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Primary liver cancer is one of the leading causes of cancer-related deaths worldwide, its early diagnosis and early treatment are of great clinical importance. The main detection tools for liver cancer include serological indicators, imaging tests and risk assessment models. With the advancement of technology and research, the sensitivity and specificity of laboratory tests for liver cancer have been substantially improved, but there are still false negatives and low rates of early diagnosis. For different causes and prevalence regions, each country has developed its clinical practice guidelines to guide risk groups for effective prevention, early diagnosis and standardized treatment. It is important to establish a liver cancer diagnosis strategy that is suitable for China's national conditions, concerning the guidelines for the vigilance and prevention of liver cancer. In this article, the advantages and disadvantages of liver cancer-related tests and the impact of future development trends on laboratory strategies are explained from the perspective of laboratory testing strategies, to provide theoretical support for the practical application of liver cancer diagnostic strategies.

ABSTRACT

Primary liver cancer is one of the leading causes of cancer-related deaths worldwide, its early diagnosis and early treatment are of great clinical importance. The main detection tools for liver cancer include serological indicators, imaging tests and risk assessment models. With the advancement of technology and research, the sensitivity and specificity of laboratory tests for liver cancer have been substantially improved, but there are still false negatives and low rates of early diagnosis. For different causes and prevalence regions, each country has developed its clinical practice guidelines to guide risk groups for effective prevention, early diagnosis and standardized treatment. It is important to establish a liver cancer diagnosis strategy that is suitable for China's national conditions, concerning the guidelines for the vigilance and prevention of liver cancer. In this article, the advantages and disadvantages of liver cancer-related tests and the impact of future development trends on laboratory strategies are explained from the perspective of laboratory testing strategies, to provide theoretical support for the practical application of liver cancer diagnostic strategies.

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Introducción: El biliotórax es una condición infrecuente definida por la presencia de bilis en el espacio pleural. Actualmente, hay alrededor de 70 casos descritos en la litera-tura. Sigue siendo relativamente desconocido, por lo tanto, poco sospechado. Esta entidad suele ser el resultado de una lesión iatrogénica, a menudo secundaria a cirugías o traumatismos del tracto biliar, que conduce a la formación de una fístula pleurobiliar.

Introduction: Bilothorax is a rare condition defined by the presence of bile in the pleural space. Currently, there are around 70 cases described in the literature. It remains relatively unknown and, therefore, little suspected. This entity is usually the result of an iatrogenic injury, often secondary to surgery or trauma to the biliary tract, leading to the formation of a pleurobiliary fistula

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Presentamos el caso de un paciente de 49 años, de sexo masculino, que consulta en el servicio de urgencias por un cuadro de dos meses de evolución, caracterizado por compromiso del estado general, baja de peso, dolor abdominal, sensación febril y elevación de los parámetros inflamatorios. Al estudio imagenológico se observa una voluminosa lesión hepática, asociada a dilatación de la vía biliar y adenopatías en hilio hepático, espacio porto-cavo y retroperitoneales (inter-cavo-aórticos), que plantea dentro de los diagnósticos diferencias un colangiocarcinoma intrahepático. Basados en esta sospecha se realiza una segmentectomía y linfadenectomía regional. El estudio histopatológico e inmunohistoquímico de la pieza quirúrgica, evidencia un proceso inflamatorio linfoplasmocitario, con la presencia de células plasmáticas IgG4 positivas, compatible con una enfermedad asociada a IgG4. Posterior a la resección se decide manejo expectante, evolucionando el paciente de forma favorable, asintomático y sin signos de recurrencia. Presentamos un caso y una breve revisión de la literatura de un pseudotumor inflamatorio hepático, entidad poco frecuente y de comportamiento benigno.

We report the case of a 49-year-old man who attended the emergency department for a two-month history of compromised general condition, weight loss, abdominal pain, fever, and elevated inflammatory parameters. An imaging study demonstrates a bulky liver tumor associated with dilation of the bile duct and retroperitoneal adenopathies (hepatic hilum, intermediate, and right lumbar groups). These findings raise intrahepatic cholangiocarcinoma within the differential diagnoses, reason why segmental hepatectomy and regional lymphadenectomy were performed. Histopathology and immunochemistry revealed a lymphoplasmacytic inflammatory process with IgG4-positive plasma cells compatible with IgG4-associated disease. After the resection, expectant management was decided, with the patient evolving favorably, asymptomatic, and without signs of recurrence. We present a case and a brief literature review of an hepatic inflammatory pseudotumor, a rare entity with a benign behavior.

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Objective: To investigate the clinical value of serum glypican-3 (GPC3) detection in predicting recurrence of primary hepatocellular carcinoma (HCC). Methods: Through univariate and multivariate logistic regression analysis, the patients pathologically diagnosed with HCC in our hospital from March 2019 to January 2021 were enrolled as the experimental group (n=113), and patients with follow-up time longer than 6 months were included in the prognosis group(n=64). At the same time,20 healthy individuals and 20 individuals with benign liver disease from the physical examination center were enrolled by simple random sampling as control group (n=40). The serum GPC3 and alpha-fetoprotein (AFP) levels were respectively detected by ELISA and chemiluminescence. Then, the study explored the influential factors of the recurrence in HCC patients and constructed the HCC-GPC3 recurrence predicting model by logistic regression. Results: In the research, the sensitivity of GPC3 for the diagnosis of HCC was 61.95% (70/113) and AFP was 52.21% (59/113), meanwhile, the specificity of GPC3 could reach 87.50% (35/40) and AFP was 90.00% (36/40),respectively; The serum GPC3 levels of HCC patients with progressive stage, tumor size≥3 cm, vascular cancer thrombosis and portal venous thromboembolism were significantly higher than that of HCC patients with early stage, tumor size<3 cm, vascular cancer thrombosis and portal venous thromboembolism (Z=2.677, 2.848, 2.995, 2.252, P<0.05), independent of different ages, presence or absence of ascites, peritoneal metastasis, cirrhosis, intrahepatic metastasis (Z=-1.535, 1.011, 0.963, 0.394, 1.510, P>0.05), respectively. Univariate analysis showed that there were no statistically significant differences between the recurrence group and the non-recurrence group in terms of different age, tumor size, presence or absence of vascular cancer thrombosis, ascites, peritoneal metastasis, cirrhosis and AFP levels (χ2=2.012, 0.119, 2.363, 1.041, 0.318, 0.360, Z=0.748, P>0.05); The ratio of those with the progressive stage, portal venous thromboembolism and intrahepatic metastasis and GPC3 levels were all higher in the recurrence group than in the non-recurrence group (χ2=4.338, 11.90, 4.338, Z=2.805, P<0.05).Including the above risk factors in the logistic regression model, the logistic regression analysis showed that the stage, the presence of portal venous thromboembolism,intrahepatic metastasis and GPC3 levels were correlated with the prognosis recurrence of HCC patients (Wald χ2 =4.421, 5.681, 4.995, 4.319, P<0.05), and the HCC-GPC3 recurrence model was obtained as: OcScore=-2.858+1.563×[stage]+1.664×[intrahepatic metastasis]+2.942×[ portal venous thromboembolism]+0.776×[GPC3]. According to the receiver operating characteristic curve(ROC), the area under the curve(AUC)of the HCC-GPC3 prognostic model was 0.862, which was better than that of GPC3 alone (AUC=0.704). The cut-off value of model SCORE was 0.699 (the cut-off value of GPC3 was 0.257 mg/L), furthermore, the total sensitivity and specificity of model were 83.3% and 82.4%, which were better than those of GPC3(60.0% and 79.4%).Kaplan-Meier showed that the median PFS was significantly shorter in HCC patients with high GPC3 levels (≥0.257 mg/L) and high values of the model SCORE (≥0.700) (χ2=12.73, 28.16, P<0.05). Conclusion: Besides diagnosing of HCC, GPC3 can may be an independent risk indicator for the recurrence of HCC and can more efficiently predicting the recurrence of HCC patients when combined with the stage, the presence or absence of intrahepatic metastasis and portal venous thromboembolism.

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Introducción: Los tumores malignos primarios del hígado son extremadamente raros. Representa solo el 1% de todas las neoplasias malignas en el grupo de edad pediátrica, con menos de 1,5 casos por millón de niños menores de 18 años, El hepatoblastoma es la neoplasia hepática primaria más común (48%); El carcinoma hepatocelular es la segunda neoplasia hepática primaria más común en la infancia (27%), y el resto son tumores vasculares y sarcomas, el tipo de carcinoma hepatocelular fibrolamelar es poco común suele observarse en el grupo de mayor edad (mediana de edad 26,4 años). Caso clínico: Se realiza el reporte del caso ya que son pocos casos reportados en la literatura, se trata de una paciente de 11 años quien sufre trauma de abdomen por caída en bicicleta, razón por la cual consulta, llama la atención sin antecedentes previos, totalmente asintomática, pero con aumento de hepatomegalia y dolor abdominal durante hospitalización, por lo cual se realizan estudios de extensión, con evidencia por resonancia magnética contrastada, de lesión tumoral que comprometía el lóbulo hepático derecho en su totalidad con confirmación por biopsia de variante fibrolamelar. Conclusión: Los tumores hepáticos son infrecuentes, la variante fibrolamelar es aún más inusual, la edad típica de presentación son adultos jóvenes, por lo cual es muy escaso su presentación en edad pediátrica; inicialmente su manejo se basa en la resección quirúrgica, dependiendo el estadio presentado al momento del diagnóstico.

Introduction: Primary malignant tumors of the liver are extremely rare. It represents only 1% of all malignancies in the pediatric age group, with less than 1.5 cases per million children under 18 years of age. Hepatoblastoma is the most common primary hepatic neoplasm (48%); Hepatocellular carcinoma is the second most common primary liver neoplasm in childhood (27%), and the rest are vascular tumors and sarcomas. The type of fibrolamellar hepatocellular carcinoma is rare, usually seen in the older age group (median age 26 ,4 years). Clinical case: The case report is made since there are few cases reported in the literature, it is an 11-year-old patient who suffers trauma to the abdomen due to a fall on a bicycle, which is why she consults, draws attention with no previous history, totally asymptomatic, but with increased hepatomegaly and abdominal pain during hospitalization, for which extension studies were performed, with evidence by contrast magnetic resonance, of a tumor lesion that involved the entire right hepatic lobe with confirmation by biopsy of a fibrolamellar variant. Conclusion: Liver tumors are infrequent, the fibrolamellar variant is even more unusual, the typical age of presentation is young adults, for which its presentation in pediatric age is very scarce; Initially, its management is based on surgical resection, depending on the stage presented at the time of diagnosis.

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Background: Cholangiocarcinoma (CCA) is a primary hepatic tumor, frequently found in patients with liver cirrhosis and biliary tract diseases. Its varieties include isolated CCA or "combined hepatocellular-cholangiocarcinoma" (cHCC-CCA). The latter is uncommon, with poorly defined diagnostic criteria and natural history. Aim: To characterize patients with cirrhosis with a pathological diagnosis of CCA and cHCC-CCA. Material and Methods: Forty-nine liver biopsies with a pathological diagnosis of CCA were reviewed. The clinical records of patients were reviewed to fetch demographic variables, etiology of cirrhosis and clinical presentation. Results: Eight of the 49 patients had cirrhosis (16% of CCA biopsies reviewed). Their median age was 64 (27-71) years and five were females. Four patients had CCA, three patients cHCC-CCA and one had a bifocal tumor. Patients in the CCA group were more commonly symptomatic. Alpha-fetoprotein and CA 19-9 levels were elevated in one of eight and four of six patients, respectively. Within 12 months from diagnosis, five of eight patients died. Conclusions: In most of these cases, the diagnosis of cHCC-CCA and CCA was made in the liver explant study without previous imaging diagnosis. This reinforces the usefulness of the histological study, in specific cases, prior to liver transplantation and emphasizes the importance of systematic explant exploration in these cases.

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Hepatocellular carcinoma (HCC), which makes up the majority of liver cancer, is induced by the infection of hepatitis B/C virus. Biomarkers are needed to facilitate the early detection of HCC, which is often diagnosed too late for effective therapy. The tRNA-derived small RNAs (tsRNAs) play vital roles in tumorigenesis and are stable in circulation. However, the diagnostic values and biological functions of circulating tsRNAs, especially for HCC, are still unknown. In this study, we first utilized RNA sequencing followed by quantitative reverse-transcription PCR to analyze tsRNA signatures in HCC serum. We identified tRF-Gln-TTG-006, which was remarkably upregulated in HCC serum (training cohort: 24 HCC patients vs. 24 healthy controls). In the validation stage, we found that tRF-Gln-TTG-006 signature could distinguish HCC cases from healthy subjects with high sensitivity (80.4%) and specificity (79.4%) even in the early stage (Stage I: sensitivity, 79.0%; specificity, 74.8%; 155 healthy controls vs. 153 HCC patients from two cohorts). Moreover, in vitro studies indicated that circulating tRF-Gln-TTG-006 was released from tumor cells, and its biological function was predicted by bioinformatics assay and validated by colony formation and apoptosis assays. In summary, our study demonstrated that serum tsRNA signature may serve as a novel biomarker of HCC.

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A 55-year-old woman was investigated for occasional epigastric pain and weight loss. T2-weighted abdominal magnetic resonance imaging and magnetic resonance cholangiography revealed a multilocular cyst with multiple septa and a solid component in the liver, measuring 6.1 × 4.8 × 6.5 cm. Given the patient's symptoms and malignant potential, a laparoscopic segmentectomy with partial resection of segments IV B and V was performed to completely remove the cystic lesion, associated with cholecystectomy. Histopathology demonstrated a cyst lined by columnar mucinous epithelium. Therefore, the diagnosis was mucinous cystic neoplasm of the liver. This article presents a case report and literature review of this entity.

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OBJECTIVE@#By optimizing the extreme learning machine network with particle swarm optimization, we established a syndrome classification and prediction model for primary liver cancer (PLC), classified and predicted the syndrome diagnosis of medical record data for PLC and compared and analyzed the prediction results with different algorithms and the clinical diagnosis results. This paper provides modern technical support for clinical diagnosis and treatment, and improves the objectivity, accuracy and rigor of the classification of traditional Chinese medicine (TCM) syndromes.@*METHODS@#From three top-level TCM hospitals in Nanchang, 10,602 electronic medical records from patients with PLC were collected, dating from January 2009 to May 2020. We removed the electronic medical records of 542 cases of syndromes and adopted the cross-validation method in the remaining 10,060 electronic medical records, which were randomly divided into a training set and a test set. Based on fuzzy mathematics theory, we quantified the syndrome-related factors of TCM symptoms and signs, and information from the TCM four diagnostic methods. Next, using an extreme learning machine network with particle swarm optimization, we constructed a neural network syndrome classification and prediction model that used "TCM symptoms + signs + tongue diagnosis information + pulse diagnosis information" as input, and PLC syndrome as output. This approach was used to mine the nonlinear relationship between clinical data in electronic medical records and different syndrome types. The accuracy rate of classification was used to compare this model to other machine learning classification models.@*RESULTS@#The classification accuracy rate of the model developed here was 86.26%. The classification accuracy rates of models using support vector machine and Bayesian networks were 82.79% and 85.84%, respectively. The classification accuracy rates of the models for all syndromes in this paper were between 82.15% and 93.82%.@*CONCLUSION@#Compared with the case of data processed using traditional binary inputs, the experiment shows that the medical record data processed by fuzzy mathematics was more accurate, and closer to clinical findings. In addition, the model developed here was more refined, more accurate, and quicker than other classification models. This model provides reliable diagnosis for clinical treatment of PLC and a method to study of the rules of syndrome differentiation and treatment in TCM.

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OBJECTIVE@#LAPTM4B-35 protein is one of the isoforms that are encoded by a cancer driver gene, LAPTM4B. This gene was primarily found and identified in our lab of Peking University School of Basic Medical Sciences. The LAPTM4B-35 protein and its encoded mRNA are significantly over-expressed in a variety of cancers, such as hepatocellular carcinoma (HCC), lung cancers (including non small-cell lung cancer and small-cell lung cancer), stomach cancer, colorectal carcinoma, pancreatic cancer, gallbladder cancer, cholangiocarcinoma, breast cancer, prostate cancer, ovarian cancer, cervical cancer, endometrial cancer, and so on. It has firmly demonstrated through lab experiments either in vivo or in vitro, as well as clinical studies that the over-expression of LAPTM4B-35 can promote cancer growth, metastasis, and multidrug resistance. Specially, the expressive level of LAPTM4B-35 is associa-ted with recurrence of HCC. The aim of this study is to identify the release of LAPTM4B-35 protein from hepatocellular carcinoma into blood of HCC patients and into the medium of cultured HCC cells, and to identify its possible form of LAPTM4B-35 protein existed in blood and cell culture medium, as well as to explore the possibility of LAPTM4B-35 protein as a novel HCC biomarker for diagnosis of HCC and prognosis of HCC patients.@*METHODS@#Immunobloting (Western blot) and enzyme-linked immunosorbent assay (ELISA) were used for identification of LAPTM4B-35 protein in the blood of HCC patients and normal individuals. Ultrafiltration and ultracentrifugation were used to isolate and purify exosomes from the culture medium of HCC cells.@*RESULTS@#LAPTM4B-35 protein existed in the blood from HCC patients and normal donors that were demonstrated through Western blot and ELISA. LAPTM4B-35 was also released into the culture medium of HCC cells in the form of exosomes. Preliminary experiments showed that the average and the median of LAPTM4B-35 protein level in the blood of HCC patients (n=43) were both significantly higher than that in the blood of normal donors (n=33) through sandwich ELISA.@*CONCLUSION@#It is promising that the LAPTM4B-35 protein which is released from HCC cells in the form of exosomes into their extraenvironment may be exploited as a novel cancer biomarker for HCC serological diagnosis.

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Introducción: Los tumores del hígado representan de 1-2 por ciento de todas las neoplasias malignas de la infancia y de 15-20 por ciento de los tumores abdominales. Objetivo: Caracterizar desde el punto de vista clínico-quirúrgico a pacientes pediátricos con diagnóstico de tumor hepático. Métodos: Estudio descriptivo y transversal realizado en el hospital pediátrico Juan Manuel Márquez. Se revisaron historias clínicas, informes histopatológicos e informes operatorios en el periodo comprendido entre el 1ro. de enero de 1997 al 31 de diciembre de 2017, para obtener los datos clínicos necesarios para la investigación. La muestra quedó conformada por 63 pacientes. Se emplearon frecuencias absolutas y porcentajes para variables cualitativas. Para las variables cuantitativas, se emplearon además medidas de tendencia central y de dispersión. Resultados: Se constató que 33 (52,4 por ciento) pacientes fueron del sexo masculino. El mayor número de enfermos se concentró en el grupo de 1 a 5 años con 36 (57,1 por ciento). El tumor más frecuente fue el hepatoblastoma y dentro de este el hepatoblastoma fetal, del cual se registraron 16 pacientes (25,4 por ciento). En 34 pacientes (54 por ciento) se combinó el tratamiento médico y el quirúrgico. Conclusiones: Predominan los pacientes masculinos, entre 1 y 5 años de edad. Se identifican principalmente tumores hepáticos epiteliales, malignos en estadio III y la variedad histológica de hepatoblastoma fetal. El tratamiento más utilizado es el médico-quirúrgico según protocolo del hospital dependiente del tipo histológico(AU)

Introduction: Liver tumors represent 1-2 percent of all the malignant neoplasms in children and the 15-20 percent of abdominal tumors. Objective: To characterize from the clinical surgical perspective the pediatric patients with a diagnosis of hepatic tumor. Methods: Descriptive and cross-sectional study conducted in Juan Manuel Márquez Pediatric Hospital. There were reviewed clinical records, histopathological reports and surgical reports from January 1st, 1997 to December 31st, 2017, to obtain necessary clinical data for the research. The sample was formed by 63 patients. There were used absolute frequencies and percentages for qualitative variables. For quantitative variables, there were used central trend and diffusion measures. Results: It was verified that 33 patients (52.4 percent) were males. The biggest number of patients was in the age group from 1 to 5 years being 36 (57.1 percent). The most frequent tumor was the hepatoblastoma and within this category the fetal hepatoblastoma, with 16 (25.4 percent) patients with that condition. In 34 patients (54 percent) were combined medical and surgical approchaes. Conclusions: There was a predominance of male patients in the ages from 1 to 5 years. There were mainly identified patients with epitelial hepatic tumors, malignant tumors in stage III and the histopatological variation of fetal hepatoblastoma. The most common treatment was the medical-surgical one according to the hospital´s protocols and depending on the histologic type(AU)

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Glypican-3 (GPC3) is a key member of Glypican family and plays an important role in the development, angiogenesis and metastasis of hepatocellular carcinoma (HCC). Most HCC overexpresses GPC3, but GPC3 is hardly detected in normal adult liver and benign liver lesions, so it is regarded as a highly specific diagnostic marker and an ideal therapeutic target for HCC. In this study, we cloned the heavy and light chain variable region gene from the monoclonal antibody targeted to GPC3 screened in the previous stage, and linked it with a segment of flexible peptide (Linker) to obtain the single chain antibody against GPC3. The single chain antibody gene was cloned into vector for prokaryotic expression and purified to obtain high purity protein. Detection shows that the single-chain antibody produced by us has the same binding activity with the full-length antibody, and can accurately target the tumor site of Huh7 tumor-bearing model mice after coupling Cy5.5 fluorescence, suggesting that the single-chain antibody has the potential to realize multi-directional liver cancer precise surgical navigation under the guidance of a probe.

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ABSTRACT Hepatocellular carcinoma (HCC) is one of the leading causes of cancer-related mortality worldwide. The Brazilian Society of Hepatology (SBH) published in 2015 its first recommendations about the management of HCC. Since then, new data have emerged in the literature, prompting the governing board of SBH to sponsor a single-topic meeting in August 2018 in São Paulo. All the invited experts were asked to make a systematic review of the literature reviewing the management of HCC in subjects with cirrhosis. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of updated recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present manuscript is the final version of the reviewed manuscript containing the recommendations of SBH.

RESUMO O carcinoma hepatocelular (CHC) é uma das principais causas de mortalidade relacionada a câncer no Brasil e no mundo. A Sociedade Brasileira de Hepatologia (SBH) publicou em 2015 suas primeiras recomendações sobre a abordagem do CHC. Desde então, novas evidências sobre o diagnóstico e tratamento do CHC foram relatadas na literatura médica, levando a diretoria da SBH a promover uma reunião monotemática sobre câncer primário de fígado em agosto de 2018 com o intuito de atualizar as recomendações sobre o manejo da neoplasia. Um grupo de experts foi convidado para realizar uma revisão sistemática da literatura e apresentar uma atualização baseada em evidências científicas visando que pudesse nortear a prática clínica multidisciplinar do CHC. O texto resultante foi submetido a avaliação e aprovação de todos membros da SBH através de sua homepage. O documento atual é a versão final que contêm as recomendações atualizadas e revisadas da SBH.

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ABSTRACT Introduction: primary liver sarcoma is a rare type of tumor, more common in children. Among adults, it represents a spectrum of neoplasms with reserved prognosis. There is no consensus on the treatment of choice of these lesions, justifying a systematic review of the literature on treatment options, prognostic factors, and survival. Material/Methods: a systematic review of articles published in Pubmed, Medline, LiLacs e SciElo, from 1966 to March/2019, presenting the keywords: primary-liver-sarcoma and primary-hepatic-sarcoma was undertaken. Studies including patients older than 18 years, and published in English, Portuguese and Spanish were included. Case reports, metastatic tumors and multiple oncologic diagnosis were excluded. The initial search listed 1,318 articles. 1,206 did not meet the inclusion criteria. After reviewing 112 eligible articles, 15 were selected (14 case series and 1 retrospective-cohort). Results: proposed treatment modalities for primary liver sarcoma included surgery and/or chemotherapy and/or radiotherapy or liver transplantation. The most common histological types were angiosarcoma (32%), leiomyosarcoma (29%), epithelioid hemangioendothelioma (15%) and embryonal sarcoma (7%). Histology, degree of differentiation and R0 resection were mentioned positive prognostic factors. Median survival ranged from two to 23 months. Five-year survival rate varied from 0% to 64%, on average 21%. Conclusion: surgical resection (R0 resection) is the main treatment for primary liver sarcomas. Development of effective systemic therapies are required to improve prognosis of patients harboring this type of tumor.

RESUMO Introdução: o sarcoma primário do fígado é uma neoplasia rara, que acomete mais frequentemente crianças. Nos adultos, representa espectro de neoplasias de prognóstico reservado. Entretanto, em nenhuma faixa etária há consenso sobre a terapêutica de escolha dessas lesões, motivando revisão sistemática com objetivo de elencar opções de tratamento, fatores prognósticos e sobrevida. Material/Método: realizamos revisão sistemática dos artigos publicados nas bases de dados Pubmed, Medline, LiLacs e Scielo, de 1966 a março/2019, contendo as palavras-chaves: primary-liver-sarcoma e primary-hepatic-sarcoma. Foram incluídos estudos que incluíram pacientes com idade a partir de 18 anos e publicados em inglês, português e espanhol. Relatos de caso, tumores metastáticos e pacientes com múltiplos diagnósticos oncológicos foram excluídos. Foram encontrados inicialmente 1.318 artigos, desses, 1.206 foram excluídos por estarem fora dos critérios de inclusão. Dos 112 artigos analisados e discutidos, 15 foram incluídos nesse artigo (14 séries de casos e 1 estudo de coorte retrospectivo). Resultado: os tratamentos propostos para o sarcoma primário do fígado em adultos incluíram cirurgia e/ou quimioterapia e/ou radioterapia ou transplante hepático. Os tipos histológicos mais frequentemente relados foram angiossarcoma (32%), leiomiossarcoma (29%), hemangioendotelioma epitelioide (15%) e sarcoma embrionário (7%). Tipo histológico, grau de diferenciação e resseção R0 foram os principais fatores de bom prognóstico. A sobrevida média total variou de dois a 23 meses. A sobrevida em cinco anos variou entre 0 e 64%, em média 21%. Conclusão: ressecção cirúrgica R0 é a base do tratamento dos sarcomas primários do fígado. Esse grupo heterogêneo de tumores requer desenvolvimento de terapias sistêmicas efetivas para melhoria do prognóstico.

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The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson's capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

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Background: Conventional serum tumor markers (CSTM) are widely used for monitoring patients with cancer. However, their usefulness as a diagnostic tool is controversial in primary or metastatic liver cancer (PMLC). Aim: To evaluate the diagnostic performance of the most commonly requested CSTM in the diagnostic approach of PMLC. Material and Methods: Review of medical records of patients aged over 18 years with a liver biopsy, attended from 2005 to 2017 in a tertiary hospital and a regional cancer center in Colombia. The results of liver biopsies were compared with tumor markers such as carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), CA 19-9, CA 125 and prostate specific antigen (PSA) using a receiver operating characteristic (ROC) curve analysis. Results: We reviewed 2063 medical records and retrieved 118 eligible patients (59 cases and 59 controls, 70% males). Thirty percent had obstructive jaundice. There was heterogeneity in the amount of tumor markers requested according to medical criteria. Only CA 19-9 showed discriminative capacity (> 17.6 U/m), with a cut-off point lower than that reported in the literature and a sensitivity of 69.5%, specificity of 91.6%, a positive likelihood ratio (LR) of 8.32, and a negative LR of 0.33. Conclusions: Except for CA 19-9, tumor markers were not useful for the initial diagnostic approach in patients with suspected primary or metastatic malignant liver tumors.

ABSTRACT

Introduction: Hepatocellular carcinoma (HCC) in cirrhosis is diagnosed, most of times, when it is not susceptible to curative treatment. Transarterial chemoembolization (TACE) is a palliative therapeutic option with heterogeneous results. The HAP score stratifies patients who will benefit from the first TACE. Objective: To evaluate if the HAP score is a prognostic factor of HCC treated with TACE. Materials and methods: Retrospective cohort study in cirrhotic patients with HCC and first TACE at the Edgardo Rebagliati Martins National Hospital, Lima-Peru, from June 2011 to June 20139. The HAP score was applied, mortality and survival were observed with a follow-up of 36 months. Results: We included 54 patients with age of 67.7±9.9 years, 59.3% Child-Pugh A and 40.7% Child-Pugh B, MELD score of 11±2.7; 51.9 and 40.7% were BCLC A and B, respectively; 66.7% had a single tumor and 70.4% had a predominant tumor <5cm. The HAP score classified 8, 14, 26 and 6 patients as HAP A, B, C and D, respectively. The overall survival was 19.5±11.2 months and 32.8±6.5 months for HAP A, 24.9±14.8 months for HAP B, 13.9±5.2 months for HAP C and 14±6.6 months for HAP D. There were no deaths at 12 months in HAP A. At 24 months, mortality for HAP C and D was 100%. At 36 months, the survival rate for HAP A and B was 75 and 42.9%, respectively. Conclusions: The HAP score is a useful tool to guide the management decisions of cirrhotic patients with HCC requiring TACE due to its value in predicting mortality and survival.

Introducción: El carcinoma hepatocelular (CHC) en cirrosis es diagnosticado, la mayoría de veces, cuando no es susceptible de tratamiento curativo. La quimioembolizacón transarterial (QETA) es una opción terapéutica paliativa con resultados heterogéneos. El HAP score estratifica a los pacientes que se beneficiarán con la primera QETA. Objetivo: Demostrar si el HAP score es un factor pronóstico del CHC tratado con QETA. Materiales y métodos: Estudio de cohortes retrospectivo en pacientes cirróticos con CHC y primera QETA en el Hospital Nacional Edgardo Rebagliati Martins, Lima-Perú, junio-2011 a junio-2013. Se aplicó el HAP score, y se observó la mortalidad y sobrevida con un seguimiento de 36 meses. Resultados: Se incluyeron 54 pacientes con edad de 67,7±9,9 años, 59,3% Child-Pugh A y 40,7% Child-Pugh B, MELD de 11±2,7; 51,9 y 40,7% fueron BCLC A y B, respectivamente; 66,7% tuvo tumor único y el 70,4% tumor predominante menor a 5 cm. Se clasificó como HAP A, B, C y D a 8, 14, 26 y 6 pacientes, respectivamente. La sobrevida general fue 19,5±11,2 meses; y 32,8±6,5 meses para HAP A, 24,9±14,8 meses para HAP B, 13,9±5,2 meses para HAP C y 14±6,6 meses para HAP D. A los 24 meses, la mortalidad para HAP C y D fue 100%. A los 36 meses, la sobrevida para HAP A y B fue 75 y 42,9%, respectivamente. Conclusiones: El HAP score es una herramienta útil que orienta al manejo del CHC tributario de QETA por su valor pronóstico de mortalidad y sobrevida.