KMT2D suppresses Sonic hedgehog-driven medulloblastoma progression and metastasis.

The major cause of treatment failure and mortality among medulloblastoma patients is metastasis intracranially or along the spinal cord. The molecular mechanisms driving tumor metastasis in Sonic hedgehog-driven medulloblastoma (SHH-MB) patients, however, remain largely unknown. In this study we define a tumor suppressive role of KMT2D (MLL2), a gene frequently mutated in the most metastatic ß-subtype. Strikingly, genetic mouse models of SHH-MB demonstrate that heterozygous loss of Kmt2d in conjunction with activation of the SHH pathway causes highly penetrant disease with decreased survival, increased hindbrain invasion and spinal cord metastasis. Loss of Kmt2d attenuates neural differentiation and shifts the transcriptional/chromatin landscape of primary and metastatic tumors toward a decrease in differentiation genes and tumor suppressors and an increase in genes/pathways implicated in advanced stage cancer and metastasis (TGFß, Notch, Atoh1, Sox2, and Myc). Thus, secondary heterozygous KMT2D mutations likely have prognostic value for identifying SHH-MB patients prone to develop metastasis.

[Management of pancreatic cystic tumors in the Alberto Sabogal Sologuren hospital]. / Manejo de tumores quísticos del páncreas en el hospital Alberto Sabogal Sologuren.

INTRODUCTION: Cystic tumors of the pancreas comprise 1% of all neoplasms of the pancreas and 10 to 15% of pancreatic cysts. There are a variety of cystic lesions, of which 90% is made up of serous cystadenomas, mucinous cystic neoplasms, intraductal mucinous neoplasms and solid pseudopapillary neoplasms. MATERIALS AND METHODS: This study describes and analyzes retrospectively the clinical, radiological, surgical, pathological and follow up of 12 patients operated on for cystic tumors of the pancreas in the hospital IV Alberto Sabogal Sologuren, in the period 2005 to 2010. RESULTS: we found 5 (41%) serous cystadenomas with a mean age of 66 years, localized 80% in the head and 20% body; 2 (17%) mucinous cystic neoplasms with a mean age of 54, all located in body, 2 (17%) intraductal mucinous neoplasms with a mean age of 63, all located in the head, and 3 (25%) solid pseudopapillary neoplasms with a mean age of 33 years, located in body 33% and 66% in tail with a predominance of females in a ratio of 3:1. Had abdominal pain (75%), weight loss (17%) and palpable mass (17%). Of the 2 cystic mucinous neoplasms, only one have a low-grade dysplasia, of the two mucinous intraductal neoplasms, one have grade moderate dysplasia and the other with a high degree, the rest of cystic neoplasms were benign. We realize 6 Pancreaticoduodenectomy, 4 corporocaudales pancreatectomies, 2 distal pancreatectomies; of them splenectomy realize in 4 patients (2 in corporocaudal pancreatectomies and 2 distal pancreatectomies). In all cases the preoperative diagnosis was based on abdominal TEM. in 4 patients was expanded with RMN for suspicion of mucinous tumor and in 2 patients was performed CPRE for suspected intraductal tumors. Two patients coursed with atelectasis, and one patient had pancreatic fistula grade A and other mild pancreatitis post-operative. No patient was reoperated. There was no mortality post operative. Postoperative was more for the pancreaticoduodenectomy group. In a 2 year follow up, no observed recurrence and all patients are alive. CONCLUSION: Preoperative diagnosis is crucial given the differences in natural history of the spectrum of lesions. Despite improved radiographic imaging, techniques, definitive diagnosis is only made after studying the resection sample.

Manejo de tumores quísticos del páncreas en el Hospital Alberto Sabogal Sologuren / Management of cystic tumors of the pancreas in the Hospital Alberto Sabogal Sologuren

INTRODUCCIÓN: Los tumores quísticos del páncreas comprenden el 1 % de todas las neoplasias del páncreas y el 10 al 15% de los quistes pancreáticos. Existe una gran variedad de lesiones quísticas, de ellos el 90% está constituido por cistoadenomas serosos, neoplasias mucinosas quísticas, neoplasias intraductales mucinosas y las neoplasias sólido pseudopapilares. MATERIALES Y MÉTODOS: El presente estudio describe y analiza en forma retrospectiva las características clínicas, radiológicas, quirúrgicas, patológicas y de seguimiento de 12 pacientes operados por tumores quísticos del páncreas en el hospital IV Alberto Sabogal Sologuren, en el periodo 2005 al 2010. RESULTADOS: Se encontraron 5 (41%) cistoadenomas serosos con una edad media de 66 años, localizándose 80% en cabeza y 20% en cuerpo; 2 (17%) neoplasias mucinosas quísticas con una edad media de 54 años, todas localizadas en cuerpo; 2 (17%) neoplasias intraductales mucinosas con una edad media de 63 años, todas localizadas en cabeza, y 3 (25%) neoplasias sólido pseudopapilares con una edad media de 33 años, ubicadas 33% en cuerpo y 66% en cola, con predominancia del sexo femenino en una relación de 3:1. Presentaron dolor abdominal (75%), baja de peso (17%) y masa palpable (17%). De las 2 neoplasias mucinosas quísticas, solo uno presentó displasia de bajo grado, de las 2 neoplasias intraductales mucinosas 1 resultó con displasia de grado moderado y otra de alto grado, el resto de neoplasias quísticas resultaron benignas. Se realizaron 6 pancreatoduodenectomías, 4 pancreatectomías corporocaudales, 2 pancreatectomías distales; de ellas se realizó esplenectomía en 4 pacientes (2 en pancreatectomías corporocaudal y 2 pancreatectomías distales). En todos los casos el diagnóstico preoperatorio se basó en TEM abdominal, en 4 pacientes se amplió con RMN por sospecha de tumor mucinoso y en 2 se realizó CPRE por sospecha de tumores intraductales. ...

INTRODUCTION: Cystic tumors of the pancreas comprise 1% of all neoplasms of the pancreas and 10 to 15% of pancreatic cysts. There are variety of cystic lesions, of which 90% is made up of serous cystadenomas, mucinous cystic neoplasms, intraductal mucinous neoplasms and solid pseudopapillary neoplasms. MATERIALS AND METHODS: This study describes and analyzes retrospectively the clinical, radiological, surgical, pathological and follow up of 12 patients operated on for cystic tumors of the pancreas in the hospital IV Alberto Sabogal Sologuren, in the period 2005 to 2010. RESULTS: We found 5 (41%) serous cystadenomas with a mean age of 66 years, localized 80% in the head and 20% body; 2 (17%) mucinous cystic neoplasms with a mean age of 54, all located in the head, and 3 (25%) solid pseudopapillary neoplasms with a mean age of 33 years, located in body 33% and 66% in tail with a predominance of females in a ratio of 3:1. Had abdominal pain (75%), weight loss (17%) and palpable mass (17%). Of the 2 cystic mucinous neoplasms, only one have a low-grade dysplasia, of the two mucinous intraductal neoplasms, one have grade moderate dysplasia and the other with a high dregree, the rest of cystic neoplasms were beningn. We realize 6 Pancreaticoduodenectomy, 4 corporocaudales pancreatectomies. 2 distal pancreatectomies; of them splenectomy realize in 4 patients (2 in corporocaudal pancreatectomies and 2 distal pancreatectomies). In all cases the preoperative diagnosis aws based on abdominal TEM. in 4 patients was expanded with RMN for suspicion of mucinous tumor and in 2 patients was performed CPRE for suspected intraductal tumors. Two patients coursed with atelectasis, and one patiernt had pancreatic fistula grande A and other mild pancreatitis post-operative. No patient was reoperated. There was no mortality post operative. Postoperative was more for the pancreaticoduodenectomy group. In a 2 year folow up, no oberved recurrence and alla patients are alive. ...

Prevalencia de infección en un Unidad de Quemados / Prevalence of infection in an burned victim unit

Se realizó un estudio descriptivo con el objetivo de determinar la prevalencia de infección en 195 pacientes ingresados en la Unidad de Quemados Pearl F. Ort del Hospital Dr. Luis E. Aybar durante el período febrero de 1993 a febrero de 1994. Encontrándose que el 54.4// de los casos desarrolló infección; el microorganismo aislado con mayor frecuencia fue la Pseudomona aeruginosa, con 34.6//; en el 28.3// la infección se localizó en la quemadura y el 30.2// del total de los pacientes fallecieron