Analysis of potential risk factors in the survival of patients with primary retroperitoneal liposarcoma / Análisis de los potenciales factores de riesgo en la supervivencia de pacientes con liposarcoma retroperitoneal primario

Introduction: The present work is an observational study of a series of variables regarding overall survival and disease-free survival in patients diagnosed with primary liposarcoma.Methods: The study is prospective with retrolective data collection that includes all patients with primary liposarcoma referred to Hospital Son Espases University Hospital, Palma de Mallorca, Spain from January 1990 to December 2019.Results: The study includes 50 patients and the compartment surgery was performed in 18 patients (36%) of cases. The mean overall survival of the sample was 15.57 years (95% CI: 12.02–19.12) and the mean disease-free survival was 6.70 years (95% CI: 4.50–8.86). Conclusion: Compartment surgery has not shown benefits in terms of overall survival and disease-free survival. The ASA classification (≥3) predicts a poor prognosis in both overall survival and disease-free survival. Resection with free margins, described on the pathological results and defined in this work as R0, show better disease-free survival. (AU)

Introducción: El presente trabajo es un estudio observacional de una serie de variables relacionadas con la supervivencia global y la supervivencia libre de enfermedad en pacientes diagnosticados de liposarcoma primario. Métodos: Este es un estudio prospectivo con recolección de datos retrolectiva que incluye a todos los pacientes con liposarcoma primario remitidos al Hospital Son Espases en Palma de Mallorca, desde enero de 1990 hasta diciembre de 2019. Resultados: El estudio incluye 50 pacientes y la cirugía compartimental se realizó en 18 (36%) de ellos. La supervivencia global media de la muestra fue de 15,57 años (IC 95% 12,02-19,12) y la supervivencia libre de enfermedad media fue de 6,70 años (IC 95% 4,50-8,86). Conclusiones: La cirugía compartimental no ha mostrado beneficios en términos de supervivencia general y supervivencia libre de enfermedad. La clasificación ASA (≥3) predice un mal pronóstico tanto en la supervivencia global como en la supervivencia libre de enfermedad. La resección con márgenes libres, descrita en los resultados patológicos y definida en este trabajo como R0, muestra una mejor supervivencia libre de enfermedad. (AU)

Analysis of potential risk factors in the survival of patients with primary retroperitoneal liposarcoma.

INTRODUCTION: The present work is an observational study of a series of variables regarding overall survival and disease-free survival in patients diagnosed with primary liposarcoma. METHODS: The study is prospective with retrolective data collection that includes all patients with primary liposarcoma referred to Hospital Son Espases University Hospital, Palma de Mallorca, Spain from January 1990 to December 2019. RESULTS: The study includes 50 patients and the compartment surgery was performed in 18 patients (36%) of cases. The mean overall survival of the sample was 15.57 years (95% CI: 12.02-19.12) and the mean disease-free survival was 6.70 years (95% CI: 4.50-8.86). CONCLUSION: Compartment surgery has not shown benefits in terms of overall survival and disease-free survival. The ASA classification (≥3) predicts a poor prognosis in both overall survival and disease-free survival. Resection with free margins, described on the pathological results and defined in this work as R0, show better disease-free survival.

Analysis of potential risk factors in the survival of patients with primary retroperitoneal liposarcoma.

INTRODUCTION: The present work is an observational study of a series of variables regarding overall survival and disease-free survival in patients diagnosed with primary liposarcoma. METHODS: The study is prospective with retrolective data collection that includes all patients with primary liposarcoma referred to Hospital Son Espases University Hospital, Palma de Mallorca, Spain from January 1990 to December 2019. RESULTS: The study includes 50 patients and the compartment surgery was performed in 18 patients (36%) of cases. The mean overall survival of the sample was 15.57 years (95% CI: 12.02-19.12) and the mean disease-free survival was 6.70 years (95% CI: 4.50-8.86). CONCLUSION: Compartment surgery has not shown benefits in terms of overall survival and disease-free survival. The ASA classification (≥3) predicts a poor prognosis in both overall survival and disease-free survival. Resection with free margins, described on the pathological results and defined in this work as R0, show better disease-free survival.

Recidiva de carcinoma nasosinusal relacionado con el virus del papiloma humano con características similares al adenoide quístico / Recurrence of human papillomavirus-related carcinoma with adenoid cystic-like features

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Tumores del estroma gastrointestinal: breve actualización y consenso de la SEAP-SEOM sobre diagnóstico patológico y molecular / A brief up-date and consensus about the pathological and molecular diagnosis of gastrointestinal stromal tumours from the SEAP-SEOM

Los tumores del estroma gastrointestinal (GIST) son las neoplasias mesenquimales más frecuentes del tubo digestivo con una incidencia de 1,1 casos/100.000 habitantes/año. Un grupo de expertos de la Sociedad Española de Anatomía Patológica y la Sociedad Española de Oncología se reunieron para realizar una breve actualización de los GIST y consensuar los aspectos relacionados con su diagnóstico patológico y molecular. Los GIST generalmente son lesiones solitarias, bien circunscritas, de dimensiones variables (< 10 mm-35cm), que pueden presentar crecimiento parietal intra o extraluminal o mixto (en reloj de arena). Histológicamente, son neoplasias no encapsuladas, con crecimiento expansivo y celularidad fusiforme (70%), epitelioide (20%) o mixta (10%). La actividad mitótica generalmente es moderada o baja y debe evaluarse exclusivamente en los territorios con mayor celularidad o con mayor número de mitosis. La gran mayoría de los GIST presentan mutaciones activadoras, mutuamente excluyentes, en los genes que codifican para los receptores tirosina quinasa de tipo iii KIT y PDGFRA; con mucha menor frecuencia también se han descrito mutaciones en otros genes, tales como BRAF, NF1, y genes del complejo SDH. El método de detección de mutaciones de KIT y PDGFRA más ampliamente utilizado es la amplificación mediante reacción en cadena de la polimerasa de los exones de interés y la secuenciación directa (método Sanger) de estos productos de amplificación. Los informes moleculares siempre deben especificar el tipo de análisis llevado a cabo y la región o mutaciones que se han evaluado, así como indicar la sensibilidad del método de detección empleado (AU)

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the digestive tract, with an incidence of 1.1 cases/100,000 inhabitants/year. A group of experts from the Spanish Societies of Pathology and Oncology met to discuss a brief update on GISTs and agree on aspects relating to the pathological and molecular diagnosis of these tumors. GISTs are generally solitary, well-circumscribed lesions of variable size (<10 mm-35cm) that may present with intra- or extra-luminal parietal growth or a mixed-type (hourglass) growth pattern. Histologically, they are unencapsulated neoplasms displaying expansive growth and spindle-shaped (70%), epithelioid (20%) or mixed cellularity (10%). Mitotic activity is generally moderate or low and should be evaluated only in areas with high cellularity or higher mitotic frequency. The great majority of GISTs harbour mutually-exclusive activating mutations in genes coding for the type iii receptor tyrosine kinases KIT and PDGFRA; less commonly, GISTs have also been reported to display mutations elsewhere, including BRAF and NF1 and SDH-complex genes. The method most widely used to detect KIT and PDGFRA mutations is amplification of the exons involved by polymerase chain reaction followed by direct sequencing (Sanger method) of these amplification products. Molecular analyses should always specify the type of analysis performed, the region or mutations evaluated and the sensitivity of the detection method employed (AU)

Histological differences between various methods of hemiepiphysiodesis: is guided growth really different?

Traditional systems of hemiepiphysiodesis are based on the application of asymmetrical compression to the physis to correct angular deformities. The guided growth method claims to act as a tension plate avoiding compression. The aim of this study was to confirm or refute this claim. Twenty-four White New Zealand rabbits were subjected to a proximal tibial hemiepiphysiodesis using either staples or a plate and two-screws method. Both methods succeeded in producing deformity. The initial existent histological differences between systems became less apparent after 6 weeks of hemiepiphysiodesis, when histological results were very similar. The findings suggest that the eight-plate system produces, like staples, compression of the physis, but the forces are applied more gradually.

NIK controls classical and alternative NF-κB activation and is necessary for the survival of human T-cell lymphoma cells.

PURPOSE: Peripheral T-cell lymphomas (PTCL) are a heterogeneous entity of neoplasms with poor prognosis, a lack of effective therapies, and a largely unknown molecular pathology. Deregulated NF-κB activity has been associated with several lymphoproliferative diseases, but its importance in T-cell lymphomagenesis is poorly understood. We investigated the function of the NF-κB-inducing kinase (NIK), in this pathway and its role as a potential molecular target in T-cell lymphomas. EXPERIMENTAL DESIGN: We used immunohistochemistry to analyze the expression of different NF-κB members in primary human PTCL samples and to study its clinical impact. With the aim of inhibiting the pathway, we used genetic silencing of NIK in several T-cell lymphoma cell lines and observed its effect on downstream targets and cell viability. RESULTS: We showed that the NF-κB pathway was activated in a subset of PTCLs associated with poor overall survival. NIK was overexpressed in a number of PTCL cell lines and primary samples, and a pivotal role for NIK in the survival of these tumor cells was unveiled. NIK depletion led to a dramatic induction of apoptosis in NIK-overexpressing cell lines and also showed a more pronounced effect on cell survival than inhibitor of kappa B kinase (IKK) knockdown. NIK silencing induced a blockage of both classical and alternative NF-κB activation and reduced expression of several prosurvival and antiapoptotic factors. CONCLUSIONS: The results of the present study indicate that NIK could be a promising therapeutic target in these aggressive malignancies.

Guided growth: the importance of a single tether. An experimental study.

BACKGROUND: During the last few years, the use of the 8-plate as a technique for hemiepiphysiodesis has gained wide acceptance, as it has been shown that it works in a more physiological way than other methods such as staples or transphyseal screws. It has mechanically been compared with a tension band plate, and for this reason, only a single plate is needed. The following study was undertaken to test the 8-plate against the staples and assess factors that can influence the difference in results. METHODS: A prospective experimental study was designed to compare the ability of 2 hemiepiphysiodesis methods, the 8-plate and the double staple, to produce angular deformity in the rabbit's tibia (group I). The variable studied was the variations in the articular line-diaphysis angle at 6 weeks. As the results showed that the 8-plate produced a bigger deformity, a second group was designed (group II) comparing single against double staple, under the hypothesis that the differences observed in the first group could be related to the number of anchors put across the physis and consequently depend on the volume of physis involved by the staples. RESULTS: In group I, the 8-plate produced a bigger angulation at 6 weeks' time than the 2 staples (a difference of 6.5 degrees, P = 0.03). Similarly, in group II, the single staple produced a greater angulation than the 2 staples (difference 6 degrees, P = 0.08). When both groups were compared, no differences in the angulation produced by the 8-plate and the single staple with respect to the 2 staples were found. CONCLUSIONS: These results suggest that one of the reasons why the 8-plate may act in a more "physiological way" (vs. the traditional 2-staple or 3-staple hemiepiphysiodesis) could be the fact that the growth plate is tethered only at a single point. Therefore, the physis retains a major potential for growth and deformity. CLINICAL RELEVANCE: The 8-plate is superior in producing/correcting angular deformity when compared with the traditionally used staples (2 or more) but not when compared with a single staple.

Screw length in the guided growth method.

BACKGROUND: The appearance of the 8-plate as a method for hemiepiphysiodesis has renewed the interest for the use of this technique. However, many questions remain unanswered about the way of action of the guided growth method. Although screw length has been said to play no role, to our knowledge, no clinical or experimental evidence exists. METHODS: An experimental prospective randomized study with 40 WNZ Rabbits aged 8 weeks was conducted. Four experimental groups were established. Each tibia was randomly allocated to one of the following groups: Staples (group 1), 8-plate either using self-taping screws of 9 mm (group 2), or 5 mm lengths (groups 3) and control (group 4). Radiological assessment of the tibial deformity was done in a weekly fashion, and ALDA (articular line-diaphyseal angle) variations at 6 weeks were used as the control variable. RESULTS: The 8-plate as a whole produced a significant bigger deformity than the staples (10°). No significant differences between the two models of the 8-plate were found along the study (3.7°). CONCLUSIONS: The 8-plate has shown to be more efficient in producing angular deformity than staples. However, the length of the screw has showed no role in the 8-plate function.