Unusual liver tumors: spectrum of imaging findings with pathologic correlation.

The liver is a common location for both primary and secondary cancers of the abdomen. Radiologists become familiar with the typical imaging features of common benign and malignant liver tumors; however, many types of liver tumors are encountered infrequently. Due to the rarity of these lesions, their typical imaging patterns may not be easily recognized, meaning their underlying pathologic features may not be discovered or suggested until an invasive biopsy is performed. In this review article, we discuss multiple hepatic neoplasms that are both unusual and rare. Some have typical imaging patterns, whereas others are non-specific and can only be included in the differential diagnosis. The clinical history and serologic findings are often critical in suggesting these entities; therefore, these are also discussed to familiarize the radiologist with the appropriate clinical setting of each. The article includes an image-rich description of each entity with accompanying figures describing the ultrasonography, computed tomography, and magnetic resonance imaging features of each disease process. Novel therapies and prognosis of several of the diseases are also included in the discussion.

Epithelioid hepatic angiomyolipoma in pregnancy: A case report.

INTRODUCTION AND IMPORTANCE: Epithelioid hepatic angiomyolipoma (HAML) is a rare benign tumor predominantly found in women. Its occurrence during pregnancy is extremely rare. Accurate diagnosis of HAML is challenging due to its radiological resemblance to other hepatic neoplasms. We present a case of epithelioid HAML in a pregnant patient, highlighting the diagnostic and management challenges encountered. CASE PRESENTATION: A 24-year-old pregnant female, in her fifth month of pregnancy, presented with right hypochondrium pain and nausea. Radiological imaging suggested the possibility of a hepatic adenoma. The patient opted to continue the pregnancy with regular monitoring of the mass as well as fetal health. After delivering a healthy baby, the patient underwent successful mass excision and cholecystectomy. Histopathology of the liver mass confirmed the diagnosis of epithelioid HAML. CLINICAL DISCUSSION: Epithelioid HAML is a rare tumor often misdiagnosed. It is more aggressive and frequently associated with tuberous sclerosis complex (TSC) compared to other subtypes. The diagnosis of HAML can be challenging due to its resemblance to Hepatocellular Carcinoma and other hepatic neoplasms on radiological imaging. Immunohistochemistry plays a crucial role in confirming the diagnosis. Surgical excision is the recommended treatment, with complete removal to minimize the risk of recurrence. CONCLUSION: This case report highlights the rarity of epithelioid HAML during pregnancy and emphasizes the importance of a multidisciplinary approach in managing hepatic neoplasms. Close monitoring is crucial, considering the potential risks to the mother and fetus. Accurate diagnosis through histopathological evaluation, immunohistochemistry and a multidisciplinary approach are essential for appropriate management.

Cistoadenoma mucinoso biliar: Un reto diagnóstico / Mucinous biliary cystadenoma: A diagnostic challenge

Introducción. El cistoadenoma mucinoso biliar es una neoplasia rara con alta probabilidad de malignidad. Su diagnóstico es un reto ya que se asemeja a otras masas benignas que pueden encontrarse en el hígado. Caso clínico. Mujer de 21 años con sensación de masa en hipocondrio derecho, a quien se le realizan marcadores tumorales y estudios de imágenes concluyendo que se trataba de un cistadenoma mucinoso biliar. Resultado. Se presenta el caso de una paciente con cistoadenoma mucinoso biliar, diagnosticada y tratada exitosamente con cirugía. Conclusión. El diagnóstico de cistoadenoma mucinoso biliar se confirma mediante marcadores tumorales y estudios radiológicos, y su tratamiento es quirúrgico debido al riesgo de malignidad

Introduction. Biliary mucinous cystadenoma is a rare neoplasm with a high probability of malignancy. Its diagnosis is a challenge since it resembles other benign masses that can be found in the liver. Clinical case. A 21-year-old woman with a sensation of a mass in the right hypochondrium, who underwent tumor markers and imaging studies, concluding with a diagnosis of biliary mucinous cystadenoma. Result. A case of a patient with biliary mucinous cystadenoma diagnosed and successfully treated by surgery is presented. Conclusion. The diagnosis of biliary mucinous cystadenoma is confirmed by tumor markers and radiological studies, and its treatment is surgical due to the risk of malignancy

Hepatocellular carcinoma with inferior vena cava and right atrium thrombus: A case report.

BACKGROUND: Hepatocellular carcinoma (HCC) with inferior vena cava and right atrium thrombus is rare, accounting for approximately 1.4%-4.9% of cases. These patients are rarely reported, but the condition is being increasingly discovered with advances in imaging techniques, and their prognosis is extremely pessimistic with no current effective treatment. This condition is further associated with unexpected sudden death by cardiac arrest and acute large area pulmonary embolism. CASE SUMMARY: A 34-year-old man with advanced HCC with a hepatic vein thrombus extending into the right atrium had a long-term, disease-free survival following 5-mo sequential treatment combined with transcatheter arterial chemoembolization and curative liver resection. No severe adverse effects were encountered, such as massive hemorrhage or pulmonary embolism. The proper selection of operative method is an important factor. CONCLUSION: HCC with a tumor thrombus extending into the right atrium has a significant impact on the survival of patients. Thrombectomy combined with adjuvant therapy may be beneficial for these patients.

Role of Radioembolization in the Management of Liver-Dominant Metastatic Renal Cell Carcinoma: A Single-Center, Retrospective Study.

PURPOSE: The management of Renal cell carcinoma (RCC) patients with liver metastases is challenging. Liver-directed therapy, such as Transarterial radioembolization (TARE), is a reasonable option for these patients; however, its safety and efficacy are not well characterized. This study evaluated the safety and efficacy of TARE in patients with liver-dominant metastatic RCC. MATERIALS AND METHODS: This is a retrospective, single-center study. Thirty-eight patients' medical records were reviewed who underwent TARE between January 1, 2009, and December 31, 2019, in a tertiary cancer center. Two were excluded from further analysis. Thirty-six patients received 51 TARE treatments. Median follow-up time was 18.2 months. Imaging data were evaluated using mRECIST or RECIST 1.1 criteria. Toxicities, treatment responses, liver progression-free survival (LPFS), and median overall survival (OS) were calculated. Univariate and multivariate analyses were conducted to reveal predictors of OS. RESULTS: Median OS from TARE was 19.3 months (95% CI, 22.6-47.4) and from diagnosis of liver metastases was 36.5 months (95% CI: 26.4-49.8). Mild, grade 1 or 2, biochemical toxicity developed in 27 patients (75%). Grade 3-4 toxicity was noted in two patients (5.5%). The objective response rate was 89%; the disease control rate was 94% (21 complete response, 11 partial response, two stable disease, and two progressive disease). Univariate and multivariate analyses showed longer survival in patients who had objective response, lower lung shunt fraction, and better baseline liver function. CONCLUSIONS: TARE is safe and effective and led to promising overall survival in patients with liver-dominant metastatic RCC. LEVEL OF EVIDENCE: Level 3, retrospective cohort study.

Liver Transplantation in Children: An Overview of Organ Allocation and Surgical Management.

Liver transplantation is the standard treatment for children with end-stage liver disease, primary hepatic neoplasms, or liver-localized metabolic defects. Perioperative mortality is almost absent, and long-term survival exceeds 90%. Organ shortage is managed thanks to advances in organ retrieval techniques; living donation and partial liver transplantation almost eliminated waiting list mortality, thus leading to expanding indications for transplantation. The success of pediatric liver transplantation depends on the prompt and early referral of patients to transplant Centers and on the close and integrated multidisciplinary collaboration between pediatricians, hepatologists, surgeons, intensivists, oncologists, pathologists, coordinating nurses, psychologists, and social workers.

Application value of Laennec approach in laparoscopic anatomical right hemihepatectomy / 中华消化外科杂志

Objective:To investigate the application value of Laennec approach in laparoscopic anatomical right hemihepatectomy (LARH).Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 2 female patients who underwent LARH via Laennec approach in the First Affiliated Hospital of Kangda College of Nanjing Medical University from May to July 2020 were collected. The two patients were 51 and 57 years old, respectively. Observation indicators: (1) surgical situations; (2) postoperative situations and follow-up. Follow-up using outpatient examination and telephone interview was conducted to detect post-operative survival and tumor recurrence of patients up to December 2020. Count data were repre-sented as absolute numbers.Results:(1) Surgical situations: 2 patients successfully underwent LARH via Laennec approach, without conversion to open surgery. The operation time was 180 minutes and 185 minutes, and the volume of intraoperative blood loss was 200 mL and 400 mL, respectively. No blood transfusion or gastrointestinal decompression was performed in either patient. (2) Postoperative situations and follow-up: 2 patients began to take liquid diet on the first day and out-of-bed activities on the postoperative second to third day. There was no postoperative bile fistula or bleeding, but different degrees of peritoneal and pleural effusion occurred to the 2 patients after operation. One case was improved after right-sided thoracentesis and chest tube drainage due to dyspnea, and the other case was cured after conservative therapy. There was no perioperative death. The duration of postoperative hospital stay of 2 patients was 13 days and 11 days, respectively. Results of pathological examination showed 1 case of hepatic hemangioma and 1 case of primary liver cancer, respectively. The Laennec capsule was observed on the hepatic vein branches of segment Ⅴ, Ⅵ, Ⅶ, Ⅷ, and the gap existed between the Laennec capsule and the hepatic vein. Two patients were followed up for 7 months and 5 months，respectively. They survived during the follow-up，without tumor recurrence.Conclusion:It is safe and feasible to perform LARH by Laennec approach.

Minimally invasive versus open right hepatectomy: comparative study with propensity score matching analysis.

BACKGROUND: Minimally invasive liver resections (MILRs) have been increasingly performed in recent years. However, the majority of MILRs are actually minor or limited resections of peripheral lesions. Due to the technical complexity major hepatectomies remain challenging for minimally invasive surgery. The aim of this study was to compare the short and long-term outcomes of patients undergoing minimally invasive right hepatectomies (MIRHs) with contemporary patients undergoing open right hepatectomies (ORHs) METHODS: Consecutive patients submitted to anatomic right hepatectomies between January 2013 and December 2018 in two tertiary referral centers were studied. Study groups were compared on an intention-to-treat basis after propensity score matching (PSM). Overall survival (OS) analyses were performed for the entire cohort and specific etiologies subgroups RESULTS: During study period 178 right hepatectomies were performed. After matching, 37 patients were included in MIRH group and 60 in ORH group. The groups were homogenous for all baseline characteristics. MIRHs had significant lower blood loss (400 ml vs. 500 ml, P = 0.01), lower rate of minor complications (13.5% vs. 35%, P = 0.03) and larger resection margins (10 mm vs. 5 mm, P = 0.03) when compared to ORHs. Additionally, a non-significant decrease in hospital stay (ORH 9 days vs. MIRH 7 days, P = 0.09) was observed. No differences regarding the use of Pringle's maneuver, operative time, overall morbidity or perioperative mortality were observed. OS was similar between the groups (P = 0.13). Similarly, no difference in OS was found in subgroups of patients with primary liver tumors (P = 0.09) and liver metastasis (P = 0.80). CONCLUSIONS: MIRHs are feasible and safe in experienced hands. Minimally invasive approach was associated with less blood loss, a significant reduction in minor perioperative complications, and did not negatively affect long-term outcomes.

Tumores hepáticos en edad pediátrica / Hepatic tumors in pediatric ages

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)

Hepatocellular adenomas: is there additional value in using Gd-EOB-enhanced MRI for subtype differentiation?

PURPOSE: To differentiate subtypes of hepatocellular adenoma (HCA) based on enhancement characteristics in gadoxetic acid (Gd-EOB) magnetic resonance imaging (MRI). MATERIALS AND METHODS: Forty-eight patients with 79 histopathologically proven HCAs who underwent Gd-EOB-enhanced MRI were enrolled (standard of reference: surgical resection). Two blinded radiologists performed quantitative measurements (lesion-to-liver enhancement) and evaluated qualitative imaging features. Inter-reader variability was tested. Advanced texture analysis was used to evaluate lesion heterogeneity three-dimensionally. RESULTS: Overall, there were 19 (24%) hepatocyte nuclear factor (HNF)-1a-mutated (HHCAs), 37 (47%) inflammatory (IHCAs), 5 (6.5%) b-catenin-activated (bHCA), and 18 (22.5%) unclassified (UHCAs) adenomas. In the hepatobiliary phase (HBP), 49.5% (39/79) of all adenomas were rated as hypointense and 50.5% (40/79) as significantly enhancing (defined as > 25% intralesional GD-EOB uptake). 82.5% (33/40) of significantly enhancing adenomas were IHCAs, while only 4% (1/40) were in the HHCA subgroup (p < 0.001). When Gd-EOB uptake behavior was considered in conjunction with established MRI features (binary regression model), the area under the curve (AUC) increased from 0.785 to 0.953 for differentiation of IHCA (atoll sign + hyperintensity), from 0.859 to 0.903 for bHCA (scar + hyperintensity), and from 0.899 to 0.957 for HHCA (steatosis + hypointensity). Three-dimensional region of interest (3D ROI) analysis showed significantly increased voxel heterogeneity for IHCAs (p = 0.038). CONCLUSION: Gd-EOB MRI is of added value for subtype differentiation of HCAs and reliably identifies the typical heterogeneous HBP uptake of IHCAs. Diagnostic accuracy can be improved significantly by the combined analysis of established morphologic MR appearances and intralesional Gd-EOB uptake. KEY POINTS: •Gd-EOB-enhanced MRI is of added value for subtype differentiation of HCA. •IHCA and HHCA can be identified reliably based on their typical Gd-EOB uptake patterns, and accuracy increases significantly when additionally taking established MR appearances into account. •The small numbers of bHCAs and UHCAs remain the source of diagnostic uncertainty.

Total laparoscopic vs. open liver resection: comparative study with propensity score matching analysis / Ressecções hepáticas totalmente laparoscópicas vs. abertas: estudo comparativo com pareamento por pontuação de propensão

ABSTRACT Background: There have been an increasing number of articles that demonstrate the potential benefits of minimally invasive liver surgery in recent years. Most of the available evidence, however, comes from retrospective observational studies susceptible to bias, especially selection bias. In addition, in many series, several modalities of minimally invasive surgery are included in the same comparison group. Aim: To compare the perioperative results (up to 90 days) of patients submitted to total laparoscopic liver resection with those submitted to open liver resection, matched by propensity score matching (PSM). Method: Consecutive adult patients submitted to liver resection were included. PSM model was constructed using the following variables: age, gender, diagnosis (benign vs. malignant), type of hepatectomy (minor vs. major), and presence of cirrhosis. After matching, the groups were redefined on a 1:1 ratio, by the nearest method. Results: After matching, 120 patients were included in each group. Those undergoing total laparoscopic surgery had shorter operative time (286.8±133.4 vs. 352.4±141.5 minutes, p<0.001), shorter ICU stay (1.9±1.2 vs. 2.5±2.2days, p=0.031), shorter hospital stay (5.8±3.9 vs. 9.9±9.3 days, p<0.001) and a 45% reduction in perioperative complications (19.2 vs. 35%, p=0.008). Conclusion: Total laparoscopic liver resections are safe, feasible and associated with shorter operative time, shorter ICU and hospital stay, and lower rate of perioperative complications.

RESUMO Racional: Com a disseminação da cirurgia hepática minimamente invasiva tem-se observado nos últimos anos número crescente de trabalhos que demonstram seus potencias benefícios. No entanto, a maior parte da evidência disponível provém de estudos observacionais retrospectivos sujeitos a vieses, em especial, os de seleção. Além disso, em muitas casuísticas são incluídas no mesmo grupo diversas modalidades de operações minimamente invasivas. Objetivo: Comparar os resultados perioperatórios (até 90 dias) de pacientes submetidos a ressecções hepáticas totalmente laparoscópicas com pacientes contemporâneos por cirurgias abertas, pareados por pontuação de propensão (propensity score matching PSM), submetidos a ressecções hepáticas convencionais. Método: Foram estudados pacientes adultos consecutivos submetidos à ressecção hepática. Para homogeneização dos grupos foi utilizado pareamento por pontuação de propensão, utilizando a variável idade, gênero, tipo de doença (benigna vs. maligna), tipo de hepatectomia (maior vs. menor) e presença de cirrose. A partir disto, os grupos foram redefinidos com proporção 1:1, pelo método nearest. Resultado: Após o pareamento foram incluídos 120 pacientes em cada grupo. Os submetidos à operação totalmente laparoscópica apresentaram menor tempo cirúrgico (286,8±133,4 vs. 352,4±141,5 min, p<0,001), menor tempo de internação em unidade de terapia intensiva (1,9±1,2 vs. 2,5±2,2dias, p=0,031), menor tempo de internação hospitalar (5,8±3,9 vs. 9,9±9,3dias, p<0,001) e redução de 45% nas complicações perioperatórias (19,2 vs. 35%, p=0,008). Conclusão: As ressecções hepáticas totalmente laparoscópicas são exequíveis, seguras e associadas à menor tempo operatório, menor tempo de internação em unidade de terapia intensiva e internação hospitalar, além de diminuição nas complicações perioperatórias.

Research advances in molecular classification for precision diagnosis and treatment of hepatocellular carcinoma / 中华消化外科杂志

Hepatocellular carcinoma (HCC),a highly prevalent malignancy and one of the leading cause of cancer death in China,remains a major public health problem in the next decades.Owing to the tremendous achievements in early diagnosis,precision liver surgery,molecular targeted therapy and immunotherapy,we have witnessed significant improvements in the long-time survival of HCC patients,if properly treated.However,HCC is a highly heterogeneous disease.Even for patients within the same clinical stage,their clinical outcome and treatment efficacy vary significantly.Great efforts to improve the molecular classification of HCC patients are needed to foster precision medicine,paving the way for novel therapeutic strategies.Advances in multi-omics,single-cell analysis,molecular imaging and artificial intelligence will lead to better understanding of the molecular classification and refine precision treatment in HCC,ultimately excluding this neoplasm from the risk list of our Healthy China.

Research advances in molecular classification for precision diagnosis and treatment of hepatocellular carcinoma / 中华消化外科杂志

Hepatocellular carcinoma (HCC), a highly prevalent malignancy and one of the leading cause of cancer death in China, remains a major public health problem in the next decades. Owing to the tremendous achievements in early diagnosis, precision liver surgery, molecular targeted therapy and immunotherapy, we have witnessed significant improvements in the long-time survival of HCC patients, if properly treated. However, HCC is a highly heterogeneous disease. Even for patients within the same clinical stage, their clinical outcome and treatment efficacy vary significantly. Great efforts to improve the molecular classification of HCC patients are needed to foster precision medicine, paving the way for novel therapeutic strategies. Advances in multi-omics, single-cell analysis, molecular imaging and artificial intelligence will lead to better understanding of the molecular classification and refine precision treatment in HCC, ultimately excluding this neoplasm from the risk list of our Healthy China.

Diabetes and cancer: A comprehensive review.

Diabetes mellitus (DM) is a common worldwide endocrine disorder characterized by hyperglycemia resulting from defects in insulin secretion and insulin action or both. A number of clinical studies have investigated diabetes and its causal relation with neoplasm. Several epidemiological studies have found that diabetic patients have an increased risk of different types of cancers, for example liver, pancreas, gastric (stomach), colorectum, kidney, and breast, and it is predicted that hyperglycemic state observed in diabetic milieu enhances the cancer risk in prediabetic and diabetic individuals. To explore the strength of evidence and biases in the claimed associations between type 2 DM (T2DM) and risk of developing cancer, an umbrella review of the evidence across published meta-analyses or systematic reviews is performed. The concurrence of T2DM with the growing burden of cancer globally has generated interest in defining the epidemiological and biological relationships between these medical conditions. Through this review, it was found that diabetes could be related to cancer. Yet, the results from most of the studies are obscure and conflicting and need a robust research so that the link between diabetes and cancer could be firmly and impeccably documented.

Longitudinal evolution of CT and MRI LI-RADS v2014 category 1, 2, 3, and 4 observations.

OBJECTIVES: This study assesses the risk of progression of Liver Imaging Reporting and Data System (LI-RADS) categories, and the effects of inter-exam changes in modality or radiologist on LI-RADS categorization. METHODS: Clinical LI-RADS v2014 CT and MRI exams at our institution between January 2014 and September 2017 were retrospectively identified. Untreated LR-1, LR-2, LR-3, and LR-4 observations with at least one follow-up exam were included. Three hundred and seventy-two observations in 214 patients (149 male, 65 female, mean age 61 ± 10 years) were included during the study period (715 exams total). Cumulative incidence curves for progression to malignant LI-RADS categories (LR-5 or LR-M) and to LR-4 or higher were generated for each index category and compared using log-rank tests with a resampling extension. Relationships between inter-exam changes in LI-RADS category and modality or radiologist, adjusted for inter-exam time intervals, were modeled using mixed effect logistic regressions. RESULTS: Median inter-exam follow-up interval and total follow-up duration were 123 and 227 days, respectively. Index LR-1, LR-2, LR-3, and LR-4 differed significantly in their cumulative incidences of progression to malignant categories (p < 0.0001), which were 0%, 2%, 7%, and 32% at 6 months, respectively. Index LR-1, LR-2, and LR-3 differed significantly in cumulative incidences of progression to LR-4 or higher (p = 0.003). MRI-MRI exam pairs had more stable LI-RADS categorization compared to CT-CT (OR = 0.460, p = 0.0018). CONCLUSIONS: LI-RADS observations demonstrate increasing risk of progression to malignancy with increasing category ranging from 0% for LR-1 to 32% for LR-4 at 6 months. Inter-exam modality changes are associated with LI-RADS category changes. KEY POINTS: • While the majority of LR-2 observations remain stable over long-term follow-up, LR-3 and especially LR-4 observations have a higher risk for category progression. • Category transitions between sequential exams using different modalities (CT vs. MRI) may reflect modality differences rather than biological change. MRI, especially with the same type of contrast agent, may provide the most reproducible categorization, although this needs additional validation. • In a clinical practice setting, in which radiologists refer to prior imaging and reports, there was no significant association between changes in radiologist and changes in LI-RADS categorization.

Sarcoma embrionario indiferenciado en dos pacientes pediátricos / Undifferentiated embryonal sarcoma in two pediatric patients

Resumen El sarcoma embrionario indiferenciado es un tumor hepático mesenquimal maligno infrecuente que ocupa el tercer lugar en frecuencia de las neoplasias primarias del hígado de la infancia; tiene crecimiento rápido y sus síntomas más frecuentes son dolor y sensación de masa abdominal; también puede presentarse ictericia, pérdida de peso, fiebre, emesis y hepatomegalia. El diagnóstico se realiza teniendo en cuenta los hallazgos en imágenes, estudios de inmuno-histoquímica y análisis patológico que aporta el diagnóstico definitivo. Inicialmente, su pronóstico era pobre, con tasas de supervivencia menores a un año; sin embargo, con la introducción de la quimioterapia asociada a la resección quirúrgica, la supervivencia a largo plazo ha mejorado con posibilidad de curación, directamente relacionada con el tratamiento oportuno. Se presentan dos casos de sarcoma embrionario indiferenciado en pacientes pediátricos y diagnosticados en la Fundación Cardioinfantil Instituto de Cardiología, los cuales fueron confirmados por patología. Finalmente, se discuten las características histopa-tológicas, hallazgos imagenológicos, diagnósticos diferenciales y opciones terapéuticas reportadas.

Abstract Undifferentiated embryonal sarcoma, an infrequent malignant mesenchymal liver tumor, occupies the third place in frequency of primary neoplasms of the liver in infancy. It has a fast-growing rate; the most frequent symptoms are pain and abdominal mass, also jaundice, weight loss, fever, emesis and hepatomegaly. The diagnosis is made taking into account the imaging findings, immune-histochemical studies and pathological analysis, the latter providing the definitive diagnosis. The prognosis of this tumor was initially poor, with survival rates of less than 1 year; however, with the introduction of chemotherapy associated with surgical resection, long-term survival has improved with the possibility of cure, directly related to timely treatment. In this article, two cases of undifferentiated embryonal sarcoma in pediatric patients are presented, diagnosed in the Fundación Cardioinfantil-Instituto de Cardiología, confirmed by pathology. Finally, the histopathological characteristics, imaging findings, differential diagnoses and the therapeutic options used are discussed, as reported in the literature.

Hepatitis autoinmune y cáncer de colon: una asociación inusual. Caso clínico y revisión de la literatura / A case of an unusual association of autoimmune hepatitis and colon cancer: report and literature review

Resumen Introducción: la hepatitis autoinmune idiopática (HAI) es una enfermedad crónica que predomina en mujeres, con episodios de actividad y remisión, favoreciendo la fibrosis hepática. El 40% de los pacientes presenta historia familiar de enfermedades autoinmunes. Al parecer, es mediada por la interacción antígeno-anticuerpo; sin embargo, su causa es desconocida. Se conoce la asociación frecuente de HAI con cáncer hepatobiliar; menos frecuente con linfomas, cáncer de piel y cáncer de colon; y casi inexistente con síndromes hereditarios de cáncer de colon. Este caso debutó con HAI y sangrado rectal causado por poliposis adenomatosa familiar (PAF) y adenocarcinoma de colon sigmoide. Caso clínico: mujer de 51 años con HAI de 1 año de evolución manejada con prednisolona y azatioprina. Se realizó una colonoscopia total por anemia en la que se encontraron múltiples pólipos entre 5 y 10 mm y 1 de 30 mm sésil, ulcerado, en colon sigmoide. Se realizó una polipectomía endoscópica múltiple que reportó un adenoma tubulovelloso con displasia de alto y bajo grado en varios pólipos y un adenocarcinoma de bajo grado en el pólipo del sigmoide. Los estudios de extensión fueron negativos para metástasis. Se realizó una repleción nutricional prequirúrgica, luego una colectomía subtotal y una procto-ileoanastomosis con ileostomía de protección. La patología de pieza quirúrgica mostró un adenocarcinoma de colon de bajo grado y adenomas tubulares y tubulovellosos con displasias de alto y bajo grado. Discusión y conclusiones: La asociación de HAI con PAF y cáncer colorrectal (CC) es infrecuente. Es conocida la correlación de HAI con cáncer hepatobiliar (asociado con cirrosis), linfomas, cáncer de piel y otros desórdenes autoinmunes. El pronóstico es malo y no puede establecerse una correlación clara con moduladores inmunes.

Abstract Introduction: Idiopathic autoimmune hepatitis (IAH) is a chronic disease that occurs predominately in women, has episodic activity and remission, and favors hepatic fibrosis. Forty percent of patients have family histories of autoimmune diseases. It is apparently mediated by antigen-antibody interaction, but its causes are unknown. IAH is frequently associated with hepatobiliary cancer, less frequently with lymphomas, skin cancer and colon cancer and very rarely with hereditary colon cancer syndrome. This case debuted IAH and rectal bleeding caused by familial adenomatous polyposis (FAP) and adenocarcinoma of the sigmoid colon. Clinical case: The patient was a 51-year-old woman who had had IAH for one year which had been managed with prednisolone and azathioprine. A total colonoscopy, performed because of anemia, found multiple polyps that measured 5 and 10 mm and one ulcerated 30 mm sessile polyp in the sigmoid colon. A multiple endoscopic polypectomy revealed a tubulovillous adenoma with high and low grade dysplasia in several polyps and a low grade adenocarcinoma in the sigmoid polyp. Tests and examinations for metastasis were negative. Following presurgical nutritional repletion, a subtotal colectomy was performed and an ileal pouch-anal anastomosis with protective ileostomy was created. The pathology of the surgical specimen showed low grade adenocarcinoma of the colon and tubular and tubulovillous adenomas with high and low grade dysplasia. Discussion and conclusions: Association of IAH with familial adenomatous polyposis (FAP) and colorectal cancer (CC) occurs infrequently although associations of IAH with hepatobiliary cancer associated with cirrhosis, lymphomas, skin cancer and other autoimmune disorders are well-known. The prognosis is bad and no clear correlation with immune modulators can be established.

Association between expression of Cullin 4B and prognosis of patients after liver transplantation / 中华消化外科杂志

Objective To investigate the effect of expression of Cullin 4B (CUL4B) on the prognosis of patients after liver transplantation for hepatocellular carcinoma (HCC).Methods The retrospective case-control study was conducted.The clinicopathological data of 79 patients who underwent liver transplantation for HCC in the First Affiliated Hospital of Sun Yat-sen University between January 1,2014 and June 30,2015 were collected.The specimens of HCC tissues were collected and embedded in paraffin,and then were detected by immunohistochemistry staining.Observation indicators:(1) expression of CUL4B in HCC tissues;(2) follow-up and survival;(3) prognostic factors analysis after liver transplantation;(4) association between expression of CUL4B and recurrence and metastasis of tumor after liver transplantation.Follow-up using outpatient examination and telephone interview was performed to detect tumor recurrence or metastasis and survival up to June 2018.Measurement data with normal distribution were represented as (x)±s.The comparison between groups of count data was done using the chi-square test.The survival curve drawn using the Kaplan-Meier method,and the survival analysis was done by Log-rank test.The univariate and multivariate analysis were respectively done using the COX regression model.The association analysis was done using the Pearson test.Results (1) Expression of CUL4B in HCC tissues:immunohistochemistry staining showed that CUL4B was mainly expressed in the cytoplasm,with a powerful brownish-yellow staining.The high expression and low expression of CUL4B in HCC tissues were detected in 64 and 15 patients,respectively.(2) Follow-up and survival:79 patients were followed up for 38-56 months,with an average time of 46 months.During the follow-up,37 patients had no tumor recurrence and 42 had tumor recurrence (32 with tumor extrahepatic metastasis and 10 with intrahepatic metastasis);36 had survival and 43 died;the 1-and 3-year overall survival rates were respectively 86.84％ and 63.25％,and 1-and 3-year tumorfree survival rates were respectively 62.31％ and 51.27％.(3) Prognostic factors analysis after liver transplantation:① Results of univariate analysis showed that preoperative alpha-fetoprotein (AFP),Child-Pugh score,maximum tumour dimension,capsular invasion,intravascular tumor thrombus,Edmonson pathological grading and expression of CUL4B were related factors affecting the 3-year overall survival rate of patients after liver transplantation for HCC [Hazard Ratio (HR) =2.17,3.36,3.66,2.43,2.19,3.36,2.84,95％ confidence interval(CI):1.17-4.04,1.53-7.42,2.10-6.42,1.33-4.17,1.08-9.04,1.58-7.59,1.17-6.32,P＜ 0.05].The preoperative alpha-fetoprotein (AFP),Child-Pugh score,maximum tumour dimension,capsular invasion,intravascular tumor thrombus,Edmonson pathological grading and expression of CUL4B were related factors affecting the 3-year tumor-free survival rate of patients after liver transplantation for HCC (HR =2.06,3.72,3.16,2.36,2.83,3.21,1.69,95％CI:1.34-4.85,1.72-8.63,1.79-7.31,1.46-4.86,1.19-8.63,1.19-7.92,1.06-4.87,P＜0.05).② Results of multivariate analysis showed that maximum tumour dimension,intravascular tumor thrombus and expression of CUL4B were independent factors affecting the 3-year overall survival rate of patients after liver transplantation for HCC [Odds ratio(OR) =3.43,3.69,2.81,95％CI:1.16-6.02,1.96-9.38,1.04-9.63,P＜0.05].The maximum tumour dimension,intravascular tumor thrombus and expression of CUL4B were independent factors affecting the 3-year tumor-free survival rate of patients after liver transplantation for HCC (OR=2.25,4.72,2.74,95％C1:1.16-4.02,1.98-9.47,1.03-7.10,P＜ 0.05).The 3-year overall survival rate in patients with high-and low-expressions of CUL4B was respectively 66.7％ and 32.8％,with a statistically significant difference (x2 =5.69,P＜0.05).The 3-year tumor-free survival rate in patients with high-and low-expressions of CUL4B was respectively 73.3％ and 18.6％,with a statistically significant difference (x2 =4.63,P＜0.05).(4) Association between expression of CUL4B and recurrence and metastasis of tumor after liver transplantation:results of Pearson test showed that expression of CUL4B was significantly associated with HCC recurrence and metastasis after liver transplantation (r =0.62,P＜0.05).The further analysis showed that expression of CUL4B was significantly associated with extrahepatic metastasis after liver transplantation (r=0.84,P ＜ 0.05).Conclusion The expression of CUL4B is associated with HCC recurrence after liver transplantation,and it can be as a predictor for HCC recurrence and distant metastasis after liver transplantation.