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Resumen Objetivos El objetivo principal es determinar la supervivencia global y la supervivencia libre de enfermedad de pacientes intervenidos de liposarcoma retroperitoneal, comparandolos en funcion de los subtipos histologicos bien diferenciado y desdiferenciado. Los objetivos secundarios son analizar descriptivamente las caracteristicas clinicas de estos pacientes e identificar otras variables independientes que puedan modificar significativamente estos perfiles de supervivencia. Metodos Se realiza un estudio observacional y analitico mediante una cohorte historica retrospectiva, seguida prospectivamente. Los criterios de inclusion fueron: cirugia de liposarcoma de localizacion retroperitoneal, subtipos histologicos bien diferenciado y desdiferenciado, entre enero de 2002 y mayo de 2019. Se incluyeron un total de 32 pacientes. Se utilizo el estimador de Kaplan-Meier para resumir los datos y la prueba log-rank para el analisis comparativo. Resultados La supervivencia global a los 5 anos fue del 59%. No se encontraron diferencias entre los pacientes con subtipo bien diferenciado con respecto al desdiferenciado (p = 0,834). La supervivencia libre de enfermedad a los 2 anos fue del 59% en los bien diferenciados y del 26% en los desdiferenciados, siendo estas diferencias estadisticamente significativas (p = 0,005). Ninguna de las otras variables estudiadas modifico significativamente estos perfiles de supervivencia. Conclusiones La supervivencia libre de enfermedad de los liposarcomas retroperitoneales desdiferenciados es significativamente menor que en los bien diferenciados, pero no puede afirmarse que haya diferencias en la supervivencia global.

Resumen La hiperplasia difusa idiopatica de celulas neuroendocrinas pulmonares es una entidad poco frecuente, actualmente reconocida por la Organizacion Mundial de la Salud como una condicion premaligna. Esta entidad puede presentarse con formacion de tumorlets (agregados de celulas neuroendocrinas de hasta 5 mm), dando una caracteristica imagen histologica y radiologica. Describimos el caso de una mujer de 67 anos que presentaba tos de larga evolucion y una TC que mostraba multiples nodulos milimetricos bilaterales pulmonares, a quien se le extrajeron 2 cunas pulmonares que evidenciaron una hiperplasia difusa idiopatica de celulas neuroendocrinas pulmonares con varias formaciones tumorlets. Tras 2 anos de seguimiento, los estudios de imagen realizados no presentan cambios significativos.

We present the case of a stromal ovarian sclerosing tumor in a woman of the third decade of life. These tumors derive from the stroma-sexual cords of the ovary, have a low incidence and exhibit a benign behavior. the available literature is reviewed to date, given the importance of proper differential diagnosis.

Background: Merkel cell carcinoma (MCC) is a malignant skin cancer with a 5-year survival rate of approximately 50%. Knowledge of MCC has increased in recent years mostly due to improved diagnosis techniques. In Spain there is lack of information regarding the incidence and tumour characteristics, and the treatment approaches are not standardised. The objective of this study was to provide information of the clinical and epidemiological characteristics of MCC patients in Spain. Methods: Retrospective, observational study involving 192 patients from 25 Spanish hospitals. Evaluated variables included overall survival and incidence rate of Merkel cell polyomavirus, in patients diagnosed from 2012 to 2016. Results: The Spanish incidence rate was estimated 0.32/100,000 inhabitants/year, with variations according to geographical regions, being slightly higher in areas with greater sunlight exposure. In total, 61.5% of tumours showed expansive growth (progressive growth of the tumour), 78.6% showed localisation in UV-exposed skin. 97.4% of patients were diagnosed by excisional biopsy. Surgery was the first line treatment in 96.6% of patients, radiotherapy in 24.6%, and chemotherapy in 6.3%. These treatments were not mutually exclusive. Median overall survival was 38.3 months (78.4% at 12 months and 60% at 24 months). MCPyV was present in 33.8% of patients. Conclusion: The incidence of MCC in Spain is one of the highest in Europe, with a slight predominance in men. The sample has shown that a biopsy is available for diagnosis in most cases. Moreover, the treatment is surgical when the tumour is localized and is associated with lymphadenectomy, and/or it is radiotherapy if widespread, This study has been developed with GETHI group (Spanish group of orphan and infrequent tumours, from the Spanish Grupo Espanol ˜ de Tumores Hu´erfanos e Infrecuentes) as promotor. This research was financially supported by Merck KGaA, Darmstadt, Germany, as part of an alliance between Merck KGaA and Pfizer. Merck KGaA, Darmstadt, Germany and Pfizer reviewed the manuscript for medical accuracy only before journal submission. The authors are fully responsible for the content of this manuscript, and the views and opinions described in the publication reflect solely those of the authors

The main objective is to establish the overall survival and disease-free survival profiles regarding the patients with retroperitoneal liposarcoma, making a comparison based on the well-differentiated and dedifferentiated histological subtypes. The secondary objectives are to descriptively analyze the clinical characteristics of said patients and to identify and analyze other independent variables that might modify these survival profiles significantly.An observational and analytical study was performed using a retrospective historical cohort that was followed prospectively. The inclusion criteria consisted of: the procedure of liposarcoma located in the retroperitoneum, the well-differentiated and dedifferentiated histological subtypes, between January of 2002 and May of 2019. As a result, 32 patients took part in this study's sample. Kaplan-Meier estimator was used to summarise the results and log-rank test was used in the comparative analysis.The overall survival at 5 years was around 59%. No differences were found between the patients with a well-differentiated subtype compared to the dedifferentiated ones (p = 0.834). The disease-free survival at 2 years was 59% regarding the well-differentiated and 26% regarding the dedifferentiated, with these differences being statistically significant (p = 0.005). None of the other studied variables modified these survival profiles significantly.Dedifferentiated retroperitoneal liposarcomas show less disease-free survival than well-differentiated liposarcomas. However, regarding overall survival no differences can be claimed.

Se presenta el caso de un tumor estromal esclerosante ovárico en una mujer de la tercera década de la vida. Estos tumores derivan del estroma-cordones sexuales del ovario, tienen una escasa incidencia y presentan un comportamiento benigno. Se revisa la literatura disponible hasta el momento, dada la importancia del adecuado diagnóstico diferencial., We present the case of a stromal ovarian sclerosing tumor in a woman of the third decade of life. These tumors derive from the stroma-sexual cords of the ovary, have a low incidence and exhibit a benign behavior. the available literature is reviewed to date, given the importance of proper differential diagnosis.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia is an infrequent lesion recently classified by the WHO as preinvasive. It can present with the formation of tumorlets (neuroendocrine cell groups up to 5 mm) which result in a typical histological and radiological image. We report a case of a 67-year-old women who presented with a chronic cough. The CT scan showed bilateral minute, multiple pulmonary nodules. A biopsy revealed a diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with several tumorlets. After two years of follow-up, imaging studies showed no significant changes.