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

Hepatoblastoma en un paciente pediátrico a propósito de un caso

La incidencia de hepatoblastoma alrededor del mundo permanece constante entre 0.5 y 1.5 casos por millón de niños por año. En los Estados Unidos de América se reporta para el hepatoblastoma una incidencia anual de aproximadamente 1 por millón en niños menores de 15 años de edad. En Ecuador, en una investigación realizada en la ciudad de Cuenca, ocupa el séptimo lugar entre los tumores pediátricos. Se trata de un tumor infrecuente, cuya incidencia parece aumentar en los últimos años. Puede aparecer de forma aislada o integrarse en el contexto de un síndrome de predisposición. Presentamos el caso de un paciente pediátrico, femenina, preescolar de 3 años de edad, sin antecedentes perinatales de importancia, producto de la tercera gesta, nacida por cesárea por distocia de presentación a las 39 semanas. Cuenta con esquema de vacunación completo para la edad. Como antecedentes patológicos personales requiere una hospitalización por enfermedad diarreica aguda a los 2 años. Sin antecedentes quirúrgicos, antecedentes patológicos familiares de tía materna con hipotiroidismo. Se realizó exámenes complementarios de sangre y de imagen, los cuales revelaron una masa abdominal dependiente de hígado compatible con hepatoblastoma con niveles de AFP superiores a 1000ng/ml

The incidence of hepatoblastoma around the world remains constant between 0.5 and 1.5 cases per million children per year. In the United States of America, an annual incidence of approximately 1 per million is reported for hepatoblastoma in children under 15 years of age. In Ecuador, in a study carried out in the city of Cuenca, it ranks seventh among pediatric tumors. It is an infrequent tumor, its incidence seems to have increased in recent years. It can appear in isolation or be part of a predisposing syndrome. We present the case of a 3-year-old preschool female pediatric patient with no significant perinatal history, product of a third pregnancy, born by cesarean section due to presentation of dystocia at 39 weeks. She had a complete vaccination for her age. As past medical history, she was hospitalized for acute diarrheal disease at 2 years of age. She had no surgical history, family pathological history except for a maternal aunt with hypothyroidism. Complementary blood and imaging tests were performed, which revealed an abdominal liver-dependent mass, compatible with hepatoblastoma with AFP levels greater than 1000 ng/ml.

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.

Trasplante hepático en hemangioendotelioma epiteloide hepático con metástasis pulmonares: reporte de un caso / Liver transplantation in hepatic epitheloid hemangioendothelioma and lung metastases: a case report

El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón,peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.

[Liver transplantation in hepatic epitheloid hemangioendothelioma and lung metastases: a case report]. / Trasplante hepático en hemangioendotelioma epiteloide hepático con metástasis pulmonares: reporte de un caso.

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.

El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón, peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.

Associating liver partition and portal vein ligation (ALPPS): A two staged procedure, in Bogotá Colombia. Case report and literature review.

INTRODUCTION: For liver tumors (primary or metastases), surgery combined with neoadjuvant, or adjuvant chemotherapy is the treatment of choice, offering long term survival time and disease-free time period (Alvarez et al., 2012) Associating liver partition and portal vein ligation, or ALPPS, it's a surgical technique that increases the future liver remnant in a short period of time, trying to avoid postoperative liver failure (PLF), and achieving R0 resections in liver malignant tumors (Alvarez et al., 2012). PRESENTATION OF THE CASE: A 43 years old woman with colorectal liver metastases in both lobes. Colorectal surgical procedure was performed 1 year previous the liver intervention, followed by adjuvant chemotherapy. Decision of a tri-segmental hepatectomy was made to resolve the metastases. Into the surgical procedure, we evaluated the liver parenchyma, and the future liver remnant tissue was insufficient, for that reason we decided to perform ALPPS procedure. DISCUSSION: Colorectal liver metastases (CLRM) are considered the most common indication for ALPPS procedure according to the international registry. Compared with the portal vein ligation, resection rate varies from 50 to 80%, and the non-resectability disease was explained by tumor progression. Postoperative mortality rate was 5.1% in young patients (<60 years old), and 8% in general for CRLM. Oncologic outcomes represent an increased disease-free survival period and overall survival time compared with non-surgical approach. CONCLUSION: The ALPPS procedure it's an interesting approach to patients with not enough liver remnant tissue, with good oncologic results in terms of disease-free survival time, and overall survival. Appropriate selection of the patient, careful postoperative management, and a multidisciplinary approach are related with good postoperative outcomes.

Impact of Brazilian expanded criteria for liver transplantation in patients with hepatocellular carcinoma: a multicenter study.

INTRODUCTION AND OBJECTIVES: Hepatocellular carcinoma (HCC) is one of the main indications for orthotopic liver transplantation (OLT). In Brazil, selection criteria for HCC is an expanded version of the Milan Criteria (MC), the so-called "Brazilian Milan Criteria" (BMC). Our aims were to evaluate post-OLT outcomes in patients with HCC and analyze the BMC performance. MATERIALS AND METHODS: We conducted a multicenter, retrospective cohort study, analyzing medical records of 1,059 liver transplant recipients with HCC. Tumor was staged according to MC and BMC and correlated with overall survival (OS) and disease-free survival (DFS). We compared the ability of MC and BMC to predict OS and DFS using Delta C-statistic. RESULTS: Post-OLT OS were 63% in five years and HCC recurrence was observed in 8% of patients. At diagnosis, 85% of patients were within MC. Patients within MC at diagnosis and in the explant showed a higher OS and DFS than patients outside MC and within BMC and patients outside both criteria (p < 0.001). Patients outside MC in the explant had an increased risk of tumor recurrence (HR: 3.78; p < 0.001) and poor survival (HR:1.77; p = 0.003). The BMC presented a lower performance than MC in properly classifying patients regarding recurrence risk. CONCLUSIONS: In a large Brazilian cohort of HCC patients submitted to liver transplantation, we observed satisfactory overall survival and recurrence rates. However, patients transplanted within the Brazilian expanded criteria had lower OS and DFS when compared to patients within MC, which may generate future discussions regarding the criteria currently used.

Mucinous cystic neoplasm of the liver with biliary communication: case report and surgical therapeutic option.

BACKGROUND: Mucinous cyst neoplasm of the liver (MCN-L) comprise less than 5% of all cystic liver lesions and is characterized by the presence of ovarian stroma and absence of bile duct communication. CASE PRESENTATION: Here, we discuss a 45-year-old woman who presented with symptomatic liver mass. Diagnostic workup detected a 4.2 × 3.6 cm septate cyst located in segments I, V, and VIII of the liver in communication with the right hepatic duct. An open right liver resection with total bile duct excision and hilar lymphadenectomy was performed. Pathology revealed a multiloculated cyst with lined mucinous epithelium and ovarian-like stroma, consistent with low-grade MCN-L. CONCLUSIONS: This case shows that unusual location and bile duct communication can be present in MCN-L.

Liver resections for metastasis: surgical outcomes of a single center academic institution.

BACKGROUND: Hepatic metastasis are frequent and liver resection may be an option for some cases, despite the high complexity of the procedure and the possibility of postoperative complications. METHODS: This retrospective comparative descriptive study aims to evaluate a series of 86 consecutive liver resections (LRs) performed for the treatment of metastatic liver tumors, comparing the results between patients undergoing major and minor LR. All patients submitted to LR from October 2010 to July 2015 at the Erasto Gaertner Hospital in Curitiba-PR were included. Quantitative numerical variables were analyzed with the Student t-test. The nonparametric Mann-Whitney U test was used for numerical variables of non-normal distribution. Categorical variables were analyzed with the Chi-square test with Fisher's correction. The data were analyzed with the SPSS 23.0 and STATA 15 programs, being p < 0.05 considered statistically significant. RESULTS: Eighty-six LR were performed, 56 cases by colorectal metastasis. The major LR corresponded to 68 cases, with 13.2% of Clavien-Dindo III-V complications and 2.9% of reoperation rate. Eighteen minor LR were performed and one patient had a postoperative complication requiring reoperation. CONCLUSION: Preoperative elevation of transaminases and jaundice negatively influence surgical outcomes in patients undergoing LR. Tumors greater than 3 cm presented worse postoperative survival. Major LR did not significantly increase the surgical morbidity rate. INSTITUTIONAL REVIEW BOARD REGISTRATION: 1.122.319/2015.

Torsion of a giant pedunculated liver hemangioma: Case report.

INTRODUCTION: Hepatic hemangioma (HH) is the most common benign neoplasm affecting the liver. Most cases are asymptomatic and incidentally found at imaging studies. Giant hepatic hemangiomas are uncommon, but pedunculated giant hemangiomas are even rarer, making their clinical and imaging diagnosis a challenge for the surgeon. PRESENTATION OF CASE: Herein; we report the case of a 26-year-old male patient, who presented to the emergency ward with acute abdominal pain associated with a palpable mass, mimicking a complicated appendicular plastron, additionaly were found it liver enzymes elevated. Then the patient was taken the operating room. An exophytic pedunculated liver tumor that projected towards the right iliac fossa was identified. It had undergone torsion on its pedicle. Transfixing hemostatic sutures were placed, proceeding with its exeresis. The patient tolerated the procedure and was discharged without complications on the third postoperative day. Pathological anatomy confirmed the diagnosis. DISCUSSION: Giant pedunculated HH is an infrequent entity that generates mass effect on intra-abdominal organs causing pain depending on its location. In our case, HH size was 13 cm with elevated liver enzymes, only previously reported in one case of a 20-cm hemangioma. Surgery is indicated for cases of giant and symptomatic lesions. CONCLUSION: Torsioned pedinculated giant liver hemangioma may be part of the differential diagnosis of a patient with acute appendicitis with elevated liver enzymes. The elevation of liver enzymes may be related to the size of the HH.

Childhood liver tumors in Argentina: Incidence trend and survival by treatment center. A report from the national pediatric cancer registry, ROHA network 2000-2015.

BACKGROUND: Information on the epidemiology of pediatric liver tumors in Latin America is limited. PURPOSE: To describe the incidence of liver tumors in a pediatric registry in Argentina according to geographic region, national trends over 16 years, and survival related to stage, age, sex, and care center. METHODS: Newly diagnosed liver tumors cases are registered in the Argentine Pediatric Oncology Hospital Registry (ROHA) with an estimated coverage of 91% of national cases. Age-standardized incidence rate per millon (ASR) was calculated based on the National Vital Statistics Reports. Five-year overall survival (OS) was estimated using the Kaplan-Meier method. The log-rank test was used to compare subgroup survival. RESULTS: Two hundred seven cases of hepatoblastoma (HB) and 73 of hepatocellular carcinoma (HCC) were identified. ASR of liver tumors was 1.8/million (95% confidence Interval [CI], 1.6-2.0) per year. ASR was 1.4 (1.2-1.6) for HB and 0.4 (0.3-0.5) for HCC. For HB, the highest incidence was found in the northwest region including the Altiplano. OS was 60.4% (53.4-66.8) for HB and 36.1% (25.2-47.2) for HCC. Five-year survival rate of children with metastatic HB treated at liver transplant hospitals (LTH) was 54.2% (30.3-73.0) compared to 13.3% (2.2-34.6) for those seen at other hospitals (OH) (P = .02), while for HCC this rate was 46.3% (30.7-60.6) at LTH compared to 17.5% (3.1-41.9) at OH (P = .01). CONCLUSIONS: The incidence rate of pediatric liver tumors was stable over the 16-year study period. Patients may benefit if at treatment initiation they are evaluated jointly with LTH specialists to define treatment strategies.

Hepatoblastoma del adulto: presentación de un caso / A case of adult hepatoblastoma

Resumen Fundamento: el hepatoblastoma del adulto (HBA) es un tumor hepático poco frecuente y con un mal pronóstico, lo cual contrasta con el hepatoblastoma infantil (HBI). Esta patología aún no ha sido comprendida completamente y hasta la fecha, no se han reportado de forma adecuada más de 50 casos en la literatura médica. Objetivo: presentar el caso de un paciente que fue egresado de nuestro hospital con el diagnóstico de carcinoma hepatocelular, aproximadamente con 3 meses de anterioridad. Caso clínico: paciente masculino de 60 años con historia de alcoholismo y de ser un fumador inveterado. Fue ingresado en nuestro hospital por dolor abdominal, en el momento del examen físico, puso de manifiesto un tumor palpable en la región del hipocondrio derecho. Este paciente había sido egresado aproximadamente 3 meses atrás, con el diagnóstico de carcinoma hepatocelular, en el curso de una cirrosis hepática. El hombre falleció por causa de la progresión de la enfermedad y la autopsia reveló la existencia de un HBA. Conclusiones : el HBA es un tumor infrecuente, de grave pronóstico y muchos casos son asintomáticos hasta el momento del diagnóstico. Dicho tumor, por lo regular, presenta un gran tamaño. Las enzimas hepáticas, la alfafetoproteína y los estudios imagenológicos revelan el diagnóstico de un hepatocarcinoma, el cual es un tumor frecuente en los adultos. Asimismo, el estudio histológico confirma el diagnóstico. Debido a su mal pronóstico, y a las mejores perspectivas de tratamiento en niños, hoy en día es lógico utilizar el tratamiento pediátrico en los adultos. Se necesitan más estudios de investigación para el manejo óptimo del HBA.

Abstract Background: In contrast to childhood hepatoblastoma, adult hepatoblastoma (HBA) is a rare and not-fully-understood liver tumor with a poor prognosis. To date, about 50 cases have been adequately reported in the medical literature. Objective: We present the case of a patient who was discharged from our hospital with a diagnosis of hepatocellular carcinoma approximately 3 months before returning. Clinical case: A 60-year-old male patient with a history of alcoholism and heavy smoking was admitted to our hospital for abdominal pain. Physical examination revealed a palpable tumor in the right hypochondrium region. This patient had been discharged approximately 3 months previously with a diagnosis of hepatocellular carcinoma in the course of liver cirrhosis. The patient died, and the autopsy revealed an HBA. Conclusions: Adult hepatoblastoma is an infrequent tumor with a severe prognosis. Many cases are asymptomatic until the time of diagnosis, and the tumor is usually very large. Liver enzymes, alpha-fetus protein, and imaging studies lead to a diagnosis of hepatocellular carcinoma which is a common tumor in adults. Histological study confirms the diagnosis. Due to the poor prognosis for HBA in contrast to better prospects for treatment of hepatoblastoma in children, it is logical to use pediatric treatment in adults. More research is needed for the optimal treatment of HBA.

Effect of Portal Vein Ligation Plus Venous Congestion on Liver Regeneration in Rats.

INTRODUCTION AND AIM: We developed a rat model of portal vein ligation (PVL) with venous congestion (PVL+C) to investigate beneficial effect PVL plus congestion for regeneration of intact liver segments. MATERIALS AND METHODS: In the PVL group, portal vein branches were ligated except the caudate lobe (CL). In the PVL + C group, the left lateral hepatic vein was ligated in addition to PVL. Chronological changes in the following variables were compared among the groups: CL weight to body weight ratio (CL/BW), embolized liver weight to body weight ratio (EL/BW), histological findings of the embolized/non-embolized liver, and expression of several mediators that affect liver regeneration in the non-embolized liver. RESULTS: Weight regeneration of CL continued up to postoperative day (POD)7 in PVL + C, but terminated at POD2 in PVL. CL/BW at POD7 was significantly higher in PVL + C than in PVL (2.41 ± 0.33% vs. 1.22 ± 0.18%, P < 0.01). In contrast, EL/BW continued to decrease up to POD7 in PVL + C but reached nadir at POD2 in PVL. Furthermore, EL/BW at POD7 was significantly smaller in PVL + C than in PVL (0.35 ± 0.03% vs. 0.67 ± 0.08%, P < 0.01). Histologically-proven injury in the embolized liver was more severe in PVL + C than in PVL. Expression of Ki-67, IL-6, TNF -a, and HGF were greater and/or more prolonged in PVL + C than in PVL. CONCLUSIONS: Our rat model of PVL + C was considered useful for investigating the beneficial effect of congestion in addition to PVC. PVL + C caused increased devastation of the embolized liver, and higher and more prolonged expression of factors promoting liver regeneration in the non-embolized liver than in PVL.

Hemangioendotelioma epitelioide hepático de presentación como lesión focal incidental única: reporte de caso / Hepatic epithelioid hemangioendothelioma of presentation as single focal incidental lesion: case report

El hemangioendotelioma epitelioide hepático (HEH) es un tumor vascular maligno poco frecuente, de origen endotelial, de lenta progresión y de bajo grado de malignidad. Suele presentarse con mayor compromiso en mujeres, sin una etiología conocida. La clínica es variable, desde casos asintomáticos hasta dolor abdominal. Las características imagenológicas más frecuentes son lesiones nodulares hepáticas coalescentes, de tamaño variable, que muestran apariencia de "lesión en diana" en tomografía computarizada (TC) y resonancia magnética (RM), con retracción capsular. El principal diagnóstico diferencial son las metástasis hepáticas. El curso evolutivo es variable e imprevisible, con una mortalidad posterior al diagnóstico de hasta el 50% de los pacientes. Las modalidades terapeúticas incluyen la extirpación quirúrgica de la lesión y el trasplante hepático como opciones más útiles, además de la quimioterapia regional/sistémica y tratamiento inmunológico. El presente caso describe las principales características clínico-patológicas de este raro tumor, con la particularidad de su forma de presentación como lesión focal incidental única, además de la modalidad terapéutica utilizada (extirpación quirúrgica) en este tipo de lesión.

Hepatic epithelioid hemangioendothelioma (HEH) is a very rare malignant vascular tumor of endothelial origin, of slow progression and low malignity degree. It is more common in women, of unknown etiology. Clinical manifestations of HEH are non-specific, from asymptomatic to abdominal pain. The more frequent radiological features are nodular hepatic lesions, of variable size, which show appearance of "target sign" in computed tomography (CT) and magnetic resonance imaging (MRI), with capsular retraction. Hepatic metastases are the principal differential diagnosis. The clinical course is variable, with a mortality rate to the diagnosis of up to 50 % of the patients. The therapeutical options include the surgical extirpation (removal) of the tumor and hepatic transplant as the more useful, besides the regional /systemic chemotherapy and immunological treatment. The present case describes the principal clinical pathological characteristics of this rare tumor, with the particularity of its form of presentation as focal lesion, besides the therapeutical modality used (surgical removal) in this type of illness.

Angiosarcoma hepático idiopático y metástasis cardíaca / Idiopathic Hepatic Angiosarcoma and Heart Metastasis

El angiosarcoma hepático es una neoplasia poco frecuente cuya incidencia se reporta en el 0,5%-2% de las tumoraciones hepáticas primarias. El diagnóstico es tardío cuando el órgano está comprometido y no es posible la terapéutica quirúrgica; la mayoría es de naturaleza idiopática (75%) y las manifestaciones clínicas y radiológicas son inespecíficas. El diagnóstico es fundamentalmente por estudio histopatológico. Se presenta el caso de un paciente masculino de 34 años, sin antecedentes de interés, con un tiempo de evolución de su enfermedad de 4 meses, que se presentó con tumoración hepática, síntomas inespecíficos y alteraciones cardiovasculares que ocasionaron la muerte por taponamiento cardíaco. Se realizó necropsia y el estudio histopatológico mostró angiosarcoma hepático con metástasis cardíaca y pleural.

Hepatic angiosarcoma is a rare malignancy whose incidence is reported to be from 0.5% to 2% of primary liver tumors. Late diagnosis is defined as diagnosis after the liver has been compromised and surgical therapy is no longer possible. Most hepatic angiosarcomas (75%) are idiopathic, and clinical and radiological findings are nonspecific. Diagnosis is mainly by histopathology. We report the case of a 34-year-old male patient with no history of interest who had had a liver tumor for four months without specific symptoms. Cardiovascular disturbances led to death by cardiac failure. An autopsy and histopathology revealed liver angiosarcoma that had metastasized to the heart and pleural cavity.

Evaluación de resecciones hepáticas en el Hospital Coromoto de Maracaibo: experiencia en 3 años / Evaluation of liver resections at the Coromoto Hospital in Maracaibo: 3-year experience

Evaluar resecciones hepáticas en la Unidad de Cirugía Hepatobiliar-pancreática del Hospital Coromoto de Maracaibo en 3 años. Métodos Estudio retrospectivo, descriptivo de 43 resecciones hepáticas (convencional ­laparoscópica), periodo enero 2013 - enero 2015 por tumores hepáticos (benignos- malignos), excluyendo lesiones quísticas, infecciosas, traumáticas, seropositivos, insuficiencia hepática. Se evaluó el grupo etario y sexo; histología, tamaño y ubicación del tumor, resección hepática, transfusiones, tiempo quirúrgico, estancia y complicaciones. Resultados: Se realizaron 30 resecciones hepáticas convencionales (RHC) y 12 resecciones hepáticas laparoscópicas (RHL). En ambos grupos predominó el sexo femenino 66,66 %, sin diferencias significativas en grupo etario (p= 0,9); ni tamaño del tumor (p=0,3). El tipo histológico predominante fueron lesiones malignas 66,66%, tumores metastásicos los más frecuentes 13 casos (30,95%), seguidos del carcinoma hepatocelular 11 casos (26,19%). Promedio de hospitalización 4,5 días para RHL y 8,12 días para RHC. Necesidad de transfusión 50% en RHL y 76,66% en RHC. Las complicaciones en RHL fue 8,33% (1 caso) no relacionada a la patología y 10% en RHC (3 casos) relacionadas a la patología, falleciendo 2 pacientes. No se evidenció diferencia en tiempo quirúrgico: 4,66 horas RHC y 4,86 horas RHL. Conclusión: La patología más frecuente fue tumores hepáticos metastasicos. La cirugía hepática laparoscópica ofrece ventajas por menores pérdidas hemáticas y menor necesidad de hemoderivados así como menor estancia hospitalaria determinando recuperación más rápida, sin diferencia en tiempo quirúrgico(AU)

To evaluate liver resections in the Hepatobiliarypancreatic Surgery Unit at Hospital Coromoto of Maracaibo in 3 years. Methods: A retrospective, descriptive study of 43 hepatic resections (conventional or laparoscopic), period January 2013 - January 2015 by liver tumors (benign - malignant), excluding infectious, traumatic, cystic lesions HIV, liver failure. Assessed the group age and sex; histology, size and location of the tumor, liver resection, transfusions, surgical time, stay and complications. Results: 30 conventional liver resections (RHC) and 12 resections performed laparoscopic (RHL). Both groups dominated the female 66,66 %, without significant differences in group age (0.9 P); or size of the tumor (0.3 P). The predominant histologic type were malignant lesions 66,66 %, metastatic tumors were the most frequent 13 (30,95%) cases, followed by hepatocellular carcinoma 11 cases (26.19 %). Average of 4.5 days for RHL and 8,12 RHC days hospitalization. Need transfusion 50% on RHL and 76,66% at RHC. Complications in RHL was 8.33% (1 case) not related to pathology and 10% related to the pathology RHC (3 cases), 2 patients dying. Showed no difference in surgical time: 4.66 hours RHC and 4.86 hours RHL. Conclusion: The most frequent pathology was metastatic liver tumors. Laparoscopic liver surgery offers advantages for minor losses blood and less need for blood products as well as lower hospital stay determining faster recovery, no difference in surgical time(AU)

Hepatectomía derecha extendida con reconstrucción vascular y biliar como tratamiento del hepatocarcinoma fibrolamelar con trombosis portal tumoral e infiltración biliar. Reporte de un caso / Right extended hepatectomy with vascular and biliary reconstruction for fibrolamelar hcc with portal vein thrombosis and biliary infiltration. Case report

El hepatocarcinoma fibrolamelar, es un tumor de ocurrencia esporádica, que se desarrolla sobre la base de un hígado sano, en ausencia de hepatitis viral, alteraciones metabólicas o algún tipode condición inflamatoria en el hígado. Alcanza una supervivencia de hasta un 70% en 5 años en sujetos sometidos a cirugía con márgenes libres de enfermedad y en aquellos en estadios iniciales.Caso clínico: Paciente masculino de 14 años de edad, con clínica de ictericia y aumento de volumen en hipocondrio derecho, estudios de imagen demuestran lesión tumoral de 16 cm que ocupa los segmentos 1,5,6,7, con trombosis tumoral en porta derecha y tronco principal, marcada dilatación de vías biliares. Se practica hepatectomía derecha extendida al lóbulo caudado, con reconstrucción vascular de la vena porta (resección y anastomosis termino-terminal entre el tronco principal y la porta izquierda), resección en bloque de la vía biliar con reconstrucción biliar tipo colangioyeyuno anastomosis al hepático izquierdo. Tiempo quirúrgico 390 minutos, pérdidas sanguíneas estimadas en 1800 ml, estancia en terapia intensiva de 7 días, complicación grado III-A deClavien por colección subdiafragmática y derrame pleural derecho,estancia hospitalaria de 21 días. Resultado histopatológico: hepatocarcinoma fibrolamelar, márgenes de resección libres, trombosis tumoral en vena porta, infiltración tumoral simulando trombosis tumoral en la vía biliar principal y hepático derecho, márgenes de vía biliar libres de enfermedad neoplásica, ausencia de metástasis ganglionar. Recibió adyuvancia con gemcitabina y oxaloplatino (6 ciclos), actualmente 16 meses de seguimiento libre de enfermedad con vida sana(AU)

The fibrolamellar hepatocellular carcinoma it is a rare liver tumor,who growth in normal liver parenquima, in absent of viral hepatitis,metabolic liver disorders or any inflammatory condition, the long term survival can reach up to 70% in 5 years.Clinic case: A 14 years old male, with ictericia, abdominal growth circumference, the US and Abdominal CT revealed: 16cm liver tumor in segments 1,5,6,7, with tumoral thrombus in right portalve in and main trunk, and intrahepatic biliary dilatation. Surgical treatment: Extended right hepatectomy to segment 1, with vascular and biliary reconstruction (resection and anastomosis of portal vein). Operative time: 390 minutes, blood looses 1800 ml, ICU 7 days, total hospital stay 21 days, Clavien III-A complications(abdominal abscess and pleural effusion). Pathological results: Fibrolamellar hepatocellular carcinoma, free margin. Adjuvant therapy based on gemcitabine and oxaloplatin (6 cylcles), actually he is 16 months of follow up, disease free and with a normal life(AU)

Tumores hepáticos malignos no hepatocelulares. 2.a parte. Aspectos morfológicos y estudios auxiliares de importancia útiles para el diagnóstico histopatológico / Malignant Hepatic Neoplasms. Part 2. Morphology and important ancillary studies useful for histopathological diagnosis

Se continúa con la revisión de los tumores malignos hepáticos primarios. Se hablará de los aspectos más importantes de los tumores primarios no hepatocelulares, siendo más frecuentes los originados en el epitelio del ducto biliar como el colangiocarcinoma, el cistoadenocarcinoma y los tumores mesenquimales, y los más infrecuentes el hemangioendotelioma epitelioide y el angiosarcoma. La principal dificultad radica en lograr el diagnóstico definitivo, el cual se basa en la exclusión de una neoplasia primaria extrahepática o de lesiones hepáticas benignas. Estudios adicionales de inmunohistoquímica, estudios de clonalidad o moleculares pueden ser de mucha utilidad.

We continue with the review of primary malignant liver tumors. This article covers the most important aspects of primary tumors that are not hepatocellular. Those that originate in the epithelium of the bile duct such as cholangiocarcinoma, cystadenocarcinoma and mesenchymal tumors occur most frequently. Epithelioid hemangioendothelioma and angiosarcoma occur less frequently. The main difficulty lies in making a definitive diagnosis which must be based on the exclusion of extrahepatic primary neoplasms and benign liver lesions. Additional immunohistochemistry and molecular studies as well as diagnostic cloning of cells can be very useful.

New concepts and outcomes for children with hepatoblastoma based on the experience of a tertiary center over the last 21 years

OBJECTIVE: The aim of this study was to summarize the experience of a tertiary center in treating hepatoblastoma for the last 21 years. PATIENTS AND METHODS: Fifty-eight cases were included. The tumor extent and prognosis were assessed using the PRETEXT system. The following data were analyzed: age at diagnosis, comorbidities, prematurity, treatment modalities, histopathological findings, surgical details and complications, treatment outcomes, chemotherapy schedules, side effects and complications. Treatment outcomes included the occurrence of local or distant recurrence, the duration of survival and the cause of death. The investigation methods were ultrasonography, CT scan, serum alpha-fetoprotein level measurement and needle biopsy. Chemotherapy was then planned, and the resectability of the tumor was reevaluated via another CT scan. RESULTS: The mean numbers of neoadjuvant cycles and postoperative cycles of chemotherapy were 6±2 and 1.5±1.7, respectively. All children except one were submitted for surgical resection, including 50 partial liver resections and 7 liver transplantations. Statistical comparisons demonstrated that long-term survival was associated with the absence of metastasis (p=0.04) and the type of surgery (resection resulted in a better outcome than transplantation) (p=0.009). No associations were found between vascular invasion, incomplete resection, histological subtype, multicentricity and survival. The overall 5-year survival rate of the operated cases was 87.7%. CONCLUSION: In conclusion, the experience of a Brazilian tertiary center in the management of hepatoblastoma in children demonstrates that long survival is associated with the absence of metastasis and the type of surgery. A multidisciplinary treatment involving chemotherapy, surgical resection and liver transplantation (including transplantations using tissue from living donors) led to good outcomes and survival indexes. .

Neoplasias hepáticas malignas: Hepatocarcinoma: papel de la biopsia hepática, estudios de inmunohistoquímica y otros aspectos importantes / Malignant Hepatic Neoplasms: Hepatocellular carcinoma: the roles of liver biopsies and immunohistochemical studies and other important issues

Continuamos con la revisión de los tumores hepáticos. Nos referiremos a los tumores hepáticos primarios malignos más frecuentes. Tal como lo mencionamos anteriormente, los tumores malignos hepatobiliares son un espectro de tumores invasivos que reciben su nombre dependiendo de su célula de origen. De acuerdo con la Agencia Internacional para el Estudio del Cáncer (IARC), los tumores hepáticos malignos constituyen la segunda causa de muerte en el mundo, con aproximadamente 745 000 muertes/año (1). Al igual que en el artículo anterior, el objetivo es identificar aquellos aspectos más relevantes de estas neoplasias, en especial los hallazgos morfológicos que generan mayor problema diagnóstico en la utilidad de los estudios de inmunohistoquímica y sus diagnósticos diferenciales. La primera parte de esta revisión se centrará en el hepatocarcinoma, el más frecuente de los tumores hepáticos epiteliales malignos, algunas de sus variantes y sus lesiones precursoras. También en el controvertido papel de la biopsia hepática para su diagnóstico.

We continue with our review of liver tumors in which we will refer to the most common primary malignant liver tumors. As mentioned previously, hepatobiliary malignancies are a spectrum of invasive tumors whose names depend on their origins. According to the International Agency for Research on Cancer (IARC), malignant liver tumors are the second leading cause of death worldwide with approximately 745,000 deaths/year. (1) As in the previous article, the aim here will be to identify the most relevant aspects of these neoplasms. In particular we will look at morphological findings that generate major diagnostic problems and at the usefulness of immunohistochemical studies and their differential diagnoses. Hepatic carcinoma, the most frequent malignant epithelial tumor of the liver is the focus of the first part of this review. We will look at some of its variants and its precursor lesions, and at the controversial role of liver biopsies for diagnosis.