[Pancreatic metastasis from undifferentiated pleomorphic sarcoma. An extremely rare entity]. / Metástasis pancreática de sarcoma pleomórfico indiferenciado. Una entidad extremadamente rara.

Undifferentiated pleomorphic sarcoma (UPS) is a very aggressive malignant tumor that originates in bone or soft tissues, being its most frequent location in the extremities. We present the case of a 71-year-old man who, two years of follow-up after a lower right limb UPS excision surgery, presented a single metastasis of sarcomatoid origin in the pancreas that was treated by cephalic pancreaticoduodenectomy. Sarcomatoid metastases to the pancreas are extremely rare. Resection of the metastasis is the only therapeutic alternative that has reported benefit in terms of the survival of these patients.

El sarcoma pleomórfico indiferenciado (SPI) es un tumor maligno muy agresivo que se origina en el hueso o tejidos blandos, siendo su localización más frecuente en las extremidades. Presentamos el caso de un varón de 71 años que a los dos años de seguimiento tras cirugía de exéresis de un SPI presenta una metástasis única de origen sarcomatoide a nivel del páncreas que se trató mediante duodenopancreatectomía cefálica. Las metástasis sarcomatoides a nivel de páncreas son extremadamente raras. La cirugía de exéresis de la metástasis es la única alternativa terapéutica que ha reportado beneficio en cuanto la supervivencia de estos pacientes.

Ruptured subcapsular liver hematoma as a rare complication of HELLP syndrome. A therapeutic challenge.

HELLP syndrome (HS), a low-incidence condition of uncertain pathogenesis associated with pregnancy hypertensive syndromes, is characterized by hemolysis, elevated liver enzymes and low platelet count. Ruptured subcapsular liver hematoma complicated with hemoperitoneum is an uncommon but very serious condition where early recognition and multidisciplinary management are key to reduce its associated maternal, infant mortality rate. Symptoms are nonspecific, characterized by por epigastric pain, nausea and vomiting; clinical suspicion and appropriate imaging studies are of crucial importance. We report the case of a 36-year-old primiparous woman at 39 weeks of gestation. She was admitted for early membrane rupture, with delivery complicated by retained placenta. During the immediate puerperium she had blood pressure > 140/90 mmHg, epigastric pain and vomiting, which required respiratory and hemodynamic support. An exploratory laparotomy was performed that revealed a massive hemoperitoneum as well as CR in the RLL with multifocal active bleeding. The left liver lobe was macroscopically normal. The patient underwent hemoperitoneum drainage and hepatic packing (HP); biopsy findings were consistent with necrosis. Polytransfusion was initiated with blood products and antihemorrhagic agents.

Ventral Portal Vein Over Gallbladder and Pancreas---An Exceptional Anatomical Variation Found in a Liver Transplant Patient: A Case Report.

BACKGROUND: Anatomic variations are well known in the liver hilum. A rare precholecystic, preduodenal, prepancreatic portal vein is described as found in a liver transplant candidate. Precholecystic location of portal vein is an exceptional finding and does not seem to have been previously described. It is associated with a preduodenal portal vein. Its position is challenging, as its surface can be mistaken with the gallbladder wall. We present the case of a patient candidate to liver transplantation. In the preoperative studies, a portal thrombosis was suspected, with recanalization by collaterals, but also a malformation was suggested. The patient had a primary biliary cirrhosis. Other findings included agenesis of inferior vena cava on the right side. During operation, the portal vein was found over the gallbladder and fixed to it, making it at first difficult to distinguish one from the other. CONCLUSIONS: A precholecystic portal vein is a rare finding that poses a challenge for the surgeon. It must be ruled out in the preoperative workout.

Estudio multicéntrico nacional sobre pancreatectomías totales / Multicentric study on total pancreatectomies

Introducción: La pancreatectomía total (PT) es una intervención infrecuente, con unas indicaciones no claramente definidas y unos resultados postoperatorios no estandarizados. Presentamos un estudio multicéntrico nacional sobre PT y una comparación con la literatura existente. Métodos: Estudio prospectivo observacional realizado mediante el registro nacional de pacientes operados de duodenopancreatectomía cefálica y PT realizadas por cualquier indicación durante el periodo comprendido entre el 1 enero y el 31 diciembre del 2015. Resultados: Se incluyó a 1.016 pacientes, pertenecientes a 73 centros; de ellos, 112 correspondían a PT. El porcentaje de PT/número total de casos es del 11%. La edad media fue 63,5 años y eran varones un 57,2%. El diagnóstico radiológico de sospecha más frecuente fue cáncer de páncreas (58/112 casos). La técnica de la PT más habitual fue «arteria mesentérica primero» (43/112 casos). Se efectuaron resecciones venosas en 23 pacientes (20,5%). El porcentaje de complicaciones postoperatorias a 90 días fue 50%, pero las complicaciones mayores (> IIIA) solo el 20,7%. La mortalidad global a 90 días fue del 8% (9 pacientes). La estancia media fue 20,7 días. Los 3 diagnósticos histológicos definitivos más frecuentes fueron: adenocarcinoma de páncreas, neoplasia mucinosa papilar intraductal y pancreatitis crónica. La tasa de R0 fue del 67,8%. Conclusiones: Este estudio demuestra que los resultados de morbimortalidad de la PT en España son similares o superiores a los publicados previamente. Es necesario un estudio más específico sobre PT centrado en complicaciones específicas, como la insuficiencia endocrina

Introduction: Total pancreatectomy (TP) is an uncommon operation, with indications that have not been clearly defined and non-standardized postoperative results. We present a national multicentric study on TP and a comparison with the existing literature Methods: A prospective observational study using data from the national registry of patients after pancreaticoduodenectomy and TP performed for any indication during the study period: January 1 to December 31, 2015 Results: 1016 patients were included from 73 hospitals, 112 of whom had undergone TP. The percentage of TP from the total number of cases was 11%. The mean age was 63.5 years, and 57.2% were males. The most frequently suspected radiological diagnosis was pancreatic cancer (58/112 cases). The most common TP technique was "mesentery artery first" (43/112 cases). Venous resections were performed in 23 patients (20.5%). The percentage of postoperative complications within 90 days was 50%, but major complications (>IIIA) were only 20.7%. The overall 90-day mortality was 8% (9 patients). The average stay was 20.7 days. The 3 most frequent definitive histological diagnoses were: adenocarcinoma of the pancreas, intraductal papillary mucinous neoplasm and chronic pancreatitis. The R0 rate was 67.8%. Conclusions: This study shows that the morbidity and mortality results of TP in Spain are similar or superior to previous publications. More precise TP studies are necessary, focused on specific complications such as endocrine insufficiency

Multicentric study on total pancreatectomies. / Estudio multicéntrico nacional sobre pancreatectomías totales.

INTRODUCTION: Total pancreatectomy (TP) is an uncommon operation, with indications that have not been clearly defined and non-standardized postoperative results. We present a national multicentric study on TP and a comparison with the existing literature METHODS: A prospective observational study using data from the national registry of patients after pancreaticoduodenectomy and TP performed for any indication during the study period: January 1 to December 31, 2015 RESULTS: 1016 patients were included from 73 hospitals, 112 of whom had undergone TP. The percentage of TP from the total number of cases was 11%. The mean age was 63.5 years, and 57.2% were males. The most frequently suspected radiological diagnosis was pancreatic cancer (58/112 cases). The most common TP technique was "mesentery artery first" (43/112 cases). Venous resections were performed in 23 patients (20.5%). The percentage of postoperative complications within 90 days was 50%, but major complications (>IIIA) were only 20.7%. The overall 90-day mortality was 8% (9 patients). The average stay was 20.7 days. The 3most frequent definitive histological diagnoses were: adenocarcinoma of the pancreas, intraductal papillary mucinous neoplasm and chronic pancreatitis. The R0 rate was 67.8%. CONCLUSIONS: This study shows that the morbidity and mortality results of TP in Spain are similar or superior to previous publications. More precise TP studies are necessary, focused on specific complications such as endocrine insufficiency.

Rectorragia como forma de presentación de un caso de síndrome de Abernethy en el adulto / Lower gastrointestinal bleeding as a form of presentation in an adult case of Abernethy syndrome

Presentamos el caso de una paciente de 29 años que consultó por rectorragias autolimitadas que fue diagnosticada de síndrome de Abernethy (shunt portosistémico congénito). Se trata de un debut clínico poco habitual en este tipo de síndrome congénito muy poco prevalente. La gran mayoría de los casos descritos en la literatura se manifiestan clínicamente en la edad pediátrica

We report the case of a 29-year-old patient who presented with a short history of lower gastrointestinal bleeding. A diagnosis of Abernethy syndrome was made (congenital extrahepatic portosystemic shunt) after this uncommon clinical presentation. The prevalence of this congenital malformation is very low and usually manifests during the pediatric age, according to previously published reports

Lower gastrointestinal bleeding as a form of presentation in an adult case of Abernethy syndrome.

We report the case of a 29-year-old patient who presented with a short history of lower gastrointestinal bleeding. A diagnosis of Abernethy syndrome was made (congenital extrahepatic portosystemic shunt) after this uncommon clinical presentation. The prevalence of this congenital malformation is very low and usually manifests during the pediatric age, according to previously published reports.