: Lactante masculino de seis meses de edad, con tumor en la región plantar medial del pie izquierdo, el cual fue resecado completamente. En el estudio histopatológico se informó como hamartoma fibroso de la infancia. Conclusiones: Puede localizarse en cualquier sitio anatómico, aunque 5 a 10 % afecta las extremidades inferiores existen pocos casos informados en el pie. En biopsias pequeñas es importante su diagnóstico diferencial con otras lesiones fibroadiposas para el adecuado tratamiento. La resección quirúrgica amplia con márgenes libres de lesión confiere un buen pronóstico a los pacientes.

BACKGROUND: Fibrous hamartoma of infancy (FHI) is an infrequent benign proliferation of the soft tissues. Ninety one percent of cases occur during the first year of life. FHI is characterized as a subcutaneous lesion with characteristic morphology with a triphasic organoid growth pattern. CLINICAL CASE: We present the case of a 6-month-old male infant with a tumor in the medial plantar region of the left foot, which was completely withered. Histopathological study reported a fibrous hamartoma of infancy. CONCLUSIONS: Fibrous hamartoma of infancy most frequently affects males. It may be localized at any anatomic site, although 5-10% of cases affect the lower limbs. Few cases are reported in the foot. This is a lesion with a characteristic morphological pattern; however, in small biopsies, its differential diagnosis is important with other fibroadipose lesions for appropriate treatment. Ample surgical resection with lesion-free borders confers a good prognosis for these patients.

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Mutations in the gene TP53, which codifies the tumor suppressor protein p53, are found in about 50% of tumors. These mutations can occur not only at somatic level, but also in germline. Pediatric cancer patients, mostly with additional family history of malignancy, should be considered as potential TP53 germline mutation carriers. Germline TP53 mutations and polymorphisms have been widely studied to determine their relation with different tumors' pathogenesis. Our aim was to analyze the occurrence frequency of germline TP53 mutations and polymorphisms and to relate these to tumor development in a pediatric series. Peripheral blood mononuclear cell samples from 26 children with solid tumors [PST] and 21 pediatric healthy donors [HD] were analyzed for germline mutations and polymorphisms in TP53 gene spanning from exon 5 to 8 including introns 5 and 7. These PCR amplified fragments were sequenced to determine variations. A heterozygous mutation at codon 245 was found in 1/26 PST and 0/21 HD. Comparative polymorphisms distribution, at position 14181 and 14201(intron 7), between HD and PST revealed a trend of association (p= 0.07) with cancer risk. HD group disclosed a similar polymorphism distribution as published data for Caucasian and Central/South American populations. This is the first study about TP53 variant frequency and distribution in healthy individuals and cancer patients in Argentina.

El gen que codifica para la proteína supresora de tumor p53 (TP53) se encuentra mutado en aproximadamente el 50% de los tumores. Estas mutaciones pueden presentarse como somáticas o en línea germinal. Los niños con tumores, sobre todo aquellos con historia familiar de enfermedad oncológica, deben considerarse potenciales portadores de mutaciones en línea germinal. Las mutaciones de TP53 y los polimorfismos son estudiados para determinar su relación con la patogénesis de diferentes tumores. El objetivo del trabajo fue analizar la frecuencia de mutaciones y polimorfismos en línea germinal de TP53 y relacionarlos con el desarrollo de tumor en un grupo de pacientes pediátricos. Se analizaron muestras de sangre periférica de 26 pacientes con tumores sólidos [PST] y 21 niños donantes sanos [HD] para determinar la presencia de mutaciones y polimorfismos de TP53 en línea germinal. Se analizó por PCR seguida de secuenciación, la región que comprende a los exones 5 a 8 (incluyendo intrones 5 y 7). En 1/26 PST se encontró una mutación heterocigótica en el codón 245. La distribución de los polimorfismos, en la posición 14181 y 14201 (intrón 7), entre HD y PST mostró una tendencia de asociación (p = 0.07) con el riesgo para desarrollar cáncer. La frecuencia de distribución de dichos polimorfismos en HD fue similar a la publicada para poblaciones caucásicas y de América Central/del Sur. Este estudio aporta información original sobre la frecuencia de distribución de las variantes TP53 en individuos sanos y con tumores en la Argentina.

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OBJETIVO: Analisar o impacto da adição da temozolamida à radioterapia em tumores de tronco cerebral em crianças. MATERIAL E MÉTODO: Entre 2000 e 2005 foram analisadas, retrospectivamente, 64 crianças com tumor do tronco cerebral. Dessas crianças, 32 receberam temozolamida(grupo 1) e 32 não a receberam (grupo 2). RESULTADOS: A idade mediana no grupo 1 foi de 8,2 anos e no grupo 2 foi de 7,5 anos. A localização tumoral era predominantemente difusa (53%) emambos os grupos. Todos os pacientes receberam radioterapia com doses superiores a 50 Gy. No grupo1 foram ministrados nove ciclos, em média, de quimioterapia (3û14 ciclos). O tempo de progressão de doença foi de 7,9 meses no grupo 2 versus 13,8 meses no grupo 1. A sobrevida global foi de 8,8 meses (0,3û30,9 meses) no grupo 1 e de 14,6 meses (4,3û33 meses) no grupo 2. CONCLUSÃO: A utilização da temozolamida após a radioterapia proporcionou aumento da sobrevida, deseis meses em média, nos pacientes pediátricos com tumor do tronco cerebral.

OBJECTIVE: To analyze the impact of adding temozolomide to radiotherapyin pediatric brain stem tumors. MATERIAL AND METHOD: Between 2000 and 2005, 64 children with brain stem tumor were analyzed: 32 received temozolomide (group 1) and 32 did not(group 2). RESULTS: The median age of patients in group 1 was 8.2 year-old and in group 2 was 7.5 year-old. The predominant tumoral localization was diffuse (53%) in both groups. All of the patients were submitted to radiotherapy. In group 1, the median number of temozolomide cycles was 9 (3û14 cycles). Time of disease progression was 7.9 months in group 2 versus 13.8 months in group 1. Overall survival was 8.8 months (0.3û30.9 months) in group 1 and 14.6 months (4.3û33 months) in group 2. CONCLUSION: In our institution,adding temozolomide to radiotherapy increased the overall survival in approximately six months in brain stem pediatric tumors.

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