Neoplasia quística mucinosa en la vía biliar intrahepática. Presentación de dos casos / Mucinous cystic neoplasm in the intrahepatic bile duct. Presentation of two cases

Introducción. Las neoplasias quísticas mucinosas del hígado son tumores poco frecuentes, equivalen a menos del 5 % de todas las lesiones quísticas hepáticas y se originan generalmente en la vía biliar intrahepática, con poco compromiso extrahepático. En la mayoría de los casos su diagnóstico es incidental dado que es una entidad generalmente asintomática con un curso benigno; sin embargo, hasta en el 30 % pueden ser malignas. En todos los casos se debe hacer una resección quirúrgica completa de la lesión. Caso clínico. Se presentan dos pacientes con diagnóstico de neoplasia quística mucinosa en la vía biliar intrahepática, así como sus manifestaciones clínicas, hallazgos imagenológicos y tratamiento. Discusión. Debido a su baja incidencia, esta patología constituye un reto diagnóstico, que se puede confundir con otro tipo de entidades más comunes. El diagnóstico definitivo se hace de forma histopatológica, pero en todos los casos, ante la sospecha clínica, se recomienda la resección completa. Conclusión. Se presentan dos pacientes con diagnóstico de neoplasias quísticas mucinosas del hígado, una entidad poco frecuente y de difícil diagnóstico

Introduction. Mucinous cystic neoplasms of the liver are rare tumors, accounting for less than 5% of all liver cystic lesions, and generally originate from the intrahepatic bile duct with little extrahepatic involvement. In most cases its diagnosis is incidental since it is a generally asymptomatic entity with a benign course; however, up to 30% can have a malignant course. In all cases, complete surgical resection of the lesion must be performed. Clinical case. Two patients with a diagnosis of mucinous cystic neoplasm in the intrahepatic bile duct are presented, as well as their clinical manifestations, imaging findings, and treatment. Discussion. Due to its low incidence, this pathology constitutes a diagnostic challenge, which can be confused with other types of more common entities. The definitive diagnosis is made histopathologically, but in all cases, given clinical suspicion, complete resection is recommended. Conclusion. Two patients with a diagnosis of mucinous cystic neoplasms of the liver are presented, a rare entity that is difficult to diagnose

Experiencia de downstaging versus criterios de Milán para trasplante hepático por carcinoma hepatocelular en una cohorte prospectiva / Experience with downstaging versus Milan criteria for liver transplantation in patients with hepatocellular carcinoma in a prospective cohort

Introducción. El acceso al trasplante hepático (TH) en pacientes con carcinoma hepatocelular (CHC) se basa en la aplicación de criterios morfológicos rigurosos estipulados desde 1996, co-nocidos como criterios de Milán. Una de las estrategias descritas para expandir estos criterios se conoce como downstaging (reducción del estadiaje tumoral mediante terapias locorregionales). El objetivo de este estudio fue describir el comportamiento postrasplante de pacientes con CHC que ingresaron dentro de los parámetros de Milán, comparado con el de aquellos pacientes llevados a terapia de downstaging en un centro colombiano. Metodología. Se incluyeron pacientes adultos con cirrosis hepática (CH) y CHC que fueron llevados a TH en el Hospital Pablo Tobón Uribe, entre julio de 2012 a septiembre de 2021. Como desenlace principal se definió recurrencia y tiempo de recurrencia de la enfermedad tumoral, muerte por todas las causas y tiempo al fallecimiento. Se evaluaron las características sociodemográficas y clínicas de cada grupo. Se incluyeron scores pronósticos de recurrencia de la enfermedad tumoral. Resultados. Se trasplantaron 68 pacientes con CH y CHC, 50 (73,5 %) eran hombres y la edad promedio fue 59 años; 51 pacientes (75 %) cumplían con los criterios de Milán y 17 (25 %) fueron llevados a terapia de downstaging previo al TH. No hubo diferencias significativas en la supervivencia global y supervivencia libre de trasplante entre los dos grupos evaluados, p=0,479 y p=0,385, respectivamente. Tampoco hubo diferencia significativa en la recurrencia de la enfermedad tumoral entre ambos grupos (p=0,81). En total hubo 7 casos de recurrencia tumoral (10,2 %) y 11 casos de muerte (16,2 %). Conclusiones. No se encontraron diferencias significativas en recurrencia y mortalidad entre los pacientes que cumplían los criterios de Milán y los trasplantados luego de la terapia de downstaging, en un tiempo de se-guimiento de 53 meses hasta el último control posterior al trasplante hepático. Esta sería la primera evaluación prospectiva de un protocolo de downstaging para CHC en Colombia.

Introduction. Access to liver transplantation (LT) in patients with hepatocellular carcinoma (HCC) is based on the application of rigorous morphological criteria stipulated since 1996, known as the Milan criteria. One of the strategies described to expand these criteria is known as downstaging (tu-mor staging reduction through locoregional therapies). The objective of this study was to describe the post-transplant performance of patients with HCC who were admitted within the Milan parameters, compared with those of patients taken to downstaging therapy, in a Colombian center. Methodolo-gy. Adult patients with cirrhosis and HCC that received LT between July 2012 and September 2021 at the Pablo Tobón Uribe Hospital were included. The main outcome was defined as recurrence and time to recurrence of the tumor disease, death from all causes, and time to death. The socio-demographic and clinical characteristics of each group were evaluated. Tumor disease recurrence prognostic scores were included. Results. Sixty-eight patients with cirrhosis and HCC received LT in the time frame, 50 (73.5%) were men and the mean age was 59 years. Fifty-one patients were trans-planted (75%) fulfilling Milan criteria, and 17 (25%) patients received downstaging therapies before LT. There were no significant differences in overall survival and transplant-free survival between the two groups, p=0.479 and p=0.385, respectively. There was also no significant difference in the recurrence of the tumor disease between both groups (p=0.81). In total there were 7 tumoral recurrences (10.2%) and 11 deaths (16.2%). Conclusions. There were no differences in recurrence and survival between patients transplanted fulfilling Milan criteria and those receiving downstaging therapies, following a mean time of 53 months after LT. This is the first prospective evaluation of the downstaging protocol in Colombia.

Performance of pre-transplant criteria in prediction of hepatocellular carcinoma progression and waitlist dropout.

BACKGROUND & AIM: Liver transplantation (LT) selection models for hepatocellular carcinoma (HCC) have not been proposed to predict waitlist dropout because of tumour progression. The aim of this study was to compare the alpha-foetoprotein (AFP) model and other pre-LT models in their prediction of HCC dropout. METHODS: A multicentre cohort study was conducted in 20 Latin American transplant centres, including 994 listed patients for LT with HCC from 2012 to 2018. Longitudinal tumour characteristics, and patterns of progression were recorded at time of listing, after treatments and at last follow-up over the waitlist period. Competing risk regression models were performed, and model's discrimination was compared estimating Harrell's adapted c-statistics. RESULTS: HCC dropout rate was significantly higher in patients beyond (24% [95% CI 16-28]) compared to those within Milan criteria (8% [95% IC 5%-12%]; p < .0001), with a SHR of 3.01 [95% CI 2.03-4.47]), adjusted for waiting list time and bridging therapies (c-index 0.63 [95% CI 0.57; 0.69). HCC dropout rates were higher in patients with AFP scores >2 (adjusted SHR of 3.17 [CI 2.13-4.71]), c-index of 0.71 (95% CI 0.65-0.77; p = .09 vs Milan). Similar discrimination power for HCC dropout was observed between the AFP score and the Metroticket 2.0 model. In patients within Milan, an AFP score >2 points discriminated two populations with a higher risk of HCC dropout (SHR 1.68 [95% CI 1.08-2.61]). CONCLUSIONS: Pre-transplant selection models similarly predicted HCC dropout. However, the AFP model can discriminate a higher risk of dropout among patients within Milan criteria.

Liver transplantation for hepatocellular carcinoma: impact of expansion criteria in a multicenter cohort study from a high waitlist mortality region.

This study aimed to compare liver transplantation (LT) outcomes and evaluate the potential rise in numbers of LT candidates with hepatocellular carcinoma (HCC) of different allocation policies in a high waitlist mortality region. Three policies were applied in two Latin American cohorts (1085 HCC transplanted patients and 917 listed patients for HCC): (i) Milan criteria with expansion according to UCSF downstaging (UCSF-DS), (ii) the AFP score, and (iii) restrictive policy or Double Eligibility Criteria (DEC; within Milan + AFP score ≤2). Increase in HCC patient numbers was evaluated in an Argentinian prospective validation set (INCUCAI; NCT03775863). Expansion criteria in policy A showed that UCSF-DS [28.4% (CI 12.8-56.2)] or "all-comers" [32.9% (CI 11.9-71.3)] had higher 5-year recurrence rates compared to Milan, with 10.9% increase in HCC patients for LT. The policy B showed lower recurrence rates for AFP scores ≤2 points, even expanding beyond Milan criteria, with a 3.3% increase. Patients within DEC had lower 5-year recurrence rates compared with those beyond DEC [13.3% (CI 10.1-17.3) vs 24.2% (CI 17.4-33.1; P = 0.0006], without significant HCC expansion. In conclusion, although the application of a stricter policy may optimize the selection process, this restrictive policy may lead to ethical concerns in organ allocation (NCT03775863).

Recurrence of hepatocellular carcinoma after liver transplantation: Prognostic and predictive factors of survival in a Latin American cohort.

BACKGROUND & AIM: Recurrence of hepatocellular carcinoma (HCC) after liver transplantation (LT) has a poor prognosis, and the adjusted effect of different treatments on post-recurrence survival (PRS) has not been well defined. This study aims to evaluate prognostic and predictive variables associated with PRS. METHODS: This Latin American multicenter retrospective cohort study included HCC patients who underwent LT between the years 2005-2018. We evaluated the effect of baseline characteristics at time of HCC recurrence diagnosis and PRS (Cox regression analysis). Early recurrences were those occurring within 12 months of LT. To evaluate the adjusted treatment effect for HCC recurrence, a propensity score matching analysis was performed to assess the probability of having received any specific treatment for recurrence. RESULTS: From a total of 1085 transplanted HCC patients, the cumulative incidence of recurrence was 16.6% (CI 13.5-20.3), with median time to recurrence of 13.0 months (IQR 6.0-26.0). Factors independently associated with PRS were early recurrence (47.6%), treatment with sorafenib and surgery/trans-arterial chemoembolization (TACE). Patients who underwent any treatment presented "early recurrences" less frequently, and more extrahepatic metastasis. This unbalanced distribution was included in the propensity score matching, with correct calibration and discrimination (receiving operator curve of 0.81 [CI 0.72;0.88]). After matching, the adjusted effect on PRS for any treatment was HR of 0.2 (0.10;0.33); P < .0001, for sorafenib therapy HR of 0.4 (0.27;0.77); P = .003, and for surgery/TACE HR of 0.4 (0.18;0.78); P = .009. CONCLUSION: Although early recurrence was associated with worse outcome, even in this population, systemic or locoregional treatments were associated with better PRS.

Colangitis esclerosante primaria. Una revisión narrativa / Primary Sclerosing Cholangitis. A narrative review

RESUMEN La colangitis esclerosante primaria (CEP) es una patología hepática crónica y rara que se caracteriza por la inflamación y fibrosis de los conductos biliares, cuya evolución puede llevar a la cirrosis, hipertensión portal y enfermedad hepática en etapa terminal. Su etiología es desconocida, pero se ha relacionado con factores genéticos y autoinflamatorios. Además, tiene una relación muy estrecha con la enfermedad inflamatoria intestinal (EII). Su presentación clínica es muy inespecífica, sus principales síntomas son el prurito y la fatiga. La prueba estándar para su diagnóstico es la colangiopancreatografía por resonancia magnética (CPRM), donde se observa un aspecto anular ocasionado por estenosis multifocales cortas con segmentos alternos normales o dilatados. Actualmente, no existe ningún tratamiento farmacológico que logre prolongar la supervivencia sin un trasplante de hígado en la CEP. Sólo se puede hacer tratamiento sintomático, especialmente del prurito. El único manejo curativo con el que se cuenta hoy en día es el trasplante hepático, aunque existe un riesgo de recurrencia de la enfermedad. Es muy importante la vigilancia de los trastornos inflamatorios intestinales, la malignidad y la enfermedad metabólica ósea en estos pacientes. Se ha visto que algunos factores, como el diagnóstico temprano, son de buen pronóstico para la enfermedad.

SUMMARY Primary sclerosing cholangitis (PSC) is a rare, chronic liver pathology characterized by inflammation and fibrosis of the bile ducts, whose evolution can lead to cirrhosis, portal hypertension and end-stage liver disease. Its etiology is unknown, but it has been related to genetic and autoinflammatory factors. In addition, it has a very close relationship with inflammatory bowel disease. Its clinical presentation is nonspecific, the main symptoms are pruritus and fatigue. The standard test for diagnosis is magnetic resonance cholangiopancreatography (MRCP), where an annular aspect is observed caused by short multifocal stenoses with alternating normal or dilated segments. Currently, there is no pharmacological treatment that can prolong the survival without liver transplantation in PSC. Symptomatic treatment is warranted, especially for pruritus. The only curative treatment that is currently available is liver transplantation, although there is a risk of recurrence of the disease. The monitoring of intestinal inflammatory disorders (IID), malignancy and metabolic bone disease in these patients is very important. It has been seen that some factors, such as early diagnosis, are good prognosis for the disease.

Direct-Acting Antivirals and Hepatocellular Carcinoma: No Evidence of Higher Wait-List Progression or Posttransplant Recurrence.

The association between direct-acting antivirals (DAAs) and hepatocellular carcinoma (HCC) wait-list progression or its recurrence following liver transplantation (LT) remains uncertain. We evaluated the impact of DAAs on HCC wait-list progression and post-LT recurrence. This Latin American multicenter retrospective cohort study included HCC patients listed for LT between 2012 and 2018. Patients were grouped according to etiology of liver disease: hepatitis C virus (HCV) negative, HCV+ never treated with DAAs, and HCV+ treated with DAAs either before or after transplantation. Multivariate competing risks models were conducted for both HCC wait-list progression adjusted by a propensity score matching (pre-LT DAA effect) and for post-LT HCC recurrence (pre- or post-LT DAA effect). From 994 included patients, 50.6% were HCV-, 32.9% were HCV+ never treated with DAAs, and 16.5% were HCV+ treated with DAAs either before (n = 66) or after LT (n = 98). Patients treated with DAAs before LT presented similar cumulative incidence of wait-list tumor progression when compared with those patients who were HCV+ without DAAs (26.2% versus 26.9%; P = 0.47) and a similar HCC-related dropout rate (12.1% [95% CI, 0.4%-8.1%] versus 12.9% [95% CI, 3.8%-27.2%]), adjusted for baseline tumor burden, alpha-fetoprotein values, HCC diagnosis after listing, bridging therapies, and by the probability of having received or not received DAAs through propensity score matching (subhazard ratio [SHR], 0.9; 95% CI, 0.6-1.6; P = 0.95). A lower incidence of posttransplant HCC recurrence among HCV+ patients who were treated with pre- or post-LT DAAs was observed (SHR, 0.7%; 95% CI, 0.2%-4.0%). However, this effect was confounded by the time to DAA initiation after LT. In conclusion, in this multicenter cohort, HCV treatment with DAAs did not appear to be associated with an increased wait-list tumor progression and HCC recurrence after LT.

Descripción de la experiencia en pacientes diagnosticados con insulinoma. Estudio multicéntrico en Medellín, Colombia / Description of the experience with patients with insulinoma diagnosis. Multicentric study in Medellín, Colombia

RESUMEN Objetivo: describir la experiencia de los pacientes con insulinoma, diagnosticados y tratados entre los años 2002 y 2017 en tres hospitales de la ciudad de Medellín, Colombia. Métodos: estudio descriptivo y retrospectivo de pacientes con criterios bioquímicos para hipoglucemia hiperinsulínica y confirmación histopatológica de insulinoma. Resultados: se estudiaron 19 casos, 10 eran mujeres (52,6 %), la edad media al diagnóstico fue 43 años (D.E 15,5). Hubo cuatro casos de insulinoma multifocal (21,1 %), tres asociados con NEM-1 (15,8 %) y dos malignos (10,6 %). Todos presentaron hipoglucemia en ayunas y 63,2 % posprandial. En la prueba de ayuno, el nadir de glucemia sucedió antes de 48 horas en todos los casos, en promedio 9 horas (D.E 8,0). El diagnóstico bioquímico fue realizado con hipoglucemia e insulina elevada en todos los casos, aunque el péptido C fue reportado en nueve pacientes (47,3 %) y las sulfonilureas en dos (11,1 %). La localización preoperatoria se hizo por imágenes en 12 individuos (68,5 %) y las pruebas invasivas fueron necesarias en seis (31,5 %). Las pruebas diagnósticas fueron positivas en un 83 % para resonancia, 50 % para ecografía endoscópica y prueba de estímulo intraarterial con calcio y 100 % para ecografía intraoperatoria. La cirugía se realizó en 18 casos (94,7 %). La mortalidad (15,8 %) fue derivada de complicaciones en el posoperatorio temprano; la curación se logró en todos los casos. Conclusiones: el insulinoma en nuestro medio tiene características demográficas y clínicas similares a otras series. Existen limitaciones locales para el acceso a los estudios bioquímicos y en el rendimiento diagnóstico de las pruebas de localización.

SUMMARY Objective: The objective of the study was to describe the characteristics of patients with insulinoma in three hospitals in Medellín, Colombia, between 2002 and 2017. Methods: A retrospective analysis of patients with hyperinsulinemic hypoglycemia and histologic confirmation of insulinoma was conducted. Results: A total of 19 cases were identified. Ten women (52.6 %) and 9 males (47.4 %). The mean age at diagnosis was 43 years (S.D: 15.5). Four cases had multifocal insulinoma (21.1%), 3 cases were secondary to multiple endocrine neoplasia type 1 (15.8 %), and 2 of them were malignant (10.6 %). All patients presented fasting hypo-glycemia, and 63.2% presented post-prandial hypoglycemia. Glucose nadir in the fasting test occurred in the first 48 hours in all cases, with a mean time to hypoglycemia of 9 hours (S.D 8.0). The biochemical diagnosis was done with increased insulin in the presence of hypoglycemia. C-peptide was done in 9 patients (47.3 %), and sulfonylureas in 2 cases (11.1 %). Preoperative localization was done by imaging in 12 cases (68.5 %), and invasive tests were required in 6 cases (31.5 %). Localization tests were positive as follows: magnetic resonance imaging in 83%, endoscopic ultrasound in 50%, selective intra-arterial calcium injection in 50 %, and intraoperative ultrasound in 100%. Eighteen patients (94. 7%) underwent surgery. Mortality (15.8 %) was secondary to early post-operative complications. Conclusions: The characteristics of patients with insulinoma in Medellín are similar to other series. However, there are important local limitations for proper biochemical testing and imaging localization. This is the largest study in our country.

Factores asociados con la supervivencia al año de pacientes operados con cáncer ampular o de páncreas / Factors associated with survival a one year of operated patients with cancer ampullary or pancreas

RESUMEN Objetivo Establecer los factores asociados a la supervivencia a un año de los pacientes operados con tumores de páncreas y ampulares. Materiales y Métodos Estudio descriptivo retrospectivo de una cohorte de pacientes con aplicación de análisis de supervivencia, basado en información secundaria. Se estimaron frecuencias absolutas y relativas para las variables cualitativas; y medidas de tendencia central y dispersión para las variables cuantitativas. La supervivencia global se estableció por medio del método Kaplan-Meier, en el análisis bivariado se utilizó la prueba Log-Rank y para establecer los factores asociados a la supervivencia, se efectuó un modelo de riesgos proporcionales de Cox. Resultados La supervivencia a un año de los 70 pacientes operados fue del 62,5 %. Los factores asociados a la supervivencia ajustada fueron la sepsis, sangrado posoperatorio, antígeno carcinoembrionario, trasfusión de glóbulos rojos, sangrado intraoperatorio, cirugías previas de abdomen y edad. Conclusiones El conocimiento que se obtuvo de la supervivencia a un año de los pacientes operados con cáncer de páncreas y ampular, permitirá crear estrategias encaminadas a la intervención de los factores asociados, y generar información que pueda conllevar a mejorar el pronóstico de los pacientes.(AU)

ABSTRACT Objective Determine the factors associated with survival a one year of patients operated with ampullary and pancreatic tumors. Materials and Methods A retrospective descriptive study of a cohort of patients with application of survival analysis based on secondary data. Absolute and relative frequencies for qualitative variables were estimated; and measures of central tendency and dispersion for quantitative variables. Overall survival was estimate by the Kaplan-Meier method, in the bivariate analysis was used the Log-Rank test and to establish the factors associated with survival was made a model Proportional Hazards of Cox. Results The survival a one year of 70 operated patients was 62,5 %. Factors associated with survival were adjusted sepsis, postoperative bleeding, carcinoembryonic antigen, red cell transfusion, intraoperative bleeding, previous abdominal surgeries and age. Conclusions The knowledge obtained from the survival a one year of patients operated with pancreatic and ampullary cancer, will create intervention strategies associated factors, generate more information that may lead to improve prognosis and reduce mortality.(AU)

Liver transplantation for hepatocellular carcinoma: evaluation of the alpha-fetoprotein model in a multicenter cohort from Latin America.

BACKGROUND & AIMS: The French alpha-fetoprotein (AFP) model has recently shown superior results compared to Milan criteria (MC) for prediction of hepatocellular carcinoma (HCC) recurrence after liver transplantation (LT) in European populations. The aim of this study was to explore the predictive capacity of the AFP model for HCC recurrence in a Latin-American cohort. METHODS: Three hundred twenty-seven patients with HCC were included from a total of 2018 patients transplanted at 15 centres. Serum AFP and imaging data were both recorded at listing. Predictability was assessed by the Net Reclassification Improvement (NRI) method. RESULTS: Overall, 82 and 79% of the patients were within MC and the AFP model respectively. NRI showed a superior predictability of the AFP model against MC. Patients with an AFP score >2 points had higher risk of recurrence at 5 years Hazard Ratio (HR) of 3.15 (P = 0.0001) and lower patient survival (HR = 1.51; P = 0.03). Among patients exceeding MC, a score ≤2 points identified a subgroup of patients with lower recurrence (5% vs 42%; P = 0.013) and higher survival rates (84% vs 45%; P = 0.038). In cases treated with bridging procedures, following restaging, a score >2 points identified a higher recurrence (HR 2.2, P = 0.12) and lower survival rate (HR 2.25, P = 0.03). A comparative analysis between HBV and non-HBV patients showed that the AFP model performed better in non-HBV patients. CONCLUSIONS: The AFP model could be useful in Latin-American countries to better select patients for LT in subgroups presenting with extended criteria. However, particular attention should be focused on patients with HBV.

[Factors associated with survival a one year of operated patients with cancer ampullary or pancreas]. / Factores asociados con la supervivencia al año de pacientes operados con cáncer ampular o de páncreas.

OBJECTIVE: Determine the factors associated with survival a one year of patients operated with ampullary and pancreatic tumors. MATERIALS AND METHODS: A retrospective descriptive study of a cohort of patients with application of survival analysis based on secondary data. Absolute and relative frequencies for qualitative variables were estimated; and measures of central tendency and dispersion for quantitative variables. Overall survival was estimate by the Kaplan-Meier method, in the bivariate analysis was used the Log-Rank test and to establish the factors associated with survival was made a model Proportional Hazards of Cox. RESULTS: The survival a one year of 70 operated patients was 62,5 %. Factors associated with survival were adjusted sepsis, postoperative bleeding, carcinoembryonic antigen, red cell transfusion, intraoperative bleeding, previous abdominal surgeries and age. CONCLUSIONS: The knowledge obtained from the survival a one year of patients operated with pancreatic and ampullary cancer, will create intervention strategies associated factors, generate more information that may lead to improve prognosis and reduce mortality.

OBJETIVO: Establecer los factores asociados a la supervivencia a un año de los pacientes operados con tumores de páncreas y ampulares. MATERIALES Y MÉTODOS: Estudio descriptivo retrospectivo de una cohorte de pacientes con aplicación de análisis de supervivencia, basado en información secundaria. Se estimaron frecuencias absolutas y relativas para las variables cualitativas; y medidas de tendencia central y dispersión para las variables cuantitativas. La supervivencia global se estableció por medio del método Kaplan-Meier, en el análisis bivariado se utilizó la prueba Log-Rank y para establecer los factores asociados a la supervivencia, se efectuó un modelo de riesgos proporcionales de Cox. RESULTADOS: La supervivencia a un año de los 70 pacientes operados fue del 62,5 %. Los factores asociados a la supervivencia ajustada fueron la sepsis, sangrado posoperatorio, antígeno carcinoembrionario, trasfusión de glóbulos rojos, sangrado intraoperatorio, cirugías previas de abdomen y edad. CONCLUSIONES: El conocimiento que se obtuvo de la supervivencia a un año de los pacientes operados con cáncer de páncreas y ampular, permitirá crear estrategias encaminadas a la intervención de los factores asociados, y generar información que pueda conllevar a mejorar el pronóstico de los pacientes.

Hepatocarcinoma fibrolamelar un tumor de adultos jóvenes poco frecuente: Reporte de un caso / Case Report of Fibrolamellar Hepatocarcinoma, a Rare Tumor of Young Adults

El hepatocarcinoma fibrolamelar (CHC-FL) es una rara variante del carcinoma hepatocelular (CHC), se presenta con mayor frecuencia en adultos jóvenes, sin distinción de sexo, se desarrolla en su manera más característica sobre un hígado previamente sano. La manifestación clásica está dada por la triada de masa palpable en hipocondrio derecho, dolor y pérdida de peso. Ante la ausencia de historia de enfermedad hepática previa, es usual que se encuentre como una neoplasia avanzada de gran tamaño, la cual tiene hallazgos característicos en la tomografía o en la RM, que permiten en un alto porcentaje de casos hacer el diagnóstico solo con las imágenes. En este tipo de neoplasias, el mejor tratamiento es la resección quirúrgica, que proporciona una sobrevida a 5 años de 58-82%, pero con unas tasas de recaídas muy elevadas, que puede variar según las series entre 33-100% de los casos. Presentamos el caso de un paciente joven, sin historia de enfermedad hepática, en el que se diagnosticó un CHC-FL mediante el uso de resonancia magnética con medio de contraste órgano-específico y que es tratado con resección quirúrgica radical.

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma (HCC) which occurs most often in young adults without regard to sex. It develops in its characteristic way on a previously healthy liver. The classical presentation is a palpable mass in the right upper quadrant, pain, and weight loss. In the absence of a prior history of liver disease, it is usual to find a large advanced neoplasia which has characteristic findings in a CT scan or MRI. A high percentage of cases can be diagnosed solely with images. The best treatment for this type of tumor is surgical resection which provides a 5-year survival rate of 58% to 82% but with very high rates of relapse. In published studies rates of relapse vary between 33% and 100%. We report the case of a young patient with no history of liver disease in which fibrolamellar hepatocellular carcinoma was diagnosed using contrast organ-specific MRI. The tumor was treated with radical surgical resection.

Acute pancreatitis: Reflections through the history of the Atlanta Consensus / Pancreatitis aguda: reflexiones a través de la historia del Consenso de Atlanta / Pancreatites aguda: reflexões através da história do Consenso de Atlanta

Acute pancreatitis is an inflammatory process with systemic and local repercussions. Most cases are mild with a low mortality rate, but 20% of patients have severe pancreatitis, with a mortality rate up to 30%. Throughout the years, the medical community has tried to reach a consensus about this disease to better understand, classify and treat it. The most important consensus is known as the Atlanta Consensus of 1992, which has been in use for many years. However, it has recently been the subject of various proposals for change and updating, which are discussed in this review.

La pancreatitis aguda es un proceso inflamatorio con repercusiones sistémicas y locales; la mayoría de los casos son leves con baja tasa de mortalidad, pero el 20% de los pacientes sufren pancreatitis grave cuya tasa de mortalidad puede llegar a ser hasta de un 30%. A lo largo de los años se ha intentado llegar a consensos acerca de esta enfermedad con el fin de orientar a la comunidad médica hacia su mejor entendimiento, clasificación y tratamiento. El más importante de estos ha sido conocido como el Consenso de Atlanta de 1992, vigente por muchos años, pero que está siendo objeto de diferentes propuestas de modificación y actualización, que se discuten en este artículo de revisión.

A pancreatites aguda é um processo inflamatório com repercussões sistémicas e locais; a maioria dos casos são leves com baixa taxa de mortalidade, mas 20% dos pacientes sofrem pancreatites grave cuja taxa de mortalidade pode chegar a ser até de um 30%. Ao longo dos anos se tentou chegar a consensos a respeito desta doença com o fim de orientar à comunidade médica para seu melhor entendimento, classificação e tratamento. O mais importante destes foi conhecido como o Consenso de Atlanta de 1992, vigente por muitos anos, mas que está sendo objeto de diferentes propostas de modificação e atualização, que se discutem neste artigo de revisão.

Síndrome de Budd Chiari: Reporte de tres casos y revisión de la literatura / Budd Chiari Syndrome: Three Case Reports and a Literature Review

Se describen los casos de tres pacientes de sexo femenino a quienes se les hizo diagnóstico de síndrome de Budd Chiari. En una paciente la presentación del síndrome fue subaguda, pudiendo ser manejada exitosamente con la colocación de TIPS. Otra con mutación del factor V Leyden asociada desarrolló disfunción hepática progresiva y requirió de trasplante hepático. En dos de los tres casos se identificó una enfermedad hematológica como trastorno de base, y en uno el uso de anticonceptivos orales como factor de riesgo. Las tres pacientes fueron sometidas a terapia anticoagulante y el manejo quirúrgico fue definido de acuerdo a su condición clínica. Sin embargo, en un caso la presentación fue aguda con falla hepática y muerte.

This article describes the cases of three female patients who were diagnosed with Budd-Chiari syndrome. One patient was subacute and could be successfully managed by placement of a transjugular intrahepatic portosystemic stent (TIPS). Another patient who had the Factor V Leiden mutation developed associated progressive liver dysfunction and required liver transplantation. A hematologic disease was identified as the underlying disorder in two of the three cases. For one patient, the use of oral contraceptives was a risk factor. Since all three patients were undergoing anticoagulant therapy, surgical management was determined according to each patient’s clinical condition. Nevertheless, the one patient who that presented acute hepatic failure did not survive.

Manejo endoscópico de las complicaciones biliares después del trasplante ortotópico de hígado / Endoscopic management of biliary complications following orthotopic liver transplantation

Antecedentes: Las complicaciones derivadas de la reconstrucción de la vía biliar en el trasplante hepático (TOH) son frecuentes y son causa importante de morbimortalidad. Actualmente, la colangiopancreatografía endoscópica retrógrada (CPRE), por su utilidad diagnóstica y terapéutica, su menor invasividad y su baja morbilidad es una alternativa atrayente en el manejo de las complicaciones biliares. Se pretende evaluar los resultados del manejo endoscópico de pacientes con complicaciones biliares después del trasplante hepático, determinar factores de riesgo y el impacto en la sobrevida. Materiales: Se revisaron las historias clínicas de los pacientes con complicaciones biliares después de trasplante hepático ingresados al programa del Hospital Pablo Tobón Uribe, fueran o no trasplantados en este centro, entre enero del 2002 y diciembre del 2010. Resultados: Se hicieron 148 CPRE en 50 pacientes (30 hombres y 20 mujeres). La edad promedio fue 47 años (5 a 71 años). Tiempo promedio de seguimiento 44 meses. Los hallazgos fueron: estenosis biliares 42 (33 anastomóticas y 9 no anastomóticas), fistulas biliares 4, defectos de llenado (cálculos o moldes biliares) en 3 y disfunción del Oddi en 1. Las estenosis fueron manejadas con prótesis plásticas (30 pacientes) o metálicas (12 pacientes), estas últimas desde el 2008. El 94% de las estenosis no isquémicas mejoraron con stent biliar, mientras menos del 50% de las estenosis isquémicas mejoraron. Conclusiones: La colangiografía endoscópica es segura y efectiva para el diagnóstico y manejo de las complicaciones biliares secundarias al trasplante hepático. Solo la edad presentó una relación directa con la posibilidad de complicaciones biliares. La sobrevida del grupo con complicaciones biliares no difiere de los pacientes trasplantados que cursaron sin complicaciones biliares.

ackground. Complications of bile duct reconstruction following liver transplantation are common and are an important cause of morbidity and mortality. Currently, endoscopic retrograde cholangiopancreatography (ERCP) is an attractive alternative for management of biliary complications. It has high diagnostic and therapeutic utility, is less invasive than other techniques and has low morbidity. The aims of this study are to evaluate the results of endoscopic management of patients with biliary complications following liver transplantation, and to then determine risk factors and their impacts on patient survival. Materials. We reviewed the medical records of patients with biliary complications following liver transplantation who were admitted to the Hospital Pablo Tobón Uribe between January 2002 and December 2010. Cases were reviewed whether or not patients had undergone transplantation at this center. Results. 148 ERCPs were performed on 50 patients (30 men and 20 women). Average patient age was 47 years (5 to 71 years old). Average follow up time was 44 months. 42 biliary strictures in (33 anastomotic and 9 non anastomotic), 4 fistulas, 3 filling defects (stones or bile casts) and 1 papillary stenosis (Oddi dysfunction) were found. Strictures were managed with plastic (30 patients) prostheses or, since 2008, with metal prostheses (12 patients). 94% of cases of non-ischemic stenosis improved with biliary stents. Conclusions. Endoscopic cholangiography is safe and effective for the diagnosis and management of biliary complications following hepatic transplantation. The only factor which showed a direct relationship with the possibility of biliary complications was age. The survival of the group with biliary complications did not differ from transplant patients without biliary complications.

Radioterapia: papel actual en el cáncer de páncreas / Radiotherapy: its present role in pancreatic cancer

El cáncer de páncreas es una enfermedad de mal pronóstico; la tasa de supervivencia de los pacientes a cinco años es de 5%-15% luego del tratamiento radical. Su evolución silenciosa pero agresiva, asociada a la sintomatología inespecífica, retarda el diagnóstico, razón por la cual solo entre 15% y 20% de las lesiones se pueden resecar en el momento del diagnóstico; ello no es posible en el caso de las restantes, bien sea por cáncer localmente avanzado (30%) o por metástasis (50%); por lo tanto, se requiere otro enfoque terapéutico para mejorar la calidad de vida de estos pacientes y si es posible prolongarles la supervivencia. La quimioterapia y la radioterapia como tratamientos únicos o en combinación no han logrado resultados satisfactorios. La investigación continúa con el fin de hallar el protocolo con el que se logre mejorar la calidad de vida y un aumento significativo en la supervivencia.

Prognosis of pancreatic cancer is poor with a five-year survival rate of 5%-15% after radical treatment. Its silent but aggressive evolution and nonspecific manifestations delay diagnosis so that only 15%-20% of lesions can be resected when diagnosis is established. The remaining ones are not resectable because they have advanced locally or because metastases are already present. Therefore, different therapeutic approaches are required in order to improve the quality of life and, if possible, to lengthen survival. Chemotherapy and radiotherapy as sole treatments, or the combination of both, have not achieved satisfactory results. Presently, investigation is very active concerning therapy of locally advanced pancreatic cancer in order to find the best therapeutic protocol in terms of quality of life and survival.

Complicaciones de la vía biliar después de trasplante ortotópico de hígado: experiencia en el Hospital Pablo Tobón Uribe - Medellín / Biliary duct complications after orthotopic liver transplants: experience from the Pablo Tobon Uribe Hospital – Medellín

Introducción: Las complicaciones del tracto biliar posteriores al trasplante ortotópico de hígado (TOH) son uno de los problemas más importantes, ya que pueden ocasionar disfunción del injerto y muerte. Objetivo: Evaluar la incidencia de las complicaciones biliares posteriores al TOH en la serie de nuestra institución. Metodología: De forma retrospectiva se evaluaron las historias clínicas de 254 pacientes a quienes se les realizó TOH con donante cadavérico y se identificaron aquellos pacientes que presentaron complicaciones biliares. Resultados: Se documentaron 25 pacientes con complicaciones biliares, para una frecuencia de 9,84%; 21 casos (84%) de estenosis biliar, de estos, 16 (64%) estenosis anastomóticas y 5 (20%) estenosis no anastomóticas. Hubo 7 casos (28%) de fugas biliares. Otras complicaciones fueron menos frecuentes. La sobrevida es de 88% con una media de seguimiento de 37,8 meses. Conclusión: Las complicaciones biliares pos-TOH continúan siendo frecuentes, sin embargo, el desarrollo en las técnicas de intervención endoscópica y percutánea han mejorado el pronóstico general de estos pacientes.

Introduction: Biliary duct complications following an orthotopic liver transplant (OLT) are some of the most important problems since they can cause graft dysfunction and death. Objective: Evaluate the incidence level of biliary complications following OLTs in our institution. Methodology: The clinical charts of 254 patients who had had OLTs with deceased liver donors were retrospectively evaluated to identify those who developed biliary complications. Results: 25 patients with biliary complications, or a frequency of 9.84%, were documented. 21 cases (84%) had biliary strictures. Of these 16 (64%) had anastomotic strictures and 5 (20%) had non-anastomotic strictures. There were 7 cases (28%) of biliary leakage. Other complications were less frequent. The survival rate is 88% with mean follow up time of 37.8 months. Conclusion: Post OLT biliary complications are still frequent; however, the development of endoscopic and percutaneous interventions has improved the general prognosis of these patients.

Reporte de dos casos de síndrome de sobreposición entre hepatitis autoinmune y cirrosis biliar primaria / Report of two overlap syndrome cases with autoimmune hepatitis and primary biliary cirrhosis

Se presentan los casos de dos pacientes mujeres con características clínicas, serológicas, inmunológicas, e histológicas mixtas entre hepatitis autoinmune y cirrosis biliar primaria; y para quienes el trasplante ortotópico de hígado se presentó como la mejor alternativa terapéutica dado el avanzado deterioro de su enfermedad.

We present the cases of two female patients with clinical, serological, immunological, and histological characteristics which mixed symptoms of autoimmune hepatitis and primary biliary cirrhosis. Orthotopic liver transplantation was chosen as the best therapeutic alternative for both patients given the advanced deterioration of their disease.

Alfa-fetoproteína y gamma-glutamiltranspeptidasa en pacientes con cirrosis hepática y carcinoma hepatocelular / Alpha-fetoprotein and gamma-glutamyltransferase in patients with liver cirrhosis and hepatocellular carcinoma

Introducción: El carcinoma hepatocelular (CHC) es una complicación de la cirrosis hepática (CH). El objetivo principal del estudio fue describir los niveles de los marcadores serológicos alfa-fetoproteína (AFP) y gamma-glutamiltranspeptidasa (GGT) en pacientes con CH y/o CHC. Métodos: Estudio de corte transversal con 99 pacientes con diagnóstico de CH y/o CHC. Resultados: Un total de 66 (66,7%) pacientes presentaban un diagnóstico de CH, 23 (23,2%) CHC asociado a CH y 10 (10,1%) CHC aislado. Los valores de AFP fueron mayores en individuos con CHC asociado a CH comparados con aquellos que solo tenían CH (20 y 2,93 ng/mL, p<0.05); los niveles de la GGT fueron también mayores en pacientes con CHC asociado a CH (208 y 109 UI/L, p<0,05). Ningún paciente con CHC tuvo en forma simultánea valores normales de AFP y GGT. Conclusiones: En pacientes con CHC asociado a CH los niveles de AFP y GGT son significativamente más altos que individuos con CH aislada.

Introduction: Hepatocellular carcinoma (HCC) is one of the complications associated with liver cirrhosis (LC). The main objective of the present study was to describe the levels of the serological markers alpha-fetoprotein (AFP) and gamma-glutamyltranspeptidase (GGT) in patients with LC and/or HCC. Methods: Cross sectional study that included 99 patients with a diagnosis of LC and/or HCC. Results: 66 (66.7%) patients had a diagnosis of LC, 23 (23.2%) had LC alongside with HCC and 10 (10.1%) had HCC without LC. AFP levels were higher in individuals with HCC associated with LC when compared with those with LC only (20 and 2.93 ng/mL, p <0.05), the levels of GGT were also higher in patients with HCC associated with LC (208 and 109 IU/L, p <0.05). Not a single patient with HCC had normal levels of AFP and GGT simultaneously. Conclusions: In patients with HCC associated with LC levels of AFP and GGT were significantly higher than those found in individuals with LC only.

Resultados de duodenopancreatectomía cefálica en el Hospital Pablo Tobón Uribe de Medellín, en un período de tres años / Results of cephalic pancreatoduodenectomy over a three year period at Hospital Pablo Tobón Uribe, Medellín, Colombia

Objetivo: evaluar los datos epidemiológicos, la patología y los resultados de una serie de pacientes sometidos a duodenopancreatectomía cefálica en un centro de referencia de cirugía hepatobiliar, pancreática y de trasplante hepático en la ciudad de Medellín, Colombia, en un período de tres años.Métodos: se recolectaron los datos de manera prospectiva de la base de datos de la Unidad de Cirugía Hepatobiliar y Pancreática de dicha institución. Resultados: entre enero de 2004 y diciembre de 2006, se llevaron a duodenopancreatectomía cefálica con intención curativa 19 pacientes, con una distribución de 31,5 porciento hombres y 68,5 porciento mujeres. La indicación principal fue carcinoma de cabeza de páncreas en el 36,8 porciento, seguido por carcinoma ampular en el 21 porciento y tumor de vías biliares y de duodeno en el 10,5 porciento. La morbilidad fue de 47,3 porciento y la mortalidad de 15 porciento. La supervivencia actuarial entre todos fue de 57,8 porciento, la supervivencia a un año fue de 68,5 porciento.Discusión: la duodenopancreatectomía cefálica es una cirugía de gran envergadura pero que, hecha de manera regular en centros de referencia, puede lograr tasas de supervivencia y de morbimortalidad similares a las reportadas en la literatura mundial.