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.

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.

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.

Hematoma subcapsular del hígado: morfología y resultados del tratamiento quirúrgico / Hepatic subcapsular hematoma: morphology and results of surgical treatment

RESUMEN: El hematoma subcapsular del hígado (HSH), es una entidad poco frecuente. La mayoría de casos reportados se asocian al embarazo, en el contexto de síndrome de recuento bajo de plaquetas, elevación de enzimas hepáticas, y hemólisis. Otras patologías relacionadas son ruptura de carcinoma hepatocelular, adenoma, hiperplasia nodular focal; y amilosis. El objetivo de este artículo fue reportar morfología y resultados del tratamiento quirúrgico observados en una serie de pacientes con HSH, en términos de morbilidad postoperatoria (MPO). Serie de casos retrospectiva de pacientes con HSH intervenidos de forma consecutiva en Clínica RedSalud Mayor Temuco, entre 2004 y 2019. La variable resultado fue MPO. Otras variables de interés fueron edad, sexo, etiología, diámetro, necesidad de transfusión y tiempo de hospitalización. Se utilizó estadística descriptiva. Se trató a 7 pacientes (71,4 % mujeres), con una mediana de edad de 46 años. La mediana del diámetro del HSH, tiempo quirúrgico y estancia hospitalaria fueron 11 cm, 105 min y 5 días, respectivamente. No hubo necesidad de reintervenciones. Con una mediana de seguimiento de 31 meses, no se verificó MPO ni mortalidad. El HSH es una condición poco frecuente, y la evidencia disponible escasa. Puede asociarse a condiciones benignas y malignas. Requiere un alto índice de sospecha para un diagnóstico oportuno.Su pronóstico depende de la etiología.

SUMMARY: Subcapsular hematoma of the liver (SHL) is a rare entity. The majority of cases are associated with pregnancy, in the context of low platelet count syndrome, elevated liver enzymes, and hemolysis. Other related pathologies are ruptured hepatocellular carcinoma, adenoma, focal nodular hyperplasia, and amyloidosis. The aim of this article was to report morphology and results of the surgical treatment observed in a series of patients with SHL, in terms of postoperative morbidity (POM). Retrospective case series of patients with SHL who were intervened consecutively at Clínica RedSalud Mayor Temuco, between 2004 and 2019. The results variable was MPO. Other variables of interest were age, sex, etiology, diameter, need for transfusion, and length of hospitalization. Descriptive statistics were used; 7 patients (71.4% women) were treated with a median age of 46 years. The median diameter of SHL, surgical time, and hospital stay were 11 months, no POM and mortality were verified. SHL is a rare condition, and the available evidence is scarce. It can be associated with benign and malignant diseases. It requires a high index of suspicion for a timely diagnosis. Its prognosis depends on the etiology.

ALPPS como opción quirúrgica en terapia reversa aplicado al tratamiento de las metástasis hepáticas del cáncer rectal sincrónico / ALPPS using liver-first reverse approach as a surgical option to treat synchronous colorectal liver metastases

RESUMEN El riesgo de insuficiencia hepática posoperatoria es la limitante de mayor importancia para el trata miento de pacientes con tumores hepáticos malignos primarios o secundarios. Entre las diferentes técnicas para incrementar la resecabilidad de tumores hepáticos se desarrolló una estrategia para pa cientes con tumores previamente considerados como irresecables, técnica conocida como ALPPS (as sociating liver partition with portal vein ligation for staged hepatectomy). Informamos acerca de una cirugía ALPPS en terapia reversa en un hombre referido a nuestro centro con diagnóstico sincrónico de cáncer rectal con metástasis hepáticas múltiples consideradas irresecable al momento del diagnóstico.

ABSTRACT The risk for postoperative liver failure is the most important limitation for the treatment of patients with primary or secondary liver cancer. Among the different strategies used to increase resectability in liver tumors, a technique known as ALPPS (associating liver partition with portal vein ligation for staged hepatectomy) was developed for patients with tumors previously considered unresectable. We report the case of a male patient referred to our center with a diagnosis of synchronous multiple liver metastases of colorectal cancer considered unresectable who underwent ALPPS using liver-first reverse approach.

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.

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)

Tumor fibroso solitario del lóbulo caudado del hígado / Solitary fibrous tumor of the liver caudate lobe

Introduction: Solitary fibrous tumor of the liver (SFTL) constitutes such a rare finding that it has been published only as case reports and to the present date there are only 45 cases published in English medical literature. This article describes the case of a patient treated at our institution with SFTL. Case report: A male 59-years-old patient was diagnosed with a SFTL incidentally found in a computed abdominal tomography taken by another causes, tumor was originating from the caudate lobe. The patient was asymptomatic. At surgery, the tumor was observed originating directly from the caudate lobe through a pedicle measuring approximately 3 cm. The tumor was resected sectioning its implantation pedicle and ligating all arterial, venous and biliary vessels. The SFTL was well encapsulated, measured 15 x 9 x 6 cm and weighted 794 g. The histological diagnosis was a SFTL and was confirmed by immunohistochemistry with monoclonal antibodies that reacted positively to CD34 and vimentin and negatively to CD117, S100, smooth-muscle α-actin and desmin. Conclusions: SFTL is an uncommon tumor. The present case is the first reported in Chilean medical literature and presented all the habitually described radiologic, surgical and pathological characteristics.

Introducción: El tumor fibroso solitario del hígado (TFSH) constituye un hallazgo tan raro que se ha publicado únicamente como reportes de caso y hasta el presente se encontraron sólo 45 casos publicados en la literatura inglesa. El presente artículo describe el caso de un paciente que fue tratado en nuestra institución por un TFSH. Reporte de caso: Paciente masculino de 59 años de edad al que, en una tomografía abdominal computarizada que se tomó por otras causas, se encontró en forma incidental un gran tumor hepático localizado en el lóbulo caudado del hígado. El paciente no refería síntomas relacionados con el tumor. En la cirugía se observó que el tumor se originaba directamente del lóbulo caudado al cual lo unía un pedículo de aproximadamente 3 cm de diámetro transverso. Se seccionó el tumor desde su base de implantación en el hígado ligándose los vasos arteriales y venosos así como los conductos biliares que se encontraron. Este medía 15 x 9 x 6 cm y pesaba 794 g, se encontraba bien encapsulado. El diagnóstico histológico fue el de un TFSH, el cual se confirmó mediante inmunohistoquímica con anticuerpos monoclonales que resultaron positivos para CD34 y vimentina en las células tumorales y negativos para CD117, S100, α-actina de músculo liso y desmina. Conclusiones: El TFSH es un tumor raro. El presente caso de un TFSH es el primero reportado en la literatura médica chilena y presentó las características radiológicas, quirúrgicas y patológicas habitualmente descritas en estos casos.

Brazilian society of hepatology recommendations for the diagnosis and treatment of hepatocellular carcinoma

ABSTRACT Hepatocellular carcinoma is a malignancy of global importance and is associated with a high rate of mortality. Recent advances in the diagnosis and treatment of this disease make it imperative to update the recommendations on the management of the disease. In order to draw evidence-based recommendations concering the diagnosis and management of hepatocellular carcinoma, the Brazilian Society of Hepatology has sponsored a single-topic meeting in João Pessoa (PB). All the invited pannelists were asked to make a systematic review of the literature and to present topics related to the risk factors for its development, methods of screening, radiological diagnosis, staging systems, curative and palliative treatments and hepatocellular carcinoma in noncirrhotic liver. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript containing the recommendations of the Brazilian Society of Hepatology.

RESUMO O carcinoma hepatocelular é uma neoplasia de importância global e associada a altos índices de mortalidade. Recentes avanços no diagnóstico e tratamento da doença tornaram necessárias que se atualizassem as recomendações sobre o manejo da doença. Para definir as recomendações sobre o diagnóstico e tratamento do carcinoma hepatocelular, a Sociedade Brasileira de Hepatologia organizou uma reunião monotemática em João Pessoa (PB). Todos expositores foram solicitados a fazer uma revisão sistemática da literatura e apresentar os temas relacionados a fatores de risco para o desenvolvimento de carcinoma hepatocelular, métodos para rastreamento, diagnóstico radiológico e sistemas de estadiamento da doença, tratamentos curativos e paliativos e carcinoma hepatocelular em fígado não cirrótico. Após o encontro, todos os expositores se reuniram para discussão dos tópico e elaboração dessas recomendações. O texto resultante foi ainda submetido a avaliação e aprovação por todos membros da Sociedade através de sua homepage. O documento atual é a versão final que contêm as recomendações da Sociedade Brasileira de Hepatologia.

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.

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.

Neoplasmas em suínos: 37 casos / Neoplasia in swine: 37 cases

Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína.

Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.

Neoplasias hepáticas benignas: variantes histológicas, problemas diagnósticos y diagnóstico diferencial / Diagnosis and Differential Diagnosis Problems with Histological Variants of Benign Liver Neoplasms

Un principio básico de la patología es que las neoplasias se diferencian según sus células de origen y en el hígado semejan sus constituyentes, sean las células hepáticas, del epitelio biliar, endoteliales, mesenquimales o una combinación de estas. Es importante recordar aquí que son las metástasis el tumor maligno más frecuente del hígado, con una relación de 30:1 en pacientes sin enfermedad hepática crónica o cirrosis subyacente; es rara la presencia de las mismas en hígados cirróticos. Las neoplasias gastrointestinales del colon, páncreas, vía biliar extrahepática, estómago, tumores neuroendocrinos y GIST, o extraintestinales del pulmón, mama, melanoma o tumores de cabeza y cuello, son las más frecuentes (1). En este artículo solo revisaremos las más frecuentes. Iniciaremos con las neoplasias benignas y las lesiones pseudotumorales haciendo especial énfasis en aquellas con dificultades diagnósticas, en la utilidad de estudios especiales de inmunohistoquímica o moleculares para su adecuada clasificación y diagnóstico diferencial.

A basic principle of pathology is that neoplasms differ according to their cells of origin. Neoplasms of the liver resemble its constituent liver, biliary, epithelial, endothelial, mesenchymal cells or some combination of these different types of cells. It is important to remember here that metastases are the most frequent malignant liver tumor occurring at ratio of 30: 1 in patients without underlying chronic liver disease or cirrhosis. Metastases are rare in cirrhotic livers. The most common primary sites are the colon, pancreas, common bile duct, stomach, neuroendocrine tumors and GIST, or extraintestinal tumors from the lung, breast, head, neck and skin (1). In this article we review only the most frequent benign neoplasms and tumor-like lesions with particular emphasis on diagnostic difficulties, special studies, and the usefulness of immunohistochemical or molecular studies for proper classification and differential diagnosis.

Hemangioendotelioma epitelioide hepático: un desafío diagnóstico para el médico radiólogo / Hepatic epithelioid hemangioendothelioma: a diagnostic challenge for the radiologist

Presentamos el caso de una paciente derivada a nuestra institución por presentar un cuadro clínico de dolor abdominal de más de un año de evolución, asociado al hallazgo de múltiples lesiones hepáticas sólidas, sugestivas de metástasis. Tras la biopsia percutánea, se diagnosticó hemangioendotelioma epitelioide hepático. Describimos las características clínico-imagenológicas de este tumor, aportando un nuevo caso de hemangioendotelioma epitelioide hepático, y analizamos los distintos hallazgos en los métodos de estudio por imágenes. Dado que se trata de una entidad poco frecuente en la que hay que pensar cuando se detectan lesiones hepáticas múltiples, es importante reconocerla e incluirla en el diagnóstico diferencial de las metástasis hepáticas.

We report a case of a patient referred to our institution presenting with 1 year of abdominal pain, associated to the finding of multiple solid liver lesions suggestive of metastases. Diagnosis of hepatic epithelioid hemangioendothelioma was performed after percutaneous liver biopsy.We describe clinical and imaging features of this tumor. Imaging findings are analized, providing a new case of hepatic epithelioid hemangioendothelioma. Since this is a rare entity that must be taken into account when multiple liver lesions are detected, it is important to recognize it and include it in the differential diagnosis of liver metastases.

Hepatectomy: a critical analysis on expansion of the indications / Hepatectomia: uma analise critica da ampliacao das indicacoes

Background: Hepatic resection has evolved to become safer, thereby making it possible to expand the indications. Aim : To assess the results from a group of patients presenting these expanded indications. Method: Were prospectively studied all the hepatectomy procedures performed for hepatic tumor resection. Patients with benign and malignant primary and secondary tumors were included. Were included variables such as age, gender, preoperative diagnosis, preoperative treatment, type of operation performed, need for transfusion, final anatomopathological examination and postoperative evolution. The patients were divided into two groups: group A, with a traditional indication for hepatectomy; and group B, with an expanded indication (tumors in both hepatic lobes, extensive resection encompassing five or more segments, cirrhotic livers and postoperative chemotherapy using hepatotoxic drugs). Results: Were operated 38 patients, and 40 hepatectomies were performed: 28 patients in group A and 10 in group B. The mean age was 57.7 years, and 25 patients were women. Three in group B were operated as two separate procedures. Groups A and B received means of 1.46 and 5.5 packed red blood cell units per operation, respectively. There were three cases with complications in group A (10.7%) and six in group B (60%). The mortality rate in group A was 3.5% (one patient) and in groups B, 40% (four patients). The imaging examinations were sensitive for the presence of tumors but not for defining the type of tumor. The blood and derivative transfusion rates, morbidity and mortality were greater in the group with expanded indications and more extensive surgery. Conclusion: The indications for liver biopsy and portal vein embolization or ligature can be expanded, with special need of cooperation of the anesthesiology department and the use of hepatic resection devices to diminish blood transfusion. .

Racional: As ressecções hepáticas evoluíram, tornando-se mais seguras, permitindo ampliação das indicações. Objetivo : Avaliar os resultados com ampliação da indicação. Método : Estudo prospectivo de todas as hepatectomias realizadas para ressecção de tumores hepáticos. Foram incluídos pacientes com tumores primários e secundários, benignos e malignos e anotados idade, gênero, diagnóstico pré-operatório, tratamento realizado no pré-operatório, tipo de operação realizada, necessidade de transfusão, exame anatomopatológico final e evolução pós-operatória. Os pacientes foram divididos em dois grupos: grupo A, aqueles com indicação tradicional de hepatectomia e grupo B, com indicação ampliada (tumor nos dois lobos hepáticos, ressecções extensas de cinco ou mais segmentos, fígados cirróticos e pós-quimioterapia com drogas hepatotóxicas). Resultados: Foram operados 38 pacientes e realizadas 40 operações, 28 no grupo A e 10 no B. A média de idade foi 57,7 anos, sendo 25 mulheres. Três do grupo B foram operados em dois tempos. Os grupos A e B receberam em média 1,46 e 5,5 concentrados de hemácias por operação, respectivamente. Os pacientes do grupo A tiveram três complicações (10,7%) e os do B seis (60%). A mortalidade no grupo A foi de 3,5% (n=1) e no grupo B ocorreram quatro óbitos (40%). Os exames de imagem foram sensíveis para presença de tumor, mas não para definir o tipo. As transfusões, a morbidade e a mortalidade foram maiores no grupo com ampliação das indicações e operações mais extensas. Conclusão: Deve-se indicar a biópsia hepática com maior frequência no pré-operatório, ...

Prevalência e achados epidemiológicos, anatomopatológicos e imuno-histoquímicos dos tumores hepáticos malignos primários de cães da Região Central do Rio Grande do Sul (1965-2012) / Prevalence and epidemiological, pathological and immunohistochemical aspects of primary canine malignant hepatic tumors in Rio Grande do Sul, Brazil (1965-2012)

A prevalência e os aspectos epidemiológicos, anatomopatológicos e imuno-histoquímicos dos tumores hepáticos malignos primários (THMP) em cães foram estudados. De 7.373 cães necropsiados em 48 anos (1965-2012), 64 morreram de THMP, o que corresponde a 0,9% do total de cães que morreram por qualquer causa, 7,8% do total de cães que morreram por tumores em geral e 33,5% do total de cães que morreram por tumores hepáticos. Desses 64 casos de THMP, 51 foram revistos histologicamente, avaliados imuno-histoquimicamente e reclassificados como carcinomas (colangiocarcinomas [n=36], carcinomas hepatocelulares [n=9] e hepatocolangiocarcinoma [n=1]) e sarcomas (hemangiossarcomas [n=5]). Dos cães com colangiocarcinomas e carcinomas hepatocelulares em que a idade estava disponível nos protocolos, 64,7% e 77,8% eram idosos, respectivamente. Na necropsia, colangiocarcinomas caracterizaram-se principalmente por ocorrerem em um padrão multinodular (83,3%), enquanto carcinomas hepatocelulares ocorreram tanto de forma massiva (44,4%) quanto nodular (44,4%). Metástases extra-hepáticas foram vistas em 77,8% e 33,3% dos casos de colangiocarcinomas e carcinomas hepatocelulares, respectivamente, e em relação aos colangiocarcinomas afetaram principalmente pulmões (52,8%), linfonodos (50%) e peritônio (19,4%). Ascite (22,2%) e icterícia (22,2%) foram achados associados ocasionalmente com ambos os tumores. Na histologia, a maior parte dos colangiocarcinomas (86,1%) e dos carcinomas hepatocelulares (55,6%) tinha padrão tubular e trabecular, respectivamente. Na imuno-histoquímica, a maioria (63,9%) dos colangiocarcinomas demonstrou imunomarcação para CK7 e nenhum imunomarcou para Hep Par 1. A maioria (55,6%) dos carcinomas hepatocelulares demonstrou imunomarcação para Hep Par 1 e nenhum imunomarcou para CK7. Os resultados aqui apresentados demonstram uma altíssima prevalência de THMP, principalmente colangiocarcinomas, e servem para auxiliar, através dos achados de necropsia, histologia e imuno-histoquímica, patologistas veterinários no diagnóstico dessa tão comum forma de câncer em cães da Região Central do RS, Brasil.

The prevalence and epidemiological and immunohistochemical aspects of primary hepatic malignant tumors (PHMT) were reviewed in dogs necropsied in a 48-year period (1965-2012). Out of those7,373 dogs, 64 died due to PHMT, which corresponds to 0.9% of the dogs dying from any cause in the period; 7.8% of dogs which deaths were caused by tumors in general; and 33.5% of all dogs dying from hepatic tumors (primary and metastatic). Out of the 64 cases of PHMT, 51 were reviewed histologically and evaluated by immunohistochemistry; they were diagnosed as carcinomas (36 cholangiocarcinomas, 9 hepatocellular carcinomas and one hepatocholangiocarcinoma) and sarcomas (5 hemangiosarcomas). In those dogs in which the age was possible determined, 64.7% (cholangiocarcinomas) and 77.8% (hepatocellular carcinomas) were old. At necropsy examination cholangiocarcinomas were characterized mainly by a multinodular pattern (83.3%) while hepatocellular carcinomas occurred both as massive (44.4%) or nodular (44.4%) distribution. Extra-hepatic metastasis occurred respectively in 77.8% and 33.3% of the cases of cholangiocarcinomas and hepatocellular carcinomas; metastatic cholangiocarcinomas affected mainly the lungs (52.8%), lymph nodes (50%) and peritoneum (19.4%). Ascites (22.2%) and icterus (22.2%) were observed frequently associated to both tumors. Histologically, most part of the cholangiocarcinomas (86.1%) and of the hepatocellular carcinomas (55.6%) presented respectively a tubular or trabecular type. Immunohistochemistry revealed that the majority (63.9%) of cholangiocarcinomas was positive for CK7 and none was marked for Hep Par 1. The majority (55.6%) of the hepatocellular carcinomas revealed positive reaction for Hep Par 1 and none was marked for CK7. The results presented here demonstrated a very high prevalence of PHMT, especially cholangiocarcinomas, in the dog. The necropsy, histological and immunohistochemical findings reported might be useful to help veterinary pathologists in the diagnosis of this common form of cancer in dogs of the Rio Grande do Sul, Brazil.

Hemangiomatosis hepática en lactantes: diagnóstico y evolución. “Hospital J. M. de los Ríos”. Caracas - Venezuela / Hemangiomatosis liver in infants: diagnosis and evolution

Las lesiones vasculares hepáticas en niños son raras pero no infrecuentes en gastroenterología pediátrica. Los hemangiomas son los tumores hepáticos vasculares más frecuentes en la infancia, la mayoría de curso benigno, algunos, incluyendo el hemangioendotelioma infantil, tienen potencial maligno. La clínica predominante es hepatomegalia, dolor abdominal, hemangiomas cutáneos e insuficiencia cardíaca congestiva; y menos frecuente esplenomegalia, ictericia, ascitis, hemorragia digestiva y anemia. Se presentan 5 lactantes entre 1 y 4 meses con diagnóstico de hemangiomatosis hepática; en tres de ellos su diagnóstico fue incidental a través de ecografía, uno presentó aumento de volumen abdominal progresivo y otro hepatomegalia; tres presentaron hemangiomas en piel. Todos cursaron con anemia. Se realizó ecografía describiéndose hepatomegalia, con múltiples imágenes redondeadas, hipoecoicas, de diferentes tamaños, en ambos lóbulos hepáticos; Tomografía axial computada abdominal: hepatomegalia con compromiso de ambos lóbulos, ocupados por áreas nodulares hipodensas. Fueron evaluados por los servicios de endocrinología, cardiología, gastroenterología y cirugía pediátrica. En uno se realizó biopsia hepática. Recibieron tratamiento con prednisona 3 - 4 mg/kg/día con descenso progresivo de la misma, seguimiento clínico y ecográfico durante 1 año. Cuatro pacientes respondieron al tratamiento, evidenciándose disminución e incluso desaparición en tres pacientes de los hemangiomas, y uno no respondió, asociándose propanolol.

Hepatic vascular lesions in children are rare but not uncommon in pediatric gastroenterology. Hemangiomas are the most common vascular liver tumors in childhood, most benign course, some, including infantile hemangioendothelioma, have malignant potential. The clinical manifestations are hepatomegaly, abdominal pain, cutaneous hemangiomas and congestive heart failure and less frequent splenomegaly, jaundice, ascites, gastrointestinal bleeding and anemia. We present five infants between 1 and 4 months with a diagnosis of hepatic hemangiomatosis, in three of them the diagnosis was made incidentally by ultrasonography, showed a progressive increase in abdominal volume and a hepatomegaly, three had skin hemangiomas. All of them presented with anemia. Hepatomegaly describing ultrasound was performed, with multiple images rounded, hypoechoic, of different sizes in both lobes, abdominal computed tomography: hepatomegaly with involvement of both lobes, occupied by hypodense nodular areas. Services were assessed by endocrinology, cardiology, gastroenterology and pediatric surgery. In one a liver biopsy was performed. Treated with prednisone 3-4 mg / kg / day with gradual decrease of the same, clinical and ultrasound for 1 year. Four patients responded to treatment, demonstrating decreased or even disappeared in three patients with hemangiomas, and one did not respond, associating propranolol.

Sarcoma indiferenciado (embrionario) de hígado en el adulto / Undifferentiated (embryonal) sarcoma of the liver in adults

We report a 48 years old women presenting with a painful abdominal mass and hepatomegaly. An abdominal CAT scan showed a focal lesion of 14 cm diameter in liver segment 4. The patient was subjected to a left hepatectomy with a normal postoperative evolution. The pathological diagnosis of the surgical piece was an undifferentiated sarcoma. Nine months later, a local relapse was detected and the patient died 22 months after the operation.

Introducción: Los sarcomas indiferenciados (embrionario) del hígado (SIEH) son neoplasias infrecuentes. Se presentan principalmente en edad pediátrica y son considerados de mal pronóstico. En adultos, existen 71 pacientes publicados en el mundo, y en nuestro país no hay casos descritos en este grupo etario. Objetivos: Presentar el caso de una paciente adulta portadora de un SIEH, que fue sometida a cirugía resectiva y realizar una revisión del tema. Caso clínico: Mujer de 48 años de edad, con historia de dolor abdominal, masa palpable en epigastrio y hepatomegalia. Marcadores tumorales negativos, tomografía abdominal revela lesión focal de 14 centímetros en segmento 4. Se aborda quirúrgicamente, biopsia rápida revela tumor sólido maligno indiferenciado, y se realiza hepatectomía izquierda. Evolución postoperatoria favorable. Mediante el análisis histopatológico, histoquímico e inmunohistoquímico se diagnostica un SIEH y se corrobora una resección R0. Al noveno mes de evolución se pesquisa foco de recidiva hepática, inicia progresivo compromiso del estado general, falleciendo 22 meses después de la cirugía. Discusión: Actualmente se recomienda resección completa del tumor seguido de quimioterapia coadyuvante, con lo cual se han logrado sobrevidas libres de enfermedad mayores a 5 años. El dar a conocer las experiencias de casos aislados en esta patología tan infrecuente, permitiría aumentar la casuística mundial, mejorar las técnicas de enfrentamiento, y evaluar el impacto de la quimioterapia en el pronóstico.

Hepatocarcinoma fibrolamelar (HFL) - Relato de caso / Fibrolamellar hepatocarcinoma (FLH) - Report case

INTRODUÇÃO: O hepatocarcinoma fibrolamelar, variante do hepatocarcinoma, é neoplasia rara, responsável por 2% das de origem hepatocelular. RELATO DO CASO: Homem de 24 anos iniciou sintoma doloroso abdominal quatro meses antes da primeira consulta com hiporexia, emagrecimento, vômitos, aumento hepático e nodulação em hipocôndrio direito. Ressonância magnética abdominal mostrou lesão hepática heterogênea, hipervascular, com cicatriz central (provável calcificação), sugestivo de hepatocarcinoma fibrolamelar e presença de hepatomegalia moderada. Realizou-se trissegmentectomia hepática (segmentos VI, VII, VIII), com margem de segurança, sendo a via de acesso a por incisão subcostal direita. Houve confirmação de carcinoma hepatocelular (variante fibrolamelar) moderadamente diferenciado. Na evolução em seis meses encontrava-se bem com exame ultrassonográfico de controle normal, exceto sinais da ressecção efetuada. CONCLUSÃO: Apesar de prognóstico sombrio, o tratamento cirúrgico do hepatocarcinoma fibrolamelar é boa opção quando ampla hepatectomia pode ser realizada.

BACKGROUND: Fibrolamellar hepatocarcinoma is rare and responsible for 2% of the hepatocarcinomas. CASE REPORT: Man with 24 years old was attended by medical institution with abdominal pain, abdominal tumor mass, vomiting and bad general conditions. MRI showed hepatic tumor suggesting hepatocarcinoma. An hepatectomy (segments VI, VII, VIII) was done. It was confirmed malignancy with fibrolamellar hepatocarcinoma. In six months follow-up ultrasonography showed normal aspect of the remained liver. CONCLUSION: Although the bad prognosis of the tumor, hepatectomy should always be tried, if technical resection is feasible.