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Introducción. La neoplasia sólida pseudopapilar de páncreas, también conocida como tumor de Frantz, es una patología poco frecuente, que comprende menos del 3 % de los tumores pancreáticos, y la mayor incidencia se observa en mujeres jóvenes entre la segunda y tercera década de la vida. Su etiología es desconocida. El tratamiento de elección es la resección quirúrgica sin linfadenectomía cuando se encuentra bien localizado. Inclusive aún cuando hay evidencia de metástasis o invasión local, el manejo quirúrgico sigue siendo el tratamiento de elección. Caso clínico. Mujer de 24 años de edad, con masa abdominal palpable, dolorosa, de tres semanas de evolución. La tomografía computarizada mencionó como presunción diagnóstica un tumor de origen pancreático. En la laparotomía se encontró una masa dependiente del cuerpo del páncreas. Se realizó pancreatectomía distal radical con esplenectomía y omentectomía parcial en bloque. Resultados. Durante la cirugía no se presentaron complicaciones y hasta el sexto mes de seguimiento, la evolución fue adecuada y sin recidiva tumoral. Conclusiones. Ante la presencia de masa abdominal, con compromiso pancreático documentado por tomografía computarizada, se debe considerar el diagnóstico de neoplasia sólida pseudopapilar de páncreas, o tumor de Frantz. El tumor de Frantz es una entidad poco frecuente que generalmente se diagnostica de forma incidental o por síntomas inespecíficos en estadios avanzados. El diagnóstico y manejo de esta neoplasia representa un reto y la resección quirúrgica continúa siendo el tratamiento indicado.

Introduction. Solid pseudopapillary neoplasia of the pancreas, also known as Frantz tumor, is a rare pathology, comprising less than 3% of pancreatic tumors, and the highest incidence is observed in young women between the second and third decades of life. Its etiology is unknown. The treatment of choice is surgical resection without lymphadenectomy when it is well located. Even when there is evidence of metastasis or local invasion, surgical management remains the treatment of choice. Clinical case. A 24-year-old woman presented with a palpable, painful abdominal mass of three weeks' duration. The computed tomography mentioned a tumor of pancreatic origin as a presumptive diagnosis. At laparotomy, a mass dependent on the body of the pancreas was found. Radical distal pancreatectomy with splenectomy and en bloc partial omentectomy was performed. Results. During the surgery there were no complications and until the sixth month of follow-up, the evolution was adequate and without tumor recurrence. Conclusions. In the presence of an abdominal mass, with pancreatic involvement documented by computed tomography, the diagnosis of solid pseudopapillary neoplasia of the pancreas, or Frantz tumor, should be considered, which must be treated surgically. Frantz tumor is a rare entity that is generally diagnosed incidentally or due to nonspecific symptoms in advanced stages. The diagnosis and management of this neoplasia represents a challenge and surgical resection continues to be the indicated treatment.

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Introducción. El esplenúnculo se ha descrito con una incidencia global del 10 al 30 %. Puede ser una entidad de origen congénito o adquirida, frecuentemente está asociada a trauma abdominal o antecedente de esplenectomía por diversas causas. Caso clínico. Mujer en edad media, con antecedente de trauma abdominal y esplenectomía por ruptura traumática, quien 30 años después presenta un cuadro de dolor abdominal. Los estudios imagenológicos identificaron una masa sólida intrapancreática. Resultados. Fue llevada a pancreatectomía distal. Los hallazgos histológicos e inmunohistoquímicos confirmaron que la masa pancreática correspondía a un esplenúnculo intrapancreático adquirido, asociado al evento traumático previo. Conclusión. Los esplenúnculos suelen constituir un "incidentaloma". Los estudios imagenológicos se encuentran limitados, pues la tomografía computarizada, la resonancia nuclear magnética y la ultrasonografía presentan características imagenológicas similares con los tumores pancreáticos hipervascularizados, por lo que se debe practicar el estudio histopatológico durante su valoración. Esta entidad se debe incluir dentro de los diagnósticos diferenciales, con mayor énfasis en aquellos pacientes con historia de trauma abdominal y esplenectomía asociada, un escenario en el que esta lesión puede simular una neoplasia sólida del páncreas, con características malignas.

Introduction. Splenunculus has been described with an overall incidence of 10 to 30%. It can be an entity of congenital or acquired origin, it is frequently associated with abdominal trauma or a history of splenectomy for various reasons. Clinical case. Middle-aged woman, with a history of abdominal trauma and splenectomy due to traumatic rupture, who 30 years later presents with abdominal pain. Imaging studies identified a solid intrapancreatic mass. Results. She was taken to OR for distal pancreatectomy. The histological and immunohistochemical findings confirmed that the pancreatic mass corresponded to an acquired intrapancreatic splenunculus, associated with the previous traumatic event. Conclusion. Splenuncles usually constitute an "incidentaloma". Imaging studies are limited, since computed tomography, magnetic resonance imaging, and ultrasonography present similar imaging characteristics with hypervascularized pancreatic tumors, so histopathological study must be included during their evaluation. This entity should be included in the differential diagnoses, with greater emphasis on those patients with a history of abdominal trauma and associated splenectomy, a scenario in which this lesion can simulate a solid neoplasm of the pancreas, with malignant characteristics.

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Presentación del caso. Se trata de una mujer de 26 años que presentó dolor en epigastrio e hipocondrio izquierdo, con aumento del perímetro abdominal y pérdida de 5 kg de peso corporal. En el examen físico se detectó una masa de gran tamaño en el epigastrio, con bordes regulares, ligeramente dolorosa al tacto y no móvil. Los estudios de imagen revelaron una neoplasia mixta en el cuerpo y cola pancreática. Intervención terapéutica. Se practicó una esplenopancreatectomía corpo-caudal, con extirpación completa del tumor. Evolución clínica. La paciente recibió cuidados especializados y vigilancia estrecha posquirúrgica en la unidad de cuidados intensivos, sin presentar complicaciones relevantes. Tras el alta hospitalaria, la paciente refirió un buen estado general en los controles de seguimiento, que incluyeron una tomografía realizada a los 12 meses, donde no se evidenciaron restos o recidivas tumorales

Case presentation. A 26-year-old woman who presented with pain in the epigastrium and left hypochondrium, with increased abdominal perimeter and loss of 5 kg of body weight. Physical examination revealed a large mass in the epigastrium, with regular borders, slightly painful to the touch and non-mobile. Imaging studies revealed a mixed neoplasm in the pancreatic body and tail. Treatment. A corpo-caudal splenopancreatectomy was performed, with complete removal of the tumor. Outcome. The patient received specialized care and close postoperative surveillance in the intensive care unit, with no relevant complications. After hospital discharge, the patient reported a good general condition in the follow-up controls, which included a computed tomography scan performed after 12 months, where no tumor remnants or recurrences were evidenced

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INTRODUCTION AND AIMS: Distal pancreatectomy is a frequent procedure and postoperative fistula, its most common complication, has an incidence of 30 to 60%. The aim of the present work was to study the role of the neutrophil-to-lymphocyte ratio and the platelet-to-lymphocyte ratio, as indicators of inflammatory response in the setting of pancreatic fistula. METHODS: A retrospective observational study was conducted on patients that underwent distal pancreatectomy. The diagnosis of postoperative pancreatic fistula was made according to the definition proposed by the International Study Group on Pancreatic Fistula. The relation of postoperative pancreatic fistula to the neutrophil-to-lymphocyte ratio and the platelet-to-lymphocyte ratio was determined in the postoperative evaluation. SPSS v.21 software was utilized for the statistical analysis and a P<.05 was considered statistically significant. RESULTS: A total of 12 patients (27.2%) developed grade B or grade C postoperative pancreatic fistula. ROC curves were constructed and a threshold of 8.3 (PPV 0.40, NPV 0.86) was established for the neutrophil-to-lymphocyte ratio, with an area under the curve of 0.71, sensitivity of 0.81, and specificity of 0.62, whereas a threshold of 33.2 (PPV 0.50, NPV 0.84) was established for the platelet-to-lymphocyte ratio, with an area under the curve of 0.72, sensitivity of 0.72, and specificity of 0.71. CONCLUSION: The neutrophil-to-lymphocyte ratio and the platelet-to-lymphocyte ratio are serologic markers that can aid in identifying patients that will present with grade B or grade C postoperative pancreatic fistula, thus helping to provide an opportune focus on care and resources.

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Introducción. El quiste hidatídico puede localizarse en cualquier órgano del cuerpo. El quiste hidatídico en el páncreas (QHP)se presenta con una prevalencia menor a 1%, la localización más frecuente es la cabeza en 61% a 69%, en el cuerpo y cola en 31% a 39%. No es fácil diferenciar el quiste hidatídico pancreático de otros tumores quísticos del páncreas, por lo que esta patología debe tenerse en cuenta en el diagnóstico diferencial de lesiones quísticas pancreáticas. Caso Clínico. Paciente femenino de 66 años quien desde el año 2016 se encontraba en control por una tumoración quística de la cola pancreática de 1,7 cm, diagnosticada en forma incidental. La paciente cursó asintomática y se controló en forma anual. El año 2021 la lesión alcanzó un tamaño de 4,7 cm. En este contexto se decide la resección quirúrgica por vía laparoscópica. Se resecó una pieza quirúrgica de 8 cm de longitud que incluye la cola del páncreas y el quiste. La histología describe la membrana prolígera y múltiples escólex viables. Conclusión. La localización primaria en la cola del páncreas de un quiste hidatídico confunde el diagnóstico con un tumor quístico mucinoso. El tratamiento quirúrgico actual en los quistes distales debería ser la resección laparoscópica.

Introduction. The hydatid cyst can be located in any organ of the body. The hydatid cyst in the pancreas occurs with a prevalence of less than 1%, the most frequent location is the head in 61% to 69%, the body and tail in 31% to 39%. It is not easy to differentiate the pancreatic hydatid cyst from other cystic tumors of the pancreas, so this pathology must be taken into account in the differential diagnosis of pancreatic cystic lesions. Clinical Case. The present report discusses a 66-year-old female patient who had been in control since 2016 for a 1.7 cm cystic tumor of the pancreatic tail. The patient was asymptomatic and was controlled annually. In 2021, the lesion reached a size of 4.7 cm. In this context, laparoscopic surgical resection was decided. An 8 cm long surgical specimen was resected, including the tail of the pancreas and the cyst. Histology describes prolific membrane and multiple viable scolexes. Conclusion. The primary location in the tail of the pancreas of a hydatid cyst confuses the diagnosis with a mucinous cystic tumor. The current surgical treatment for distal cysts should be laparoscopic resection.

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Abstract True splenic artery aneurysms are exceedingly rare and the medical literature contains only a limited number of reports on this pathology. Presently, there remains a lack of consensus regarding the optimal management and treatment approaches for patients in this category. Over the course of the last century, significant changes have occurred in the realm of surgical options, transitioning from open and endovascular procedures to the more advanced laparoscopic and robotic interventions. The propensity for these aneurysms to rupture underscores the need for timely intervention. The risk of rupture is notably elevated in patients harboring giant splenic artery aneurysms. In this report, we present the case of a 55-year-old woman diagnosed with a giant splenic artery aneurysm measuring 12x12 cm in diameter. She presented with notable weakness, discomfort, and pain in the left subcostal area. In response to her complaints and after thorough evaluation, we opted for a surgical procedure encompassing distal pancreatic resection in conjunction with splenectomy and resection of the giant splenic artery aneurysm.

Resumo Os aneurismas verdadeiros da artéria esplênica são extremamente raros, e há um número limitado de relatos sobre essa condição na literatura médica. Atualmente, não há consenso sobre as abordagens ideais de manejo e tratamento para pacientes que se enquadram nessa categoria. Ao longo do século passado, ocorreram mudanças significativas no domínio das opções cirúrgicas, passando de procedimentos abertos e endovasculares para intervenções laparoscópicas e robóticas mais avançadas. A propensão à ruptura do aneurisma ressalta a necessidade de intervenção em tempo oportuno. O risco de ruptura é notavelmente elevado em pacientes com aneurismas gigantes da artéria esplênica. Neste relato, apresentamos o caso de uma mulher de 55 anos diagnosticada com aneurisma gigante de artéria esplênica medindo 12x12 cm de diâmetro. A paciente apresentava fraqueza notável, desconforto e dor na região subcostal esquerda. Em resposta às suas queixas e após avaliação minuciosa, optamos por um procedimento cirúrgico que incluiu pancreatectomia distal associada a esplenectomia e ressecção do aneurisma gigante da artéria esplênica.

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ABSTRACT BACKGROUND: Minimally invasive distal pancreatectomy (MIDP) is associated with less blood loss and faster functional recovery. However, the benefits of robotic assisted distal pancreatectomy (RDP) over laparoscopic distal pancreatectomy (LDP) are unknown. AIMS: To compare RDP versus LDP for surgical treatment of benign lesions, pre-malignant and borderline malignant pancreatic neoplasias. METHODS: This is a retrospective study comparing LDP with RDP. Main outcomes were overall morbidity and overall costs. Secondary outcomes were pancreatic fistula (PF), infectious complications, readmission, operative time (OT) and length of hospital stay (LOS). RESULTS: Thirty patients submitted to LDP and 29 submitted to RDP were included in the study. There was no difference regarding preoperative characteristics. There was no difference regarding overall complications (RDP - 72,4% versus LDP - 80%, p=0,49). Costs were superior for patients submitted to RDP (RDP=US$ 6,688 versus LDP=US$ 6,149, p=0,02), mostly due to higher costs of surgical materials (RDP=US$ 2,364 versus LDP=1,421, p=0,00005). Twenty-one patients submitted to RDP and 24 to LDP developed pancreatic fistula (PF), but only 4 RDP and 7 LDP experienced infectious complications associated with PF. OT (RDP=224 min. versus LDP=213 min., p=0.36) was similar, as well as conversion to open procedure (1 RDP and 2 LDP). CONCLUSIONS: The postoperative morbidity of robotic distal pancreatectomy is comparable to laparoscopic distal pancreatectomy. However, the costs of robotic distal pancreatectomy are slightly higher.

RESUMO RACIONAL: A pancreatectomia distal minimamente invasiva (PDMI) está associada a menos perda sanguínea e recuperação funcional mais rápida, no entanto, os benefícios da pancreatectomia distal robótica (PDR) são desconhecidos quando comparada a pancreatectomia distal laparoscópica (PDL). OBJETIVOS: Comparar PDR versus PDL no tratamento cirúrgico de lesões benignas, neoplasias pancreáticas malignas, pré-malignas e limítrofes. MÉTODOS: Estudo retrospectivo comparando PDL com PDR. Os desfechos primários avaliados foram morbidade e custos hospitalares. Os desfechos secundários foram fístula pancreática (FP), complicações infecciosas, readmissão, tempo cirúrgico e tempo de internação hospitalar (TIH). RESULTADOS: Trinta pacientes submetidos a PDL e 29 submetidos a PDR foram incluídos no estudo. Não houve diferença em relação às características pré-operatórias. Não houve diferença em relação às complicações gerais (PDL - 72,4% versus PRD - 80%, p=0,49). Os custos foram superiores para PDR (PDR=US$ 6688 versus PDL=US$ 6149, p=0,02), principalmente devido aos custos mais elevados de materiais cirúrgicos (PDR=US$ 2364 versus PDL=1421, p=0,00005). Vinte e um pacientes submetidos a PDR e 24 submetidos a PDL desenvolveram fístula pancreática (PF), no entanto, apenas 4 submetidos PDR e 7 a PDL apresentaram complicações infecciosas associadas a FP. O tempo cirúrgico (PDR=224 min. versus PDL=213 min., p=0,36) e a conversão para cirurgia aberta (1 PDR e 2 PDL) não tiveram diferença estatística. CONCLUSÕES: A morbidade pós operatória da pancreatectomia distal robótica é comparável à pancreatectomia distal laparoscópica. Entretando, os custos da pancreatectomia distal robótica são mais elevados.

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El tumor pseudopapilar de páncreas, conocido como tumor de Frantz, es excepcional; comprende entre el 0.2 y 2.7 % de los carcinomas de páncreas. Su etiología es desconocida y predomina en mujeres jóvenes. Son tumores de bajo potencial maligno, que cursan asintomáticos y dan síntomas por compresión regional: dolor gravativo y/o masa abdominal palpable. La probabilidad de una forma definidamente maligna es de 15 %. Anatómicamente se localizan con mayor frecuencia en la cola del páncreas, seguido por la cabeza y el cuerpo. El tratamiento es la resección quirúrgica y su pronóstico es excelente, con sobrevida a 5 años de casi 100 %. El diagnóstico es anátomo-patológico, considerando el aspecto microscópico y el perfil inmunohistoquímico. Se presenta un caso de una mujer de 21 años, en la cual se realizó una cirugía de excéresis completa del tumor y que se mantiene a asintomática a 24 meses de operada.

Pseudo papillary tumor of the pancreas, also called Frantz´s tumor, is a very rare disease; between 0.2 and 2.7 % of pancreatic carcinomas. Ethiology is unknown; is observed in young women. It´s a tumors with low malignant potential, usually of asymptomatic evolution by long time and the symptoms are due to regional compression: pain or abdominal mass. The possibility of a malignant form is around 15 %. Anatomically, they are most frequently located in the tail of the pancreas, followed by the head and body. The treatment is the surgical resection; with an excellent prognosis and a 5-year survival almost 100 %. The diagnosis is with pathological study, considering the microscopic appearance and the immunohistochemical profile. Its perform an analysis of the literature and once case of a 21 years old, woman is reported. A complete resection surgery of the tumor was performed and remains asymptomatic at 24 months of follow-up.

O tumor pseudopapilar do pâncreas, conhecido como tumor de Frantz, é excepcional; compreende entre 0,2 e 2,7 % dos carcinomas pancreáticos. Sua etiologia é desconhecida e predomina em mulheres jovens. São tumores de baixo potencial maligno, que são assintomáticos e apresentam sintomas devido à compressão regional: dor intensa e/ou massa abdominal palpável. A probabilidade de uma forma definitivamente maligna é de 15 %. Anatomicamente, eles estão localizados com mais frequência na cauda do pâncreas, seguido pela cabeça e corpo. O tratamento é a ressecção cirúrgica e seu prognóstico é excelente, com sobrevida em 5 anos de quase 100 %. O diagnóstico é anátomo-patológico, considerando o aspecto microscópico e o perfil imuno-histoquímico. Apresenta-se o caso de uma mulher de 21 anos submetida a cirurgia de excisão completa do tumor e que permanece assintomática 24 meses após a cirurgia.

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Resumen: La hipoglucemia hiperinsulinémica del recién nacido es una enfermedad hereditaria que cursa con alteraciones en las células pancreáticas; la incidencia estimada es de 1/50,000 nacidos vivos aumentando en casos de consanguinidad. Patología considerada como causa principal de hipoglucemias en este grupo etario con alto riesgo de daño cerebral y retraso mental que impacta significativamente en el neurodesarrollo. La euglucemia y la prevención de lesión cerebral por neuroglucopenia son los principales objetivos del tratamiento médico. Durante el transanestésico de una pancreatectomía, los niveles de glucosa son de utilidad como marcadores; el objetivo del abordaje perioperatorio es mantener estabilidad hemodinámica y euglucemia a través de un adecuado manejo de líquidos. Se presenta el caso de paciente femenino de dos meses de edad con diagnóstico de hipoglucemia hiperinsulinémica con hipoglucemias severas asociadas a crisis convulsivas, ingresa a quirófano para pancreatectomía subtotal.

Abstract: Hyperinsulinemic hypoglycemia of the newborn is a hereditary disease that presents with alterations in pancreatic cells; the estimated incidence is 1/50,000 newly borns increasing in cases of consanguinity. Pathology considered as the main cause of hypoglycemia in this age group with a high risk of brain damage and mental retardation, significantly impacting neurodevelopment. Euglycemia and the prevention of brain injury from neuroglucopenia are the main objectives of medical treatment. During the transanesthetic of a pancreatectomy, the glucose levels are useful as markers; the objective of the perioperative management is to keep hemodynamic stability and euglycemia through proper fluid handling. In this case report we present a two-month-old female patient with a diagnosis of hyperinsulinemic hypoglycemia with severe hypoglycemia associated with seizures that enters the operating room for subtotal pancreatectomy.

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AIMS: To compare the short-term outcomes of patients undergoing open DP (ODP) and laparoscopic DP (LDP); and to analyze the association between the section site of the pancreas and pancreatic fistula. MATERIALS AND METHODS: Clinical, perioperative, and histopathologic data of patients who underwent ODP and LDP between 2009 and 2019 were retrospectively analyzed. RESULTS: 70 patients were included. 39 (56%) underwent ODP and 31 (44%) underwent LDP. The tumor size in ODP group was 70mm and in LDP group was 45mm (p = 0,032) Blood loss was lower in LDP group (229mL versus 498mL) (p = 0,001). Operative time, spleen preservation, B/C pancreatic fistula, major morbidity, reoperation, and length of hospital stay, were similar in both groups. There was no postoperative mortality. No differences were found in B/C pancreatic fistula rate regarding to pancreatic transection site. CONCLUSIONS: LDP is a safe procedure, with perioperative outcomes similar to ODP and with less blood loss. The pancreatic transection site did not influence post-operative pancreatic fistula rate.

OBJETIVOS: Comparar los resultados a corto plazo de pacientes intervenidos mediante pancreatectomía distal abierta (PDA) y laparoscópica (PDL); y analizar si el lugar de la sección del páncreas tiene relación con la formación de fístula pancreática. MATERIALES Y MÉTODOS: Serie retrospectiva y descriptiva de las PD realizadas, desde enero del 2009 a diciembre del 2019. Se compararon las características clínicas, perioperatorias e histopatológicas de pacientes con PDA y PDL. RESULTADOS: Se incluyeron 70 pacientes. Treinta y nueve casos (56%) con PDA y 31 casos (44%) con PDL. El tamaño tumoral promedio en la PDA fue de 70 mm y en la PDL 45 mm (p = 0.032). La pérdida sanguínea fue menor en la PDL (229 vs. 498 ml) (p = 0.001). No se encontró diferencia significativa en tiempo operatorio, porcentaje de preservación esplénica, fístula pancreática B/C, reoperación, morbilidad mayor y estancia hospitalaria. No hubo mortalidad postoperatoria. No hubo diferencias en la formación de fístula pancreática con respecto al lugar de sección del páncreas. CONCLUSIONES: La PDL es un procedimiento seguro, con resultados perioperatorios similares a la PDA y con menor pérdida sanguínea. El lugar de sección del páncreas no tuvo relación con la formación de fístula pancreática.

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Introdução: A neoplasia de pâncreas é uma importante causa de mortalidade em nosso meio. A cirurgia é o melhor tratamento por permitir uma maior sobrevida livre da doença, porém menos de 20% dos casos são considerados ressecáveis no momento do diagnóstico. O grande desafio são os tumores com invasão vascular. Objetivos: Avaliamos os pacientes submetidos à pancreatectomia com invasão vascular num período de 10 anos (2008-2018), no A.C.Camargo Cancer Center, com necessidade de revascularização. Analisamos as técnicas usadas, taxa de perviedade, uso de anticoagulantes e mortalidade associada à doença. Metodologia: Realizamos uma coorte retrospectiva, com 67 pacientes, cujas informações foram obtidas pelo prontuário informatizado. As análises estatísticas foram realizadas por meio do software IBM SPSS versão 25. Resultados: Identificamos uma maior incidência da doença no sexo feminino, 37 pacientes (55,2%). A idade média foi de 60,7 anos (DP: 10,5), a comorbidade mais frequente foi o diabetes mellitus em 26 pacientes (38,8%). O vaso mais restaurado foi a veia porta em 25 pacientes (37,3%). A revascularização mais realizada foi o reparo primário em 46 pacientes (68,7%) e o tratamento neoadjuvante com quimioterapia e radioterapia foi realizado em 37 (55%) e 23 pacientes (34,3%), respectivamente. O adenocarcinoma foi o tipo histológico mais prevalente, presente em 54 pacientes (81,8%). A margem tumoral livre da doença foi de 71,2% (47 pacientes). A taxa de perviedade entre os pacientes submetidos a exame de imagem, foi de 88,4% nos pacientes com adenocarcinoma submetidos ao reparo primário contra 54% dos pacientes com derivação com prótese e p=0,04. A sobrevida geral global em 5 anos é de 34,8%, de 31,8% quando avaliamos apenas os pacientes com neoplasia maligna e de 23,5% nos pacientes com adenocarcinoma.Conclusão: A perviedade precoce foi semelhante nas diversas modalidades de reconstrução, mas a tardia foi maior nos pacientes submetidos ao reparo primário. O uso de anticoagulante não determinou aumento da sobrevida. A sobrevida em 5 anos nos pacientes com adenocarcinoma foi de 23,5% e de 31,8% nos pacientes com neoplasia maligna.

Introduction: Pancreatic cancer is an important cause of mortality in the world. Surgery is the best treatment as it allows a longer disease-free survival, yet less than 20% of the cases are considered resectable at the time of diagnosis. The great challenge are tumors with vascular invasion. Objectives: We evaluated the patients who underwent pancreatectomy with vascular invasion over a period of 10 years (2008-2018), at the A.C.Camargo Cancer Center, which needed revascularization. We analyzed the techniques used, patency rate, use of anticoagulants and disease-associated mortality. Methodology: We performed a retrospective cohort with 67 patients, whose information were obtained from these patients' computerized medical records. Statistical analyzes were performed using the IBM SPSS software version 25. Results: We identified a higher incidence of the disease in females, 37 patients (55.2%). The mean age was 60.7 years (SD: 10.5), the most frequent comorbidity was diabetes mellitus in 26 patients (38.8%). The most repaired blood vessel was the portal vein in 25 patients (37.3%). The most performed type of revascularization was primary repair in 46 patients (68.7%) and neoadjuvant treatment with chemotherapy and radiotherapy was performed in 37 (55%) and 23 patients (34.3%), respectively. Adenocarcinoma was the most prevalent histological type, present in 54 patients (81.8%). The disease-free tumor margin was 71.2% (47 patients). The patency rate among the 42 patients who underwent imaging examination was 88.4% in patients with adenocarcinoma underwent primary repair versus 54% in patients with prosthesis grafts, p=0.04. Overall 5-year overall survival is 34.8%, 31.8% when evaluating only patients with malignant neoplasia, and 23.5% in patients with adenocarcinoma.Conclusion: Early patency was similar in different reconstruction modalities, but late patency was better in patients who underwent primary repair. The use of anticoagulants did not determine an increase in survival rates. The 5-year survival in patients with adenocarcinoma was 23.5% and 31.8% in patients with other malignant tumors

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ABSTRACT - BACKGROUND: Laparoscopic pancreatectomy is currently a widely used approach for benign and malignant lesions of the pancreas. AIMS: This study aimed to describe how to perform a laparoscopic distal pancreatectomy using The Clockwise Technique. METHODS: An 18-year-old female patient presented with a well-defined tumor in the pancreatic body with 4 cm in diameter that suggested a diagnosis of solid pseudopapillary tumor (Frantz's tumor). The patient was recommended for laparoscopic distal pancreatectomy by using The Clockwise Technique. RESULTS: The clockwise, caudal-to-cephalic approach appears to have other significant technical advantages that facilitate the performance of the procedure. CONCLUSIONS: A laparoscopic distal pancreatectomy performed using The Clockwise Technique provides satisfactory outcomes.

RESUMO - RACIONAL: A pancreatectomia laparoscópica está se tornando uma abordagem amplamente usada para lesões benignas e malignas do pâncreas. OBJETIVOS: Descrever como realizar a pancreatectomia distal laparoscópica usando a Clockwise Technique. MÉTODOS: Paciente feminina com 18 anos de idade, apresentando tumor bem definido no corpo pancreático com 4 cm de diâmetro que sugeria o diagnóstico de tumor sólido pseudopapilar (tumor de Frantz). O paciente foi considerado para pancreatectomia distal laparoscópica pela Clockwise Technique. RESULTADOS: A Clockwise Technique parece apresentar outras vantagens técnicas significativas que facilitam a realização do procedimento. CONCLUSÕES: A pancreatectomia distal laparoscópica foi realizada com a clockwise technique, obtendo-se resultados satisfatórios.

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ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.

RESUMO RACIONAL: A neoplasia sólida pseudopapilífera do pâncreas é um tumor pancreático incomum, mais frequente em mulheres jovens. A polipose adenomatosa familiar, por sua vez, é uma condição genética associada a câncer colorretal e que também aumenta o risco de desenvolvimento de outros tumores. OBJETIVOS: Discutir a associação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera, que é bastante rara. MÉTODOS: Reportamos dois casos de pacientes com polipose adenomatosa familiar, um homem e uma mulher, que desenvolveram neoplasia sólida pseudopapilífera do pâncreas e foram submetidos a ressecção laparoscópica com preservação esplênica. RESULTADOS: As vias de sinalização da ß-catenina e Wnt tem um papel importante na tumorigênese da neoplasia sólida pseudopapilífera, e sua ativação constitutiva devido a inativação do gene adenomatous polyposis coli na polipose adenomatosa familiar pode explicar a relação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera. CONCLUSÕES: A ressecção do cólon deve ser priorizada, com preferência pela abordagem minimamente invasiva para minimizar o risco de desenvolvimento de tumor desmoide. A ressecção pancreática geralmente não requer linfadenectomia extensa para neoplasia sólida pseudopapilífera, portanto, a preservação esplênica é factível.

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RESUMEN Introducción: Los tumores pancreáticos en el embarazo son muy raros, el diagnóstico y tratamiento en este grupo de pacientes es complejo. Objetivo: Presentar el primer caso de un leiomioma primario del páncreas en una gestante. Caso clínico: Embarazada de 26 años de edad, sin antecedentes relevantes. Primigesta, que se encontraba cursando la semana 13 de gestación. Durante una evaluación ecográfica de rutina, se le detecta una tumoración en la glándula pancreática. Se remitió a la consulta multidisciplinaria de atención a la materna y en conjunto deciden realizar la interrupción voluntaria del embarazo. Luego la paciente fue remitida a la consulta de la especialidadde Cirugía General. Al recibir a la misma, se quejaba de dolor en epigastrio e hipocondrio izquierdo, además de hipo ocasional. Al examen físico se encontró una tumoración palpable en epigastrio. Se realizaron exámenes analíticos básicos sin encontrarse alteraciones, en los estudios de imagen realizados, ecografía abdominal y tomografía contrastada de abdomen se demostró una tumoración en el cuerpo y cola del páncreas. Se planificó tratamiento quirúrgico electivo, se realizó pancreatectomía distal con esplenectomía, por invasión del estómago, se complementó con gastrectomía subtotal. La paciente evoluciona sin complicaciones. La biopsia informó un tumor mesenquimal benigno primario de páncreas, tipo leiomioma, bien encapsulado. Conclusiones: Se presenta el primer caso de un leiomioma primario del páncreas en una gestante. Los tumores pancreáticos en embarazadas son enfemedades raras, el manejo por un equipo multidisciplinario es la mejor forma de obtener resultados satisfactorios para la paciente.

ABSTRACT Introduction: Pancreatic tumors during pregnancy are very rare, diagnosis and treatment are always difficult. Objective: To present the first case of a primary pancreatic leiomyoma in a pregnant woman. Case report: A 26 years old primiparous pregnant is reported, with no relevant backgrounds. She was in her first trimesters of pregnancy. Diagnosed with a pancreatic tumor during a routine evaluation. She was referred to a multidisciplinary consult, deciding the voluntary interruption of pregnancy, then she is evaluated by the General Surgery specialty clinic for complaining of epigastric pain and hiccup. On physical examination an epigastric tumor was found. Auxiliary exams were normal. Imagenologic studies confirm a tumor in the pancreatic body and tail. Surgical treatment was scheduled, distal pancreatectomy with splenectomy, due to invasion of gastric wall subtotal distal gastrectomy was performed. Patient evolution without complications. Biopsy reported a well capsulated, leiomyoma type, primary mesenchymal pancreatic tumor. Conclusions: The first case of a primary pancreatic leiomyoma in a pregnant woman is presented. Pancreatic tumors during pregnancy are rare. Multidisciplinary approach is the best way to improve results.

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Introducción. En las hernias paraesofágicas tipo IV se produce la herniación del estómago junto a otros órganos abdominales. La herniación del páncreas es muy infrecuente.Caso clínico. Varón de 57 años que acude por dolor torácico, disnea e intolerancia al decúbito. En la tomografía computarizada toracoabdominal se observa hernia diafragmática que contiene colon transverso, intestino delgado y páncreas, con reticulación de la grasa alrededor del mismo, compatible con pancreatitis aguda. Conclusión. La asociación de hernia hiatal con páncreas herniado y pancreatitis es extremadamente infrecuente. El diagnóstico se estableció mediante tomografía computarizada y el tratamiento fue conservador, con cirugía diferida de la hernia de hiato.

Introduction. In type IV paraesophageal hernias, the stomach is herniated along with other abdominal organs. Herniation of the pancreas is very rare. Clinical case. A 57-year-old man presented with chest pain, dyspnea, and intolerance to decubitus. The thoracoabdominal computed tomography shows a diaphragmatic hernia containing the transverse colon, small intestine and pancreas, with reticulation of fat around it, compatible with acute pancreatitis. Conclusion. The association of hiatal hernia with herniated pancreas and pancreatitis is extremely rare. The diagnosis was established by computerized tomography and the treatment was conservative, with delayed surgery for the hiatal hernia.

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RESUMEN Antecedentes: la pancreatectomía posneoadyuvancia en paciente sin progresión de la enfermedad es el tratamiento estándar en el cáncer pancreático "borderline"; sin embargo, no existe hasta ahora ninguna serie nacional publicada. Objetivo: evaluar la morbilidad y mortalidad de la pancreatectomía en pacientes con cáncer de páncreas resecable "borderline" tratados previamente con neoadyuvancia. Material y métodos: se analizaron 15 pacientes tratados en el período 2011-2018. Se evaluaron los datos epidemiológicos, el tipo de neoadyuvancia, la respuesta radiológica posneoadyuvancia, la morbilidad, mortalidad y supervivencia. Se compararon los 15 pacientes pancretectomizados posneoadyuvancia con 15 pacientes pancreatectomizados sin neoadyuvancia previa. Resultados: de los 15 pacientes, 8 eran del sexo masculino y el promedio de edad fue de 66,5 años. El tipo de neoadyuvancia más frecuente fue folforinox (n = 6) y gemcitabina/placitaxel (n = 5); se adicionó radioterapia en 8 pacientes. La evaluación radiológica posneoadyuvancia mostró enfermedad estable en 10 pacientes y respuesta parcial en 4. Se realizaron 11 duodenopancreatectomías y 4 esplenopancreatectomías. En 10 pacientes fue necesario algún tipo de resección vascular. La morbilidad fue del 60% (9/6), no se registró mortalidad y la supervivencia media fue de 23,4 meses. No hubo diferencias significativas en la morbilidad, mortalidad y supervivencia cuando se comparó con los 15 pacientes pancreatectomizados sin neaodyuvancia. Conclusión: el tratamiento con neoadyuvancia en cáncer de páncreas avanzado permite ampliar su resecabilidad. La pancreatectomía posneoadyuvancia, en centros de alto volumen, tiene morbilidad, mortalidad y supervivencia similares a las de las pancreatectomías que no requieren neaoadyuvancia.

ABSTRACT Background: Pancreatectomy after neoadjuvant therapy in patients without disease progression is the standard treatment for borderline pancreatic cancer; however, no national series have been published to date. Objective: The aim of this study is to evaluate morbidity and mortality of patients with borderline resectable pancreatic cancer undergoing pancreatectomy after neoadjuvant therapy. Material and methods: A total of 15 patients treated between 2011 and 2018 were analyzed. The epidemiologic data, type of neodajuvant therapy, radiological evaluation of the response to neoadjuvant therapy, morbidity, mortality and survival were evaluated. These 15 patients who underwent pancreatectomy after neoadjuvant therapy were compared with 15 pancreatectomized patients without previous neoadjuvant therapy. Results: Mean age was 66.5 years and 8 patients were men. The most common neoadjuvant therapy regimens were FOLFIRINOX (n = 6) and gemcitabine/paclitaxel (n = 5); 8 patients required additional radiation therapy. The radiological evaluation of the response to neoadjuvant therapy showed stable disease in 10 patients and partial response in 4. Eleven patients underwent pancreaticoduodenectomy and 4 underwent splenectomy and pancreatectomy. Ten patients required some type of vascular resection. Morbidity was 60% (9/15), there were no deaths and mean survival was 23.4 months. There were no significant differences in morbidity, mortality and survival with the 15 pancreatectomized patients without previous neoadjuvant therapy. Conclusion: Neoadjuvant therapy has extended resectability of advanced pancreatic cancer. In high volume centers, pancreatectomy after neoadjuvant therapy has similar morbidity, and survival to those of pancreatic resections without previous neoadjuvant therapy.

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Resumen La placenta y el hígado son los encargados del metabolismo de los carbohidratos. La glucosa es fundamental para el metabolismo cerebral. La hipoglucemia se define con valores < 47 mg/dl. La hipoglicemia que persiste más de 7 días se atribuye a problemas metabólicos o endocrinológicos y requiere un flujo de glucosa > 12 mg/kg/min para alcanzar normo-glicemia. La hipoglicemia hiperinsulinémica congénita persistente (HHCP) es poco común (1:50,000 nacidos vivos), es la causa más común de hipoglicemia persistente secundaria a una secreción inadecuada de insulina, que puede afectar el neurodesarrollo. Hay una forma difusa y una focal, con manifestaciones clínicas idénticas, pero con mecanismos patológicos diferentes. El tratamiento médico es a base de diazóxido y ocreótide. En el 95% de los casos no hay respuesta al tratamiento médico, requiriendo pancreatectomía subtotal. Se utilizó ocreótide y nifedipino. La tomografía computada con emisión de positrones (PET/TC 18F-DOPA) encontró incremento en la capación pancreática de insulina, se realizó pancreactectomía. Se egresó sin complicaciones y en seguimiento pediátrico sin alteraciones neurológicas.

Abstract The placenta and liver are responsible for the metabolism of carbohydrates. The glucose is fundamental for brain metabolism. Hypoglycaemia is defined as values < 47 mg/dl. Hypoglycaemia that persists for more than 7 days is attributed to metabolic or endocrine problems and requires glucose flow > 12 GKM to reach normoglycemia. Persistent congenital hyperinsulinemic hypoglycemia (PCHH) is uncommon (1:50,000 live births) is the most common cause of persistent hypoglycemia secondary to inadequate insulin secretion, can significantly affect neurodevelopment. There is a diffuse and a focal form, with identical clinical manifestations, but with different pathological mechanisms. The medical treatment is diazoxide and ocreotide. In 95% of cases there is no response to medical treatment, requiring subtotal pancreatectomy. Ocreotide and nifedipine were used. Positron emission computed tomography (PET/CT 18F-DOPA) found an increase in pancreatic insulin capacity, a pancreactectomy was performed. He was discharged without complications and in pediatric follow-up without neurological alterations.

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Solid pseudopapillary tumor of the pancreas is a rare entity, more frequent in women between the 2nd and 4th decades. The diagnosis is usually incidental and it can be reached by computed tomography or magnetic resonance imaging. Subsequent pathological confirmation is necessary for an adequate treatment. A retrospective study of six cases was carried out. All the patients were female, between 14 and 56 years of age, in which 50% the tumor were an incidental finding. We had three cases located in the head and three in the body of the pancreas. We performed three pancreaticoduodenectomies and three distal pancreatectomies with splenic preservation, without disease recurrence.

El tumor sólido-quístico de páncreas es poco frecuente y predomina en mujeres entre la segunda y la cuarta décadas de la vida. Los pacientes son generalmente asintomáticos. El diagnóstico se realiza por imágenes con tomografía o resonancia magnética, y con la posterior confirmación patológica para poder ofrecer un tratamiento adecuado. Presentamos una serie de seis casos. Todas las pacientes fueron de sexo femenino, de entre 14 y 56 años. El 50% fueron un hallazgo incidental. Tuvimos tres casos localizados en la cabeza y tres en el cuerpo del páncreas. Se realizaron tres duodenopancreatectomías cefálicas y tres pancreatectomías distales con preservación esplénica, con buena evolución y sin recidiva.

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RESUMEN: La neoplasia quística mucinosa del páncreas (NQMP) es un tumor infrecuente, que afecta predominantemente a mujeres (90-95 %), afectando especialmente entre la quinta y séptima década de la vida; cuyo descubrimiento suele ser incidental. Por lo general, son lesiones solitarias, sin compromiso del conducto pancreático principal; poco sintomáticas y asociadas a malignidad (10 % a 40 %). El objetivo de este manuscrito fue reportar un caso de neoplasia quística mucinosa gigante del páncreas intervenida quirúrgicamente y revisar la evidencia existente respecto de sus características morfológicas, terapéuticas y pronósticas. Mujer de 29 años, con masa abdominal poco sintomática. El diagnóstico se verificó por medio de ultrasonografía, tomografía axial computarizada y resonancia nuclear magnética. Se intervino quirúrgicamente, realizándose pancreatectomía corporocaudal con preservación esplénica, sin incidentes. La paciente fue dada de alta al quinto día, sin complicaciones, y evolucionó de forma adecuada, sin complicaciones postoperatorias. La NMQP es una lesión compleja, que puede asociarse a malignidad, pero el diagnóstico preoperatorio de malignidad no puede establecerse con seguridad. El pronóstico depende de un diagnóstico precoz y un tratamiento oportuno.

SUMMARY: Mucinous cystic neoplasm of the pancreas (MCNP) are variable types of tumors, which predominantly affect women (90-95 %), and usually appear incidentally in the 5th to 7th decade of life. They are generally solitary lesions, with no involvement of the main pancreatic duct, rarely symptomatic and are associated with malignancy (10 % to 40 %). The aim of this manuscript was to report a case of giant mucinous cystic neoplasm of the pancreas surgically treated and review the existing evidence regarding its morphological, therapeutic and prognosis characteristics. Patient: A 29-year-old woman with a slightly symptomatic abdominal mass. The diagnosis was verified with ultrasound, computed axial tomography and magnetic nuclear resonance. The patient underwent surgery; an uneventful corporocaudal pancreatectomy with splenic preservation was performed. She was discharged on the fifth day, and has evolved adequately, without postoperative complications. MCNP is a complex lesion, which can be associated with malignancy, but the preoperative diagnosis of malignancy cannot be established with certainty. Its prognosis depends on early diagnosis and timely treatment.

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