Malformación linfática quística gigante del hígado: dos casos pediátricos con diferente abordaje quirúrgico / Giant cystic lymphatic malformation of the liver: two pediatric cases with different surgical approach

Introducción. Las malformaciones linfáticas quísticas, también llamadas linfangiomas quísticos, aparecen muy raramente de forma aislada en el hígado. Casos clínicos. Se presentan dos pacientes femeninas de edad preescolar con marcada hepatomegalia, dependiente de lesiones quísticas multitabicadas, secundarias a malformación linfática quística gigante del hígado, que fueron tratadas en el Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Resultados. En ambos casos el diagnóstico se apoyó en los estudios de imágenes, la laparoscopia y el análisis histopatológico. En un caso el tratamiento fue la hepatectomía derecha, mientras que en el otro se empleó la escleroterapia, ambas con evolución favorable. Conclusión. A pesar de su rareza, este diagnóstico no debe obviarse ante un paciente pediátrico con lesiones hepáticas quísticas. El tratamiento de elección es la resección quirúrgica, pero su indicación y envergadura debe valorarse de forma individualizada

Introduction. Cystic lymphatic malformations, also called cystic lymphangiomas, are very rarely found in the liver. Clinical cases. Two pediatric female preschool-age patients. presented with hepatomegaly due to multi-septated cystic lesions of the liver, who received treatment at Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Results. We report two pediatric cases with giant cystic lymphatic malformation of the liver. In both cases, the diagnosis were based on imaging, laparoscopy and pathology. In one case the treatment was right hepatectomy, whereas in the other, sclerotherapy was performed, both with a favorable outcome. Conclusion. Despite its rarity, this diagnosis should be considered in pediatric patients with hepatic cystic lesions. The recommended treatment is surgical resection, but its indication and extent should be assessed individually for each patient.

Perihilar cholangiocarcinoma resection: Is it beneficial for survival in elderly patients?

INTRODUCTION: As the population ages, surgeons are growing frequently faced with hard choices among a vast array of treatment options for the elderly. This study was to investigate safety and efficacy of resection in elderly patients with perihilar cholangiocarcinoma (PHCC). PATIENTS AND METHODS: Literature reading and meta-analysis unveiled that elderly PHCC patients held a higher risk of death within 90 days after hepatectomy relative to younger patients, but their 5-year overall survival and disease-free survival were comparable. Among PHCC patients who underwent hepatectomy, the proportion of elderly patients with tumor classification Bismuth I-II and tumor stage pStage 1-3 was significantly higher than that of younger patients. RESULTS: Curative resection R0 was more common in elderly patients than younger patients, but the difference was not statistically significant. Because of more comorbidities and less physiological reserve of elderly patients, they seemed to suffer more postoperative complications. CONCLUSION: Considering improved life expectancy, it is crucial to treat elderly PHCC patients appropriately and attempts should be made to radical surgery based on comorbidities and functional status.

Resecciones hepáticas laparoscópicas: ¿qué hemos aprendido luego de más de 20 años de experiencia? / Laparoscopic liver resections: What have we learned after more than 20 years of experience?

Introducción: El objetivo de este estudio es describir nuestra experiencia en los últimos 8años de resecciones hepáticas laparoscópicas (RHL) por tumores benignos y malignos, para evaluar indicaciones y resultados, y comparar los resultados con nuestra experiencia previa y con otros centros de referencia a nivel mundial. Métodos: A partir de una base de datos prospectiva de la Unidad de Cirugía Hepatopancreatobiliar y Trasplante Hepático del Hospital Italiano de Buenos Aires se analizaron de forma retrospectiva los pacientes que fueron sometidos a RHL entre septiembre de 2014 y junio de 2022 (períodoB) y se compararon los resultados con nuestra propia experiencia de RHL realizadas entre los años 2000 y 2014, publicada previamente (períodoA). Resultados: La indicación quirúrgica más frecuente fue por metástasis de cáncer colorrectal (26,4%). El 15,7% de las resecciones fueron hepatectomías mayores y el procedimiento más frecuentemente realizado fueron hepatectomías típicas y atípicas (58,4%), seguido por la hepatectomía lateral izquierda (20,3%). La tasa de complicaciones mayores fue del 10,1%. La mortalidad en los primeros 90días postoperatorios fue del 1%. La mediana de estancia postoperatoria fue de 4 (IQR: 3-6) días. La sobrevida global al año, a los 3 y a los 5años fue del 94%, del 84% y del 70%, respectivamente, con una media de seguimiento de 22,9meses. Conclusiones: Las resecciones hepáticas por vía laparoscópica en manos de cirujanos entrenados continúan creciendo de manera segura, y hemos visto un aumento en la indicación de RHL para patologías malignas y resecciones mayores, tendencia que acompaña al resto de los grandes centros del mundo y se ha convertido en el método de elección para el tratamiento quirúrgico de la mayoría de los tumores hepáticos. (AU)

Introduction: The aim of this study is to describe our experience in the last 8years of laparoscopic liver resections (LLR) for benign and malignant tumors, to evaluate indications and results, and to compare the results with our previous experience and with other reference centers worldwide. Methods: Based on a prospective database of the Hepatopancreatobiliary Surgery and Liver Transplantation Unit of the Hospital Italiano de Buenos Aires, patients who underwent LLR between September 2014 and June 2022 were retrospectively analyzed (periodB) and where compared to our own experience from 2000-2014 previously published (periodA). Results: Colorectal liver metastasis was the main indication for surgery (26.4%). Major hepatectomies accounted for 15.7% of resections and the most frequently performed procedure was typical and atypical hepatectomies (58.4%) followed by left lateral hepatectomy (20.3%). The total postoperative major complications rate was 10.1% and the 90-day postoperative mortality was 1%. The median postoperative stay was four (IQR: 3-6) days. The overall survival rate estimated at 1, 3 and 5 years was 94%, 84% and 70%, respectively, with a median follow-up of 22.9months. Conclusions: LLRs in the hands of trained surgeons continue to grow safely, and we have seen an increase in the indication of LLR for malignant pathologies and major resections, a trend that follows the rest of the major centers in the world and has become the method of choice for surgical treatment of most liver tumors. (AU)

Metodologías de Medición de Volumetría Hepática / Methodologies For Measuring Hepatic Volumetry

Lograr determinar el volumen total de un hígado (VHT), o volumetría hepática, pasa a ser de relevancia en diversas situaciones, tales como, vigilancia del progreso de una enfermedad de carácter crónico, planificación de resecciones y trasplantes hepáticos; y observación del clearance hepático de algunos fármacos hepatotropos. La VHT se puede realizar utilizando métodos de segmentación en el curso de una tomografía computarizada (TC), ya sean estos manual, automáticos, y semiautomáticos; mediante resonancia nuclear (RN), utilizando softwares de distintas generaciones (1ª a 4ª). La medición de VHT está indicada en pacientes sometidos a resecciones hepáticas mayores, en el contexto del tratamiento de neoplasias (carcinoma hepatocelular, colangiocarcinoma, metástasis hepáticas o tumores benignos de gran tamaño), abscesos (piogénicos, amebianos), y después de un traumatismo hepático complejo; así como también en la etapa preoperatoria de un trasplante hepático. El objetivo de este manuscrito fue generar un documento de estudio sobre métodos para determinar volumetría hepática.

SUMMARY: Being able to determine the total hepatic volume (THV), or THV, becomes relevant in various situations, such as monitoring the progress of a chronic disease, planning resections and liver transplants; and observation of the hepatic clearance of some hepatotropic drugs. THV can be performed using segmentation methods in the course of a computed tomography (CT), whether manual, automatic, or semi-automated; by nuclear resonance (NR), using software from different generations (1st to 4st). THV measurement is indicated in patients undergoing major liver resections, in the context of treatment of neoplasms (hepatocellular carcinoma, cholangiocarcinoma, liver metastases or large benign tumors), abscesses (pyogenic, amoebic), and after liver trauma complex, as well as in the preoperative stage of a liver transplant. The aim of this manuscript was to generate a study document regarding methods for determine hepatic volumetry.

Prognostic analysis and outcome of hilar cholangiocarcinoma after radical resection: a retrospective study.

OBJECTIVES: The predictive factors affecting the survival of hilar cholangiocarcinoma (HC) are ambiguous. This study aimed to identify the predictors and recurrence patterns of HC. METHODS: A retrospective analysis of the clinicopathological findings of 126 patients with HC from 2009 to 2019 was performed. RESULTS: The proportion of Bismuth I and II HC in the recurrence group was higher than that in the non-recurrence group (p < 0.01). The recurrence group had poorer tumor differentiation, a more advanced N stage, and a higher incidence of perineural invasion compared with the non-recurrence group. N stage and tumor differentiation were independently associated with disease-free and overall survival of patients (p < 0.01). Bile duct resection (BDR) combined with hepatectomy was more favorable to disease-free and overall survivals than BDR alone in Bismuth I and II HC, although p values were marginal (p = 0.072 and p = 0.045). A higher proportion of patients in the non-recurrence group underwent BDR combined with hepatectomy than that in the recurrence group (p < 0.01). CONCLUSIONS: N stage and tumor differentiation are the two independent predictors of patient survival. BDR combined with hepatectomy is recommended for patients with Bismuth I and II hilar cholangiocarcinoma.

OBJETIVOS: Los predictores que afectan a la supervivencia del colangiocarcinoma hiliar son ambiguos. Este estudio tiene como objetivo identificar los factores predictivos y los patrones de recurrencia del colangiocarcinoma hiliar. MÉTODOS: Se aplicó un análisis retrospectivo con126 pacientes con colangiocarcinoma hiliar desde 2009 hasta 2019. RESULTADOS: La proporción de colangiocarcinoma hiliar Bismuth I y II en el grupo de recurrencia fue mayor que en el grupo de no recurrencia (p < 0.01). El tumor del grupo de recidiva tenía un estadio N más avanzado que el del grupo de no recidiva. El estadio N se asocia de forma independiente con la supervivencia libre de enfermedad y global del paciente (p < 0.01). La resección de la vía biliar combinada con la hepatectomía benefició más a la supervivencia libre de enfermedad y global que la resección de la vía biliar sola en el colangiocarcinoma hiliar (p = 0.072 y p = 0.045). Una mayor proporción de pacientes se sometió a resección de la vía biliar combinada con hepatectomía en el grupo de no recidiva que en el de recidiva (p < 0.01). CONCLUSIONES: El estadio N fue el predictor independiente. Se recomienda la resección de la vía biliar combinada con hepatectomía para los pacientes con colangiocarcinoma hiliar Bismuth I y II.

Resecabilidad de las metástasis hepáticas según la lateralidad del tumor colorrectal primario y la temporalidad de presentación. Serie de casos en dos instituciones de alta complejidad de la ciudad de Medellín / Resectability of liver metastasis according to the laterality of the primary colorectal tumor and the temporality of presentation. Case series in two high-complex institutions in the city of Medellín

Introducción. La neoplasia colorrectal es una patología oncológica muy frecuente a nivel mundial y una de las causas más comunes de mortalidad por cáncer. La epidemiologia, diagnóstico y tratamiento han sido ampliamente estudiadas, mientras que los datos sobre la enfermedad metastásica siguen siendo escasos. El hígado es el órgano más comúnmente afectado y algunos estudios sugieren diferencias en sobrevida y resecabilidad según la localización del tumor primario. El objetivo de este estudio fue establecer el comportamiento y resecabilidad de neoplasias avanzadas colorrectales en dos hospitales de la ciudad de Medellín, Colombia. Métodos. Estudio analítico retrospectivo para identificar los patrones de las metástasis hepáticas y sus características en función de las diferencias clínicas, histológicas y endoscópicas del tumor colorrectal primario entre 2015 y 2020. Resultados. Se recolectaron 54 pacientes con neoplasia colorrectal y metástasis hepáticas, 21 (39 %) derechas y 33 (61 %) izquierdas. El número de metástasis promedio fue de 3,1 en tumores del lado derecho y de 2,4 del izquierdo y el tamaño promedio de cada lesión fue de 4,9 y 4,2 cm, respectivamente. La tasa de resecabilidad fue del 42 % en los tumores derechos y del 82 % en los izquierdos. Las lesiones metacrónicas presentaron una tasa de resecabilidad del 90 % y las sincrónicas del 61 %. Conclusión. En este estudio, las lesiones originadas en neoplasias primarias del colon izquierdo y las lesiones metacrónicas fueron factores pronósticos favorables para la resecabilidad, un factor que impacta en la sobrevida y el tiempo libre de enfermedad de estos pacientes.

Introduction. Colorectal tumor is the most frequent pathology worldwide and one of the most common causes of mortality attributed to cancer. Epidemiology, diagnosis and treatment have been extensively studied, while information on metastatic disease remains scarce, despite being the main cause of death. Some studies suggest differences in terms of survival and resectability according to the anatomical location of the primary tumor. The aim is to establish the behavior and resectability of advanced cancers in two high-complex hospitals in the city of Medellín, Colombia. Methods. Cross-sectional observational study from secondary sources of information based on a retrospective cohort, using available data from adult patients with colorectal cancer and liver metastases between 2015 and 2020. Results. Fifty-four patients with colorectal neoplasms and liver metastases were collected, of which 21 (39%) were on the right side. The average number of liver metastases was 3.1 on the right side and 2.4 on the left, and the average size of each metastatic lesion was 4.9 cm and 4.2 cm, respectively. The resectability rate was 42% in the right tumors and 82% in the left ones. Metachronous lesions had a resectability rate of 90% and synchronous ones 61%. Conclusion. The complete resectability of liver metastatic lesions is the only therapeutic alternative with impact, in terms of survival and disease-free time in these patients. The favorable prognostic factors for the resectability of these lesions in our study were those originating from left primary tumors and metachronous lesions, where less liver tumor involvement was evidenced

Resección hepática laparoscópica versus hepatectomía abierta para lesiones benignas: estudio comparativo con propensity score matching / Laparoscopic liver resection versus open liver resection for benign lesions: a comparative study with propensity score matching

RESUMEN Antecedentes : las ventajas de la hepatectomía videolaparoscópica (HVL) hicieron que gane cada vez más campo para el tratamiento de los tumores hepáticos benignos (THB). Objetivo : comparar los resultados perioperatorios de pacientes sometidos a HVL con los de los operados con hepatectomía abierta (HA) por THB, emparejados con propensity score matching (PSM). Material y métodos : estudio descriptivo, retrospectivo y comparativo de HA y HVL por THB entre agosto de 2010 y junio de 2021. Se analizaron variables demográficas, preoperatorias, intraoperatorias y posoperatorias. Para evitar sesgos de las distintas covariables entre los grupos se realizó un PSM 1:1. Resultados : de 403 hepatectomías, se analizaron 82 por THB. De ellas 36 (44%) fueron HA y 46 (56%) HVL. Edad media 45 ±14 años, 65% mujeres. Tras realizar el PSM, quedaron dos grupos de 28 pacientes cada uno. En HA, 5 (18%) pacientes requirieron transfusiones y ninguno en HVL (p = 0,01). Las complicaciones mayores se presentaron en 4 (14%) pacientes en HA, y ninguna en HVL (p = 0,03). Se reoperaron 4 (14%) pacientes con HA y ningún paciente con HVL (p = 0,03). La estadía hospitalaria total fue significativamente mayor en las HA (p = 0,04). No se registraron muertes a los 90 días en ninguno de los dos grupos. Conclusión : la HVL por THB es una técnica segura y eficaz, ya que los pacientes presentaron menor requerimiento transfusional, número de reoperaciones, de complicaciones mayores y de estadía hospitalaria que con HA. Por las ventajas encontradas, la HVL podría ser considerada la técnica de elección en cirugía por THB.

ABSTRACT Background : The advantages of laparoscopic liver resection (LLR) have increased its use for the treatment of benign liver tumors (BLTs). Objective : The aim of this study was to compare the perioperative outcomes of patients undergoing LLR with those operated on with open liver resection (OLR) for BLTs using propensity score matching (PSM). Material and methods : We conducted a descriptive and retrospective study comparing OLRs with LLRs performed between August 2010 and June 2021. The demographic, perioperative, intraoperative and postoperative variables were analyzed. We used PSM with 1:1 matching to avoid biases of the different covariates between the groups. Results : Of 303 liver resections, 82 corresponded to BLTs and were included in the analysis; 36 (44%) were OLRs and 46 (56%) were LLRs. Mean age was 45 ±14 years and 65% were women. After PSM, two groups of 28 patients each were constituted. Five patients (18%) in the OLR group and none in the LLR required transfusions (p = 0.01). Major complications, occurred in 4 (14%) patients in the OLR group and in no cases in the LLR group (p = 0.03). Four (14%) undergoing OLR required reoperation versus no patients with LLR (p = 0.03). Total length of hospital stay was significantly longer in OLR (p = 0.04). There were no deaths in any of the groups within 90 days. Conclusion : LLR for BLTs is a safe and effective technique, with lower requirement for transfusions, fewer reoperations and major complications and shorter length of hospital stay than OLR, Therefore, LLR could be considered the surgical technique of choice for BLTs.

Influence of the tumor site and histopathology after resection for non-colorectal non-neuroendocrine liver metastases. A single center experience / Influencia del origen e histopatología tumoral tras la resección de metástasis hepáticas de origen no colorrectal no neuroendocrino. Experiencia unicéntrica

Introduction: It remains unclear whether liver resection is justified in patients with non-colorectal non-neuroendocrine liver metastases (NCNNLM). A single-center study was conducted to analyse overall survival (OS), disease-free survival (DFS), and potential prognostic factors in patients with different types of NCNNLM. Method: A retrospective analysis of all patients who underwent liver resection of NCNNLM from January 2006 to July 2019 was performed. Results: A total of 62 patients were analyzed. 82.3% presented metachronous metastases and 74.2% were unilobar. The most frequent primary tumor site (PTS) were breast (24.2%), urinary tract (19.4%), melanoma (12.9%), and pancreas (9.7%). The most frequent primary tumor pathologies were breast carcinoma (24.2%), non-breast adenocarcinoma (21%), melanoma (12.9%) and sarcoma (12.9%). The most frequent surgical procedure performed was minor hepatectomy (72.6%). R0 resection was achieved in 79.5% of cases. The major complications’ rate was 9.7% with a 90-day mortality rate of 1.6%. The 1, 3 and 5-year OS/DFS rate were 65%/28%, 45%/36% and 46%/28%, respectively. We identified the response to neoadjuvant therapy and PTS as possible prognostic factors for OS (P =0.06) and DFS (P =0.06) respectively. Conclusion: Based on the results of our series, NCNNLM resection produces beneficial outcomes in terms of OS and DFS. PTS and the response to neoadjuvant therapy could be the main prognostic factors after resection. (AU)

Introducción: No está totalmente aclarado si la resección hepática está justificada en pacientes con metástasis hepáticas no colorrectales no neuroendocrinas (MHNCNN). Hemos realizado un estudio en un solo centro para analizar la supervivencia global (SG), la supervivencia libre de enfermedad (SLE) y posibles factores pronósticos en pacientes con diferentes tipos de MHNCNN. Método: Se realizó un análisis retrospectivo de todos los pacientes que se sometieron a resección hepática de MHNCNN desde enero de 2006 hasta julio de 2019. Resultados: Se analizaron un total de 62 pacientes. El 82,3% presentó metástasis metacrónicas y el 74,2% fueron unilobares. El sitio original del tumor primario (STP) más frecuente fue mama (24,2%), tracto urinario (19,4%), melanoma (12,9%) y páncreas (9,7%). Las histopatologías tumorales primarias más frecuentes fueron el carcinoma de mama (24,2%), el adenocarcinoma no mamario (21%), el melanoma (12,9%) y el sarcoma (12,9%). El procedimiento quirúrgico realizado con mayor frecuencia fue la hepatectomía menor (72,6%). La resección R0 se logró en el 79,5% de los casos. La tasa de complicaciones mayores fue del 9,7% con una tasa de mortalidad a los 90 días del 1,6%. La tasa de SG / SLE a 1, 3 y 5 años fue de 65% / 28%, 45% / 36% y 46% / 28%, respectivamente. Identificamos la respuesta a la terapia neoadyuvante y el STP como posibles factores pronósticos de SG (p = 0,06) y SLE (p = 0,06) respectivamente. Conclusión: Según los resultados de nuestra serie, la resección de MHNCNN produce resultados beneficiosos en términos de SG y SLE. El STP y la respuesta a la terapia neoadyuvante podrían ser los principales factores pronósticos tras la resección. (AU)

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

Laparoscopic liver resections: What have we learned after more than 20 years of experience?

INTRODUCTION: The aim of this study is to describe our experience in the last 8 years of laparoscopic liver resections (LLR) for benign and malignant tumors, to evaluate indications and results, and to compare the results with our previous experience and with other reference centers worldwide. METHODS: Based on a prospective database of the Hepatopancreatobiliary Surgery and Liver Transplantation Unit of the Hospital Italiano de Buenos Aires, patients who underwent LLR between September 2014 and June 2022 were retrospectively analyzed (period B) and where compared to our own experience from 2000 to 2014 previously published (period A). RESULTS: Colorectal liver metastasis was the main indication for surgery (26.4%). Major hepatectomies accounted for 15.7% of resections and the most frequently performed procedure was typical and atypical hepatectomies (58.4%) followed by left lateral hepatectomy (20.3%). The total postoperative major complications rate was 10.1% and the 90-day postoperative mortality was 1%. The median postoperative stay was four (IQR: 3-6) days. The overall survival rate estimated at 1, 3 and 5 years was 94%, 84% and 70%, respectively, with a median follow-up of 22.9 months. CONCLUSIONS: LLRs in the hands of trained surgeons continue to grow safely, and we have seen an increase in the indication of LLR for malignant pathologies and major resections, a trend that follows the rest of the major centers in the world and has become the method of choice for surgical treatment of most liver tumors.

Influence of the tumor site and histopathology after resection for non-colorectal non-neuroendocrine liver metastases. A single center experience.

INTRODUCTION: It remains unclear whether liver resection is justified in patients with non-colorectal non-neuroendocrine liver metastases (NCNNLM). A single-center study was conducted to analyse overall survival (OS), disease-free survival (DFS), and potential prognostic factors in patients with different types of NCNNLM. METHOD: A retrospective analysis of all patients who underwent liver resection of NCNNLM from January 2006 to July 2019 was performed. RESULTS: A total of 62 patients were analyzed. 82.3% presented metachronous metastases and 74.2% were unilobar. The most frequent primary tumor site (PTS) were breast (24.2%), urinary tract (19.4%), melanoma (12.9%), and pancreas (9.7%). The most frequent primary tumor pathologies were breast carcinoma (24.2%), non-breast adenocarcinoma (21%), melanoma (12.9%) and sarcoma (12.9%). The most frequent surgical procedure performed was minor hepatectomy (72.6%). R0 resection was achieved in 79.5% of cases. The major complications' rate was 9.7% with a 90-day mortality rate of 1.6%. The 1, 3 and 5-year OS/DFS rate were 65%/28%, 45%/36% and 46%/28%, respectively. We identified the response to neoadjuvant therapy and PTS as possible prognostic factors for OS (P =0.06) and DFS (P =0.06) respectively. CONCLUSION: Based on the results of our series, NCNNLM resection produces beneficial outcomes in terms of OS and DFS. PTS and the response to neoadjuvant therapy could be the main prognostic factors after resection.

Partial hepatectomy using linear cutter stapler: are there advantages? / Hepatectomia parcial empregando grampeador linear cortante: há vantagens?

ABSTRACT BACKGROUND: Morbidity of liver resections is related to intraoperative bleeding and postoperative biliary fistulas. The Endo-GIA stapler (EG) in liver resections is well established, but its cost is high, limiting its use. The linear cutting stapler (LCS) is a lower cost device. AIMS: To report open liver resections, using LCS for transection of the liver parenchyma and en bloc stapling of vessels and bile ducts. METHODS: Ten patients were included in the study. Four patients with severe abdominal pain had benign liver tumors (three adenomas and one focal nodular hyperplasia). Among the remaining six patients, four underwent liver resection for the treatment of colorectal liver metastases, three of which had undergone preoperative chemotherapy. The other two cases were one patient with metastasis from a testicular teratoma and the other with metastasis from a gastrointestinal neuroectodermal tumor. RESULTS: The average length of stay was five days (range 4-7 days). Of the seven patients who underwent resections of segments II/III, two presented postoperative complications: one developed a seroma and the other a collection of abdominal fluid who underwent percutaneous drainage, antibiotic therapy, and blood transfusion. Furthermore, the three patients who underwent major resections had postoperative complications: two developed anemia and received blood transfusions and one had biloma and underwent percutaneous drainage and antibiotic therapy. CONCLUSIONS: The use of the linear stapler in hepatectomies was efficient and at lower costs, making it suitable for use whenever EG is not available. The size of the LCS stapler shaft is more suitable for en bloc transection of the left lateral segment of the liver, which is thinner than the right one. Further studies are needed to evaluate the safety of LCS for large liver resections and resections of tumors located in the right hepatic lobe.

RESUMO RACIONAL: A morbidade das ressecções hepáticas está relacionada a sangramento intraoperatório e fístulas biliares pós-operatórias. O grampeador Endo-GIA (EG) em ressecções hepáticas está bem estabelecido, mas o seu custo é elevado, limitando seu uso. O grampeador de corte linear (LCS) é um dispositivo com menor custo. OBJETIVOS: Relatar ressecções hepáticas abertas, empregando o LCS para transecção do parênquima hepático e grampeamento em bloco de vasos e ductos biliares. MÉTODOS: Dez pacientes foram incluídos no estudo. Quatro pacientes com dor abdominal importante apresentavam tumores hepáticos benignos (três adenomas e um hiperplasia nodular focal). Dentre os demais seis pacientes, quatro foram submetidos à ressecção hepática para o tratamento de metástases hepáticas colorretais, sendo que três deles haviam sido submetidos à quimioterapia pré-operatória. Os dois outros casos foram um paciente com metástase de teratoma testicular e o outro com metástase de tumor neuroectodérmico gastrointestinal. RESULTADOS: O tempo médio de internação foi de 5 dias (variação=4-7 dias). Dos sete pacientes submetidos a ressecções dos segmentos II/III, dois apresentaram complicações pós-operatórias: um paciente desenvolveu seroma e o outro uma coleção de fluido abdominal submetido a drenagem percutânea, antibioticoterapia e transfusão de sangue. Além disso, os três pacientes submetidos a ressecções maiores tiveram complicações pós-operatórias: dois pacientes desenvolveram anemia e receberam transfusões de sangue e um paciente apresentou biloma e foi submetido a drenagem percutânea e antibioticoterapia. CONCLUSÕES: O emprego do grampeador linear nas hepatectomias foi eficiente e a custos mais baixos, tornando-o adequado para uso sempre que EG não estiver disponível O tamanho da haste do grampeador LCS é mais adequado para a transecção em bloco do segmento lateral esquerdo do fígado, que é mais fino que o direito. Novos estudos são necessários para avaliar a segurança do LCS para grandes ressecções hepáticas e ressecções de tumores localizados no lobo hepático direito.

Cambios fisiopatológicos y complicaciones de las resecciones hepáticas / Physiopathological changes and complications of liver resections / Alterações fisiopatológicas e complicações das ressecções hepáticas

Las modernas técnicas quirúrgicas y anestésicas han permitido ampliar el número de intervenciones quirúrgicas a nivel hepático por diversas patologías. Logrando disminuir su moralidad pero manteniendo al día de hoy elevados niveles de morbilidad. Durante la cirugía hepática se producen cambios hemodinámicos vinculados a la movilización del hígado, a los clampeos y a las pérdidas sanguíneas independientemente de la vía de abordaje. En el postoperatorio las complicaciones o cambios fisiopatológicos derivan de las lesiones producidas por los fenómenos de isquemia y reperfusión; y aquellas producidas por la regeneración hepática. Dicha capacidad depende no solo de la cantidad de hígado remanente sino también de la posible hepatopatía preexistente. La insuficiencia hepática postoperatoria es la complicación más temida y se manifiesta con ictericia, ascitis, encefalopatía y alteraciones en la paraclínica como la hiperbilirrubinemia y descenso del tiempo de protrombina. Las complicaciones quirúrgicas dependen del procedimiento realizado y se dividen principalmente en biliares y vasculares. Las secuelas de las hepatectomías dependen de factores como el estado general del paciente, la presencia hepatopatía, el acto quirúrgico y la cantidad y calidad del hígado remanente.

Modern surgical and anesthetic techniques have made it possible to increase the number of liver surgeries for various pathologies. This has reduced morbidity but still maintains high levels of morbidity. During hepatic surgery, hemodynamic changes related to liver mobilization, clamping and blood loss occur independently of the approach route. In the postoperative period, complications or pathophysiological changes derive from the lesions produced by ischemia and reperfusion phenomena; and those produced by hepatic regeneration. This capacity depends not only on the amount of remaining liver but also on the possible pre-existing hepatopathy. Postoperative liver failure is the most feared complication and manifests with jaundice, ascites, encephalopathy and paraclinical alterations such as hyperbilirubinemia and decreased prothrombin time. Surgical complications depend on the procedure performed and are mainly divided into biliary and vascular. The sequelae of hepatectomies depend on factors such as the patient's general condition, the presence of liver disease, the surgical procedure and the quantity and quality of the remaining liver.

As modernas técnicas cirúrgicas e anestésicas tornaram possível aumentar o número de cirurgias hepáticas para várias patologias. Isto levou a uma diminuição da morbidade, mas ainda mantém altos níveis de morbidade. Durante a cirurgia hepática, ocorrem alterações hemodinâmicas ligadas à mobilização hepática, pinçamento e perda de sangue, independentemente da via de aproximação. No período pós-operatório, complicações ou alterações fisiopatológicas derivam de lesões causadas por fenômenos de isquemia e reperfusão, e aquelas causadas pela regeneração hepática. Esta capacidade depende não apenas da quantidade de fígado restante, mas também de uma possível doença hepática pré-existente. A insuficiência hepática pós-operatória é a complicação mais temida e se manifesta com icterícia, ascite, encefalopatia e alterações paraclínicas, tais como hiperbilirrubinemia e diminuição do tempo de protrombina. As complicações cirúrgicas dependem do procedimento realizado e são divididas principalmente em biliares e vasculares. As seqüelas de hepatectomias dependem de fatores como o estado geral do paciente, a presença de doença hepática, o procedimento cirúrgico e a quantidade e qualidade do fígado restante.

Hemangioma hepático gigante único: serie de casos con seguimiento de pacientes tratados quirúrgicamente / Single giant liver hemangioma: case series with follow-up of patients treated surgically

El tratamiento del hemangioma hepático gigante (HHG), sigue siendo motivo de controversia. El objetivo de este estudio fue reportar los resultados de pacientes con HHG resecados quirúrgicamente en términos de morbilidad postoperatoria (MPO). Serie de casos con seguimiento. Se incluyeron pacientes con HHG, sometidos a cirugía de forma consecutiva, en Clínica RedSalud Mayor, entre 2011 y 2020. La variable resultado fue MPO. Otras variables de interés fueron: tiempo quirúrgico, estancia hospitalaria y mortalidad. Las pacientes fueron seguidas de forma clínica. Se utilizó estadística descriptiva, con medidas de tendencia central y dispersión. Se intervinieron 5 pacientes, con una mediana de edad de 38 años. La medianas del tiempo quirúrgico y estancia hospitalaria; fueron 75 min y 4 días respectivamente. La MPO fue 20 % (1 caso de seroma). Con una mediana de seguimiento de 41 meses, los pacientes se encuentran asintomáticos y no se ha verificado morbilidad alejada. La resección quirúrgica de un HHG se puede realizar con escasa morbilidad, tanto en términos numéricos como de gravedad de la complicación observada.

SUMMARY: Treatment of giant hepatic hemangioma (GHH) remains controversial. The aim of this study was to report the outcomes of surgically resected GHH patients in terms of postoperative morbidity (POM). Case series with follow-up. Patients with GHH who underwent surgery consecutively at the RedSalud Mayor Clinic between 2011 and 2020 were included. The outcome variable was POM. Other variables of interest were surgical time, hospital stay and mortality. The patients were followed up clinically. Descriptive statistics were used, with measures of central tendency and dispersion. Five patients underwent surgery, with a median age of 38 years. The median surgical time and hospital stay; were 75 min and 4 days respectively. The MPO was 20 % (1 case of seroma). With a median follow-up of 41 months, the patients are asymptomatic, and no distant morbidity has been verified. Surgical resection of GHH can be performed with low morbidity, both in terms of numbers and the severity of the complication observed.

ALPPS monosegmento, una variante de hepatectomía extrema para resección de metástasis hepáticas de cáncer colorrectal

La resección quirúrgica con márgenes negativos de las metástasis hepáticas de cáncer colorectal, representa la única opción de tratamiento con potencial curativo, sin embargo, el 85% de estos pacientes son considerados irresecables a la evaluación inicial, ya sea por número, tamaño y localización de las metástasis o por un remanente hepático futuro insuficiente. ALPPS es una técnica quirúrgica compleja, que permite en dos tiempos operatorios, la resección de extensas porciones de parénquima hepático tras un periodo de incremento volumétrico del remanente, que alcanza el 80% en un periodo de 9 días, lo que supera ampliamente a otras técnicas como la embolización/ligadura portal preoperatoria. Pese a cuestionamientos iniciales relativos a la morbimortalidad asociada, la significativa ganancia de masa hepatocelular lograda con ALPPS, ha permitido el tratamiento exitoso de pacientes con alta carga tumoral metastásica hepática, tensionando principios fundamentales clásicamente considerados para la realización de hepatectomías mayores en forma segura. El objetivo de este trabajo es presentar la experiencia inicial con la aplicación de la técnica de ALPPS llevado al extremo de dejar un remanente hepático constituido por un único segmento.

R0 resection of colorectal liver metastases is the main curative treatment option; however, 85% of patients are considered initially unresectable, either due to number, size and location of metastases or insufficient future liver remnant. ALPPS is a complex surgical technique, which allows, in two operative times, the resection of extensive portions of liver parenchyma after a period of volumetric remnant increase, which reaches 80% in 9 days, far exceeding other techniques as well as preoperative portal embolization / ligation. Despite initial doubts regarding the associated morbidity and mortality, the significant gain in hepatocellular mass achieved with ALPPS has allowed the successful treatment of patients with high hepatic metastatic tumor burden, questioning fundamental principles classically considered for safely performing major hepatectomies. The aim of this article is to show the initial experience with the ALPPS technique application taken to the extreme of leaving a liver remnant made up of a single segment.

Application and validation of a surgical risk calculator in a liver surgery cohort of patients in Veracruz, Mexico.

OBJECTIVE: Multiple models have tried to predict the morbidity and mortality of liver resections (HR). This study aims to determine the efficacy and validity of the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) surgical risk calculator in a cohort of patients undergoing HR in Veracruz, Mexico. MATERIAL AND METHODS: Retrospective analysis of patients undergoing HR between 2005 and 2019. To estimate the performance of the calculator, the observed results were compared with the average risk predicted by the calculator, using ROC curve, Brier score and Z test. RESULTS: 67 patients were evaluated, mean age 51.9 years of age, 50.7% female. The majority of resections (56.7%) were for malignancy and 62.1% were partial hepatectomies (up to 3 liver segments). The morbidity was 25.4% and the mortality 4.5%. There was a good prediction in the complications (serious complication: C = 0.725 statistic, Brier score 0.26, p = 0.006 and any statistical complication C = 0.731, Brier score 0.33, p = 0.005) and mortality (C = 0.922 statistic, Brier score 0.005, p = 0.014). CONCLUSIONS: The application of the ACS-NSQIP calculator in patients undergoing HR has good discrimination power and good predictive ability. Prediction of postoperative risks achieves a preoperative planning of the appropriate procedure.

OBJETIVO: Múltiples modelos han intentado predecir la morbilidad y mortalidad de las resecciones hepáticas (RH). Este estudio tiene por objetivo determinar la eficacia y validez de la calculadora de riesgo quirúrgico del American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) en una cohorte de pacientes sometidos a RH en Veracruz, México. MATERIAL Y MÉTODOS: Análisis retrospectivo de pacientes sometidos a RH entre 2005 y 2019. Se compararon los resultados observados con la media del riesgo previsto por la calculadora, mediante Curva ROC, score de Brier y prueba Z. RESULTADOS: Se evaluaron 67 pacientes, con 51.9 años de edad media, un 50.7% de sexo femenino. La mayoría de las resecciones (56.7%) fueron por patología maligna y el 62.1% fueron hepatectomías parciales. La morbilidad fue del 25.4% y la mortalidad del 4.5%. Hubo una predicción buena en las complicaciones (complicación seria, estadística: C: 0.725, score Brier: 0.26, p = 0.006 y cualquier complicación, estadística: C: 0.731, score Brier: 0.33, p = 0.005) y la mortalidad (estadística C: 0.922, score Brier: 0.005, p = 0.014). CONCLUSIONES: La aplicación de la calculadora ACS-NSQIP en pacientes sometidos a RH tiene buen poder de discriminación y buena habilidad predictiva. Predecir riesgos postoperatorios logra una adecuada planeación preoperatoria del procedimiento.

Desafío y futuro de la cirugía robótica hepática y pancreática. Análisis de 64 casos en una unidad especializada / Challenge and future of liver and pancreatic robotic surgery. Analysis of 64 cases in a specialized unit

Introducción: La cirugía robótica ha demostrado su eficacia en ciertos procedimientos quirúrgicos. Sin embargo, en cirugía hepática y pancreática (HBP) su uso es todavía poco frecuente. Se presenta la experiencia inicial en cirugía robótica HBP de una unidad especializada en un hospital de tercer nivel. Método: Se han estudiado en forma prospectiva los resultados de los pacientes intervenidos de cirugía HBP robótica entre abril de 2018 y octubre de 2020. Los datos analizados corresponden a datos demográficos, técnicas quirúrgicas realizadas y morbimortalidad asociada. Resultados Se intervinieron 64 pacientes, sometidos a 35 hepatectomías (mayores [6,7%], anatómicas [52,9%], limitadas [34,4%], quistectomías [3%] y marsupializaciones [3%]) y 29 pancreatectomías/resecciones duodenales (distales [48,2%], centrales [6,9%], cefálicas [13,8%], enucleaciones [24,1%], ampulectomías [3,5%] y resecciones duodenales [3,5%]).En cirugía hepática el tiempo operatorio medio fue de 204,4 minutos (100-265 min), la mediana de complicaciones postoperatorias según la escala de Clavien-Dindo fue de uno (1-4), las pérdidas hemáticas medias de 166,7 mL (100-300 mL), no existió conversión y la estancia postoperatoria media de cuatro días (2-14 días).En cirugía pancreática el tiempo operatorio medio fue de 243,8 minutos (125-460 min), la mediana de complicaciones postoperatorias de dos (1-4), las pérdidas hemáticas de 202,3 mL (100-500 mL) asociadas a una tasa de conversión del 17,8% y una estancia media de siete días (3-23 días). Conclusiones: La cirugía robótica HBP es segura y factible. Se sugiere que su uso facilita la cirugía conservadora de parénquima, el acceso a segmentos posteriores hepáticos y la realización de anastomosis en la reconstrucción pancreática respecto a la cirugía laparoscópica (AU)

Introduction: Robotic surgery has proven effective in certain surgical procedures. However, in liver and pancreatic surgery (HBP) its use is still rare. The initial experience in HBP robotic surgery of a specialized unit of a tertiary hospital is presented. results of patients undergoing robotic HBP surgery between April 2018 and October 2020 have been prospectively studied. The data analyzed correspond to demographic data, surgical techniques performed, associated morbidity and mortality.Results64 patients were operated, corresponding to 35 hepatectomies (major [6.7%], anatomic [52.9%], limited [34.4%], cystectomies [3%] and marsupialization [3%]), 29 pancreatectomies (distal [48.2%], central [6.9%], cephalic [13.8%], enucleations [24.1%], ampullectomies [3.5%] and duodenal resections [3.5%]).In liver surgery the mean operative time was 204.4 minutes (100-265 min), the median postoperative complications according to the Clavien-Dindo scale was one (1-4), the mean blood losses 166.7 mL (100-300 mL), there was no conversion and the mean postoperative stay was four days (2-14 days).In pancreatic surgery, the mean operative time was 243.8 minutes (125-460 min), the median of postoperative complications was two (1-4), blood loss of 202.3 mL (100-500 mL) associated to a conversion rate 17.8% and an average stay of seven days (3-23 days).Conclusions: Robotic HBP surgery is safe and feasible. It is suggested that its use facilitates parenchymal sparing surgery, access to posterior liver segments and anastomosis in pancreatic reconstruction compared to laparoscopic surgery (AU)

Challenge and future of liver and pancreatic robotic surgery. Analysis of 64 cases in a specialized unit.

INTRODUCTION: Robotic surgery has proven effective in certain surgical procedures. However, in liver and pancreatic surgery (HBP) its use is still rare. The initial experience in HBP robotic surgery of a specialized unit of a tertiary hospital is presented. METHOD: The results of patients undergoing robotic HBP surgery between April 2018 and October 2020 have been prospectively studied. The data analyzed correspond to demographic data, surgical techniques performed, associated morbidity and mortality. RESULTS: 64 patients were operated, corresponding to 35 hepatectomies (major [6.7%], anatomic [52.9%], limited [34.4%], cystectomies [3%] and marsupialization [3%]), 29 pancreatectomies (distal [48.2%], central [6.9%], cephalic [13.8%], enucleations [24.1%], ampullectomies [3.5%] and duodenal resections [3.5%]). In liver surgery the mean operative time was 204.4 min (100-265 min), the median postoperative complications according to the Clavien-Dindo scale was one (1-4), the mean blood losses 166.7 mL (100-300 mL), there was no conversion and the mean postoperative stay was four days (2-14 days). In pancreatic surgery, the mean operative time was 243.8 min (125-460 min), the median of postoperative complications was two (1-4), blood loss of 202.3 mL (100-500 mL) associated to a conversion rate 17.8% and an average stay of seven days (3-23 days). CONCLUSIONS: Robotic HBP surgery is safe and feasible. It is suggested that its use facilitates parenchymal sparing surgery, access to posterior liver segments and anastomosis in pancreatic reconstruction compared to laparoscopic surgery.

Hepatectomía derecha totalmente laparoscópica para trasplante hepático con donante vivo adulto-adulto: técnica quirúrgica y experiencia inicial en Chile / Pure laparoscopic right hepatectomy for adult-to-adult living donor liver transplantation: surgical technique and initial experience in Chile

Resumen El trasplante hepático con donante vivo (THDV) es un procedimiento complejo y desafiante para el cirujano, ya que exige garantizar tanto la máxima seguridad para el donante, así como también, la mejor calidad del injerto para el receptor. Debido a lo anterior, la implementación de la cirugía mini-invasiva ha sido lenta en esta área. Sin embargo, en los últimos 10 años, gracias a los avances que ha experimentado la cirugía hepática laparoscópica, ha aumentado el interés de algunos grupos altamente especializados por incorporar la cirugía mini-invasiva a la cirugía del donante, principalmente en trasplante hepático donante vivo adulto-pediátrico (THDVA-P). Los favorables resultados obtenidos en esta área incluso han llevado a los expertos en el tema, a categorizar el abordaje laparoscópico para la cirugía del donante como el procedimiento estándar en THDVA-P. Contrario a lo anterior, la implementación de la laparoscopía para trasplante hepático donante vivo adulto-adulto (THDVA-A), es más compleja y requiere en su mayoría, una hepatectomía de lóbulo derecho o izquierdo para cumplir con las necesidades volumétricas del receptor. Esta cirugía es de mayor dificultad y riesgo para el donante, por lo que su indicación por vía mini-invasiva está limitada a centros de alto volumen y preparación, tanto en laparoscopía, como en trasplante hepático. En este trabajo, se busca dar a conocer la técnica quirúrgica y nuestra experiencia inicial con la primera hepatectomía derecha totalmente laparoscópica (HDTL) para THDVA-A realizada en Chile.

Living donor liver transplantation is a complex and challenging procedure. The surgeon needs to guarantee maximum safety for the donor, as well as the best quality of the graft for the recipient. For this reason, the implementation of mini-invasive surgery has been slow in this area. However, in the last 10 years, due to the advances in laparoscopic liver surgery, the interest of some highly specialized groups has increased in incorporating mini-invasive surgery into donor surgery, mainly in pediatric living donor liver transplantation. The favorable results obtained in this field, have even led to turn this procedure, into the technique of choice for pediatric living donor liver transplantation. Nevertheless, this procedure is even more challenging for adult-to-adult living donor transplantation. To meet the volumetric criteria of an adult, a complete hepatectomy of right or left lobe is mostly required. This surgery is of greater complexity and risk for the donor, so its indication by minimally invasive approach is limited to high-volume centers with preparation, both in laparoscopy and liver transplants. In this report we seek to present our surgical technique and initial experience with the first pure laparoscopic right hepatectomy for adult-to-adult living donor liver transplantation carried out in Chile.

Primary Malignant Liver Tumors: eight-year experience in a Pediatric Hospital in Brazil. A cross-sectional study / Neoplasias Malignas Primárias do Fígado: experiência de oito anos de um Hospital Pediátrico no Brasil. Estudo transversal

ABSTRACT Introduction: liver tumors are rare neoplasms in childhood (1-2%), and about 2/3 are malignant. Hepatoblastoma (HB) is the most frequent, followed by hepatocellular carcinoma (HCC). In both, the main treatment is surgical resection. Currently, chemotherapy and liver transplantation have improved outcomes. Objective: study of the epidemiological profile and evolution of liver cancer cases in a referral pediatric hospital. Methodology: a retrospective survey of medical records of patients aged up to 18 years with a diagnosis of primary malignant hepatic neoplasm between 2012 and 2020, carried out in the largest exclusively pediatric hospital in Brazil. Results: a total of 13 patients with malignant liver tumors (HB 12, HCC 1) were treated. Of the HB cases, 66,7% were male, with a mean age of 2 years and the main alteration in the palpable abdominal mass. Tumors involved an average of 3 liver segments, more in the right lobe (54%). Only one patient was treated with surgery without neoadjuvant therapy, another one underwent transplantation like the first treatment, and another 2 required liver transplantation as a rescue. The middle follow-up time of patients with HB was 39 months and only 1 case died due to febrile neutropenia. The 5-year overall and disease-free survival was 91.7% and 81.5%, respectively. Conclusion: Advanced staging at the time of diagnosis has always been a poor prognostic factor in patients with primary malignant liver tumors. However, the results and survival have improved with the advancement of chemotherapy, surgical technique, and liver transplantation.

RESUMO Introdução: tumores hepáticos são neoplasias raras na infância (1-2%), sendo que cerca de 2/3 são malignos. O hepatoblastoma (HB) é o mais frequente, seguido do carcinoma hepatocelular (CHC). Em ambos, o principal tratamento é a ressecção cirúrgica completa. Atualmente, a quimioterapia e o transplante hepático têm melhorado os resultados. Objetivo: estudo do perfil epidemiológico e evolução dos casos de cânceres hepáticos em um hospital pediátrico de referência. Método: Levantamento retrospectivo de prontuários de pacientes até 18 anos com diagnóstico de neoplasia maligna primária hepática entre 2012 e 2020 realizado no maior hospital exclusivamente pediátrico do Brasil. Resultados: foram atendidos 13 pacientes com tumores malignos hepáticos (HB 12, CHC 1). Dos casos de HB, 66,7% eram do sexo masculino, com idade média de 2 anos e a principal alteração foi massa abdominal palpável. Os tumores envolviam em média 3 segmentos hepáticos, mais em lobo direito (54%). Um paciente foi tratado com cirurgia sem neoadjuvância, um foi submetido a transplante inicialmente e outros 2 necessitaram de transplante hepático como resgate. O tempo de seguimento dos pacientes com HB foi de 39 meses e apenas 1 caso foi a óbito por neutropenia febril. A sobrevida geral e livre de doença em 5 anos foi de 91,7% e 81,5% respectivamente. Conclusão: o estadiamento avançado no momento do diagnóstico sempre foi um fator de mau prognóstico em pacientes com tumores hepáticos malignos primários. Entretanto, os resultados e a sobrevida têm melhorado significativamente com o avanço da quimioterapia, da técnica cirúrgica e do transplante hepático.