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.

Infrecuente tumor hepatico: Hepatoblastoma. Caso Clínico

Objetivo: Comunicar el primer caso de hepatoblastoma adulto en Uruguay. Actualmente existen 69 casos reportados en la literatura internacional. Materiales y Método: Revisión de historia clínica de paciente de 33 años, sexo femenino. Operada en 2017 con diagnóstico de tumor estromal gástrico. En la exploración se constató tumoración hepática en lóbulo izquierdo que se biopsia. Resultado: neoplasia maligna de estirpe epitelial-carcinoma- diferenciación neuroendócrina. Pobre respuesta a tratamiento neoadyuvante. PET/CT evidenció ausencia de captación con DOTATATE asociado a actividad metabólica con FDG que sugiere TNE poco diferenciado. Se realiza segmentectomía II - III, colecistectomía y esplenectomía por implante en superficie esplénica. Anatomía patológica: hepatoblastoma subtipo epitelial. Bazo sin infiltración. Bordes de resección hepática libres de tumor. Resultados: Buena evolución postoperatoria. Al momento del reporte la paciente tiene una sobrevida de 47 meses bajo quimioterapia. Conclusiones: El hepatoblastoma en el adulto se caracteriza por una evolución agresiva y de mal pronóstico. Su diagnóstico es exclusivamente histológico. La única terapia pretendidamente curativa es la resección completa como lo fue en nuestra paciente. La sobrevida en menores de 45 años con tratamiento quirúrgico es de 12 meses.

Aim: To communicate the first adult hepatoblastoma case in Uruguay. There are only 69 adult cases re- ported in the international literature. Materials and Method: Review of the clinical history of a 33 year old female patient. During surgery of gastric estromal tumor in 2017, exploration on the left hepatic lobe found a tumor which biopsy showed a malignant neoplasy of a epitelial carcinoma with neuroendocrine differenciation. Poor response to neadyuvant treatment. PET/CT negative for DOTATATE, but positive caption with FDG that suggest poor differenciate neuroendocrine tumor. Hepatic surgery was performed with II and III segmentectomy, cholecistectomy and splenectomy because tumor implants on the surface. Results: Pathological anatomy: hepatoblastoma with epithelial differenciation. No infiltration of the spleen. Tumor margins negative. Good postoperative evolution with 47 months of survival under chemotherapy at the time of this report. Conclusions: Adult hepatoblastoma is an agressive tumor with high rates of recurrence and bad prognoses. The only diagnose is by histological findings. The only curative treatment is the surgical resection, as on our case. The survival of patients under 45 years old, with surgical treatment is generally 12 months.

Effects of sevoflurane inhalation anesthesia on intraoperative hepatic blood flow in infants with hepatoblastoma / 肿瘤研究与临床

Objective:To evaluate the effect of different concentrations of sevoflurane inhalation anesthesia on hepatic blood flow in children with hepatoblastoma (HB).Methods:A total of 22 children who underwent radical resection of HB in Shanxi Children's Hospital from January 2019 to August 2021 were perspectively enrolled, including 15 males and 7 females, with an average age of 17±7 months. There were 19 normal children undergoing ultrasound examination without other systemic diseases who received hernia, cryptorchidism, hydrocele of tunica vaginalis surgery during the same period, including 11 males and 8 females, with an average age of 18±5 months. After inhaling different concentrations of sevoflurane preoperatively and intraoperatively, ultrasound apparatus was used to detect the hepatic blood flow (HBF) in HB children. The portal blood flow (PBF), hepatic artery blood flow (HABF), PBF/HABF and HBF were compared between HB children and non-HB children before operation and after inhalation of 1% sevoflurane, 2% sevoflurane.Results:Compared with non-HB children, children with HB had lower PBF [41.9 ml/min (26.8 ml/min, 63.1 ml/min) vs. 66.7 ml/min (41.4 ml/min, 137.2 ml/min), Z = -2.62, P = 0.008], increased HABF [31.2 ml/min (20.4 ml/min, 50.3 ml/min) vs. 12.9 ml/min (5.5 ml/min, 25.0 ml/min), Z = -3.59, P < 0.001], decreased PBF/HABF [1.3 (1.2, 1.4) vs. 6.1 (5.0, 7.5), Z = -5.68, P < 0.001], and the difference in HBF between the both groups was statistically significant ( P>0.05). Compared with the 2% sevoflurane HB group, PBF was reduced [41.1 ml/min (25.0 ml/min, 62.0 ml/min) vs. 63.0 ml/min (40.5 ml/min, 78.3 ml/min), Z = -2.09, P = 0.036] and PBF/HABF was reduced [1.3 (1.1,1.5) vs. 1.8 (1.6, 1.9), Z = -4.01, P<0.001] in the 1% sevoflurane group, and the differences in HABF and HBF were statistically significant (all P>0.05). Conclusion:Relatively low concentration of sevoflurane reduces HBF by reducing PBF for HB children after radical surgery.

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

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

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

Resultados del tratamiento del hepatoblastoma en los Andes Ecuatorianos / Results of the treatment of hepatoblastoma in the Ecuadorian Andes

RESUMEN: El hepatoblastoma (HB), es una neoplasia maligna, que se origina en el hígado. La supervivencia (SV) depende de la extensión de avance de la enfermedad. El objetivo de este estudio fue determinar diferencias en la SV actuarial global (SVAG) y libre de enfermedad (SVLE) en pacientes con HB, según la extensión de su enfermedad. Serie de casos con seguimiento. Se incluyeron pacientes de entre 4 y 160 meses de edad tratados en un centro oncológico de Los Andes ecuatorianos (2000-2019). Las variables resultado fueron: lóbulo afectado, metástasis pulmonar, infiltración vascular, estadio PRETEXT, riesgo, histología, niveles de alfafetoproteína (AFP), remisión completa (RC), SVAG y SVLE. Se utilizó estadística descriptiva y analítica (Chi2, exacto de Fisher y corrección por continuidad). Se realizaron análisis de SV con curvas de Kaplan Meier y log-rank. Fueron estudiados 28 pacientes (53,6 % hombres), con una mediana de edad de 40 meses. Se verificaron metástasis pulmonares e infiltración vascular en el 25,0 % y 35,7 % de los casos respectivamente. La histología, estadio clínico y riesgo alto fueron mayoritariamente tipo epitelial (42,8 %), PRETEXT II (50,0 %) y riesgo alto (67,8 %) respectivamente. La media de AFP al diagnóstico fue 1055712ng/ml y 9 pacientes alcanzaron RC. La SVAG y SVLE general a 19 años fue 33,1 % y 26,0 % respectivamente. Según su extensión, la SVAG y la SVLE para los pacientes de riesgo estándar y alto fueron 50,0 % y 25,4 % (p=0,148); y 50,0 % y 14,7 % (p=0,037) respectivamente. La SVAG y SVLE verificadas son menores a las reportadas en otros estudios. La SVLE según su extensión, presentó diferencia significativa, sin embargo, este resultado debe ser tomado con cautela debido al número pequeño de pacientes.

SUMMARY: Hepatoblastoma (HB), is a malignant neoplasm, which originates in the liver. Survival (SV) depends on the extent of disease progression. The objective of this study was to determine differences in overall SV (OS) and disease-free (DFS) in patients with HB, according to the extent of their disease. Case series with follow-up. Patients between 4 and 160 months of age treated at an oncology center in the Ecuadorian Andes (2000-2019) were included. The result variables were affected lobe, lung metastasis, vascular infiltration, PRETEXT stage, risk, histology, alpha-fetoprotein levels (AFP), complete remission (RC), OS and DFS. Descriptive and analytical statistics (Chi2, Fisher's exact and continuity correction) were used. SV analyzes were performed with Kaplan Meier and log-rank curves. In this analysis 28 patients (53.6 % men), with a median age of 40 months, were studied. Lung metastases and vascular infiltration were verified in 25.0 % and 35.7 % of the cases, respectively. Histology, clinical stage, and high risk were mainly epithelial type (42.8 %), PRETEXT II (50.0 %), and high risk (67.8 %), respectively. The mean AFP at diagnosis was 1055712 ng / ml and 9 patients achieved CR. OS and DFS at 19 years were 33.1 % and 26.0 % respectively. According to their extension, the OS and DFS for standard and high risk patients were 50.0 % and 25.4 % (p = 0.148); and 50.0 % and 14.7 % (p = 0.037) respectively. The verified OS and DFS are lower than those reported in other studies. DFS according to its extension, presented a significant difference, however, this result should be considered with caution due to the small number of patients.

A Survival Case of Premature Infant with Hepatoblastoma (Fetal Pattern) along with Other Serious Comorbidities and Surgical Interventions

An estimated ratio (i.e., 1 in 10) babies are born too early every year. Roughly 1 million children die each year due to impediments raised pertaining to preterm birth. One such extreme preterm male baby was presented in the neonatal care unit with respiratory distress and grunting. Baby was confirmed to have ventricular septal defect along with patent ductus arteriosus and craniosynostosis which was treated with medications and surgical managements. He was also engaged with various prophylactic and empirical antibiotic therapies to cover the microbial growth. The most disturbing stage here was the appearance of liver mass sizing 5.8 cm × 1.3 cm accompanied with area of necrosis, diagnosed with hepatoblastoma which was evident with the aid of ultrasound. Hence, chemotherapy was commenced which was in accordance with Societe Internationale d Oncologie Pediatrique Epithelial Liver Tumor Study Group-3. Although the existing comorbidities haunted the baby for a long time, he finally made it successfully to get into track by fighting all the hurdles bravely, which was a sheer miracle. Along with the clinicians/surgeons, we Clinical Pharmacists worked hand in hand to ensure the baby to be receiving optimized drug regimen keeping in mind the risk-benefit ratio.

Application of liver three-dimensional visualized reconstruction technique in hepatectomy for children with complicated hepatoblastoma / 临床肝胆病杂志

Objective To investigate the application of liver three-dimensional (3D) visualized reconstruction technique in hepatectomy for children with complicated hepatoblastoma. Methods A retrospective analysis was performed for the clinical data of 30 children with hepatoblastoma who underwent hepatectomy for radical resection in PLA Rocket Force Characteristic Medical Center from January 2018 to October 2020, and according to whether liver 3D visualization with IQQA-Liver system was performed before surgery, the children were divided into 3D reconstruction group with 15 children and control group with 15 children. The two groups were compared in terms of perioperative parameters, short-term prognosis, and follow-up conditions. The independent samples t -test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the Fisher's exact test was used for comparison of categorical data between two groups. Results Compared with the control group, the 3D reconstruction group had a significantly higher mean age (55.7±10.2 years vs 28.2±2.7 years, P 0.05). The median follow-up after surgery was 9.5 months. In the 3D reconstruction group, 2 children experienced recurrence and were diagnosed at 10 and 12 months, respectively, after surgery, and they were treated with chemotherapy at the moment; in the control group, 4 children experienced recurrence, which was higher than that in the 3D reconstruction group ( P =0.651), and among these 4 children, 2 had recurrence at 7 months after surgery, received liver transplantation, and survived up to now, and the other 2 children died shortly after recurrence. Conclusion 3D visualized reconstruction technique helps to perform hepatectomy for children with complicated hepatoblastoma more safely and accurately, especially extended hepatectomy for patients with stage POST TEXT III/IV hepatoblastoma, thereby avoiding liver transplantation.

Predictive value of CT imaging features in preoperative high-risk group of childhood hepatoblastoma / 中华放射学杂志

Objective:To investigate the value of CT findings of childhood hepatoblastoma (HB) in predicting preoperative tumor risk stratification.Methods:Totally 46 children with HB confirmed by surgery and pathology were retrospectively enrolled from October 2010 to October 2019 in Shenzhen Children′s Hospital and Xuzhou Children′s Hospital. The preoperative abdominal plain CT and three-phasic contrast-enhanced CT with complete clinical files were evaluated. According to the clinical risk stratification established by the multidisciplinary diagnosis and treatment consensus for children with HB, the HB children were divided into high-risk group and non-high-risk group with 16 and 30 cases respectively. The maximum diameter of tumor, relative tumor volume index, cystic change or necrosis, bleeding, calcification, fibrous septations, tumor rupture, liver capsule retraction and subcapsular effusion were evaluated. Enhancement percentage and enhancement index on arterial, venous and delayed phases of each tumor were measured and calculated. Pearson′s χ 2 test or Fisher′s exact test were used to compare the differences in gender and lesion morphological characteristics between the high-risk group and the non-high-risk group. Two independent sample t test or Mann-Whitney U test were used to compare the differences in age, gestational age, birth weight, α-fetoprotein, platelets, maximum diameter of tumor, relative tumor volume index and CT parameters of the lesion between the two groups. Statistically significant features were included in the binary logistic regression analysis and independent predictors related to high-risk group were obtained. The ROC curve was used to determine the critical value of the high-risk group. Results:There were statistically significant differences in age, maximum diameter of tumor, relative tumor volume index and tumor rupture between the high-risk group and the non-high-risk group (all P<0.05). The logistic regression analysis showed that the maximum diameter of tumor (OR=1.906, P=0.004) and tumor rupture (OR=16.558, P=0.005) were risk factors of the high-risk group. Based on ROC curve, the optimum cut-off point of maximum diameter of tumor to predict high-risk group was 10.5 cm. Tumor rupture, maximum diameter of tumor and maximum diameter of tumor combined with tumor rupture for predicting the incidence of high-risk group resulted in the area under the curve of 0.744, 0.807 and 0.879, respectively. The sensitivity and specificity of maximum diameter of tumor combined with tumor rupture were 75.0% and 96.7%, respectively. Conclusion:The age of onset in high-risk group is relatively older. The maximum diameter of tumor greater than 10.5 cm accompanied by tumor rupture can be regarded as a high-risk sign.

Hepatectomía central combinada con ligadura de la vena porta derecha en un niño con hepatoblastoma bilobar: reporte de un caso / Central hepatectomy combined with ligation of the right portal vein in a child with bilobar hepatobastoma: a case report

Resumen El hepatoblastoma es un tumor maligno, la resección quirúrgica es la meta del tratamiento. Paciente de 7 meses de edad con masa hepática en los segmentos IV A y B, V y VIII, clasificada como PRETEXT III, se realizó hepatectomía central conservando segmentos VI, VII, II, III y doble derivación biliodigestiva. La vena porta derecha involucrada, se ligó para producir hiperplasia compensadora izquierda, conservando el derecho como auxiliar. Hígado izquierdo en 14 días aumentó 48.1 %. Como alternativa al trasplante, en un tiempo quirúrgico se combinó hepatectomía central con ligadura de la vena porta derecha.

Abstract Hepatoblastoma is a malignant tumor. Surgical resection is the goal of treatment. A 7-month-old female patient with a hepatic mass in segments IV A and B, V, and VIII, classified as PRETEXT III. A central hepatectomy preserving segments VI, VII, II, and III, and a double biliodigestive derivation were performed. The right portal vein involved was ligated to produce a compensatory hyperplasia of the left liver, retaining the right one as an auxiliary. At 14 days, the left liver had increased by 48.1%. As an alternative to transplantation, central hepatectomy was combined with ligation of the right portal vein in a single surgical time.

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)

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

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

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

Multidisciplinary diagnosis and treatment of a child with hepatoblastoma and suspected coronavirus disease / 中国肿瘤临床

Objective: To discuss the diagnosis, treatment, and multidisciplinary management of a child with hepatoblastoma (HB) and suspected coronavirus disease (COVID-19). Methods: A child with HB and suspected COVID-19 was admitted to the Children's Hospital, Zhejiang University School of Medicine, in February 2020. The diagnosis and treatment plan were discussed by a multidisciplinary team (MDT). After the MDT discussion, necessary examinations and tests were performed, including routine blood tests, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ribonucleic acid (RNA) and antibody tests, tumor marker tests, chest and abdomen computed tomography (CT), and an ultrasound. The administered treatment included the cisplatin+5-fluorouracil+vincristine (C5V) regimen and cefotaxime. Results: The patient was diagnosed as low-risk HB and completed the first stage of HB chemotherapy after the diagnosis of COVID-19 was ruled out. After 1 week in the hospital, the patient's body temperature returned to normal, the disposition and appetite improved, and all indexes reached the discharge standard. After 14 days of isolation, the patient was discharged. Conclusions: For children with newly diagnosed HB suspected of having COVID-19 during the pandemic period, clinicians must consider the prevention and control of COVID-19 while diagnosing and treating HB as soon as possible. Multidisciplinary management is beneficial for ensuring the development of a safe and efficient treatment plan.

Therapeutic effect and clinical cost of multi-disciplinary team model of hepatoblastoma in children / 北京大学学报(医学版)

OBJECTIVE@#To summarize and analyze the treatment process, long-term efficacy and clinical economics of children's hepatoblastoma (HB) in multi-disciplinary team (MDT) mode, so as to provide basis for the rational choice of diagnosis and treatment.@*METHODS@#From January 2014 to February 2019, 13 cases of hepatoblastoma in children who completed the whole treatment course in the Pediatric Hematology Tumor Ward of Peking University First Hospital were collected and analyzed, and were followed up until June 30, 2020. There were 9 males and 4 females who were diagnosed and treated according to the MDT process in the hospital. The median age was 16 months (2-54 months), 69.23% (9/13) were under 2 years old. The characteristics, diagnosis and treatment process and treatment effect of the cases were summarized, and the cost of clinical treatment was analyzed.@*RESULTS@#According to the pretreatment extent of disease(PRETEXT), there were 1, 9 and 3 children with stages Ⅱ, Ⅲ and Ⅳ. 76.92% (10/13) of the patients had the largest tumor diameter > 10 cm. All the patients received preoperative neoadjuvant chemotherapy (8 patients received 4 cycles of chemotherapy, and 6 patients changed the chemotherapy plan), surgical treatment and postoperative chemotherapy, the tumor volume decreased by more than 50% (64%-95%) in 12 cases, except 1 case with no significant increase of alpha fetal protein and multiple lesions.The median length of stay was 87 days (68-214 days), the median cost of stay was 200 000 yuan (115 000-500 000 yuan), the median length of stay was 7 days (5-17 days), the median cost of stay was 20 000 yuan (15 000-60 000 yuan), and the incidence of postoperative complications was 7.69% (1/13). All the patients were followed up for 16-69 months. All the patients survived.@*CONCLUSION@#Under the MDT mode, the treatment is seamless connection, the long-term prognosis of children with HB is good, and the total hospitalization cost and time are within the acceptable range. Standard preoperative neoadjuvant chemotherapy can significantly reduce the tumor, improve the resection rate, reduce postoperative complications, reduce the total individual expenditure, shorten the total hospital stay, and further improve the long-term disease-free survival rate.

The CT imaging features of hepatoblastoma in term neonates / 中华放射学杂志

Objective@#To investigate the CT features of hepatoblastoma in term neonates.@*Methods@#The clinical data and abdominal CT features of 7 children with neonatal hepatoblastoma confirmed by surgical pathology from January 2015 to January 2019 in Hunan Children′s Hospital were retrospectively analyzed, focusing on the position, size, shape, density and dynamic enhancement characteristics of the mass.@*Results@#All 7 cases were solitary intrahepatic mass, which affected the liver SⅥ in 2 cases, SⅦ in 2 cases, SⅤ+Ⅵ in 1 case, SⅡ+Ⅲ in 1 case, SⅣa+Ⅴ+Ⅷ in 1 case. The maximum diameters were 2.9-10.2 cm (median maximum diameter 4.7 cm). Four cases tumors were spherical shape, while 3 cases were irregular lobulation and extended to the outside of live. The boundary was clear in 6 cases and fuzzy in 1 case. Necrosis, calcification, patchy hemorrhage was shown in 4 cases, 1 case, 5 cases, respectively. All 7 cases tumors showed heterogeneous enhancement, with multiple nodular and lamellar obvious enhancement at the center and edge of the tumor in arterial phase, and gradually filling-in, presented as multiple bands and island-like enhancement, with prominent edge enhanced but no enhancement in the necrotic area in portal venous and delayed phase. The tumor invaded portal vein and bile duct in hilar area in 1 case, with the intra-hepatic bile duct dilation. The caliber of the abdominal aorta below celiac trunk became thinner in 3 cases. 6 cases were epithelial fetal type and 1 case was mixed type with pathological confirmed.@*Conclusion@#The main CT manifestations of neonatal hepatoblastoma are spherical or lobulated, with varied degrees of necrotic, hemorrhage and calcification, and heterogeneous enhancement with gradually regional expansion.

Clinicopathological profile of hepatoblastoma: An experience from a tertiary care center in India

Background: Hepatoblastoma is the most common primary hepatic malignancy in the pediatric population. Advances in pathological evaluation, imaging, risk stratification, neo-adjuvant chemotherapy, and surgery including transplantation have improved survival of these children in the western countries. However, a successful outcome in developing countries such as India with limited resources poses great challenges to the clinician and the family. Histology plays a major role in determining the prognosis of these patients. Methods: A retrospective study was done on 10 children diagnosed with hepatoblastoma between January 2010 and December 2015 in our institution. Clinical, laboratory, radiological, histopathological diagnoses, treatment, and outcome data were collected and analyzed. Results: The median age of these children at diagnosis was 11 months, and only 1 child was premature at birth. Most children were presented with abdominal distension. One child had lung metastasis at presentation. Elevated alpha fetoprotein levels were present in 90% of the children. The histological types were fetal, embryonal, macrotrabecular, and mixed epithelial-mesenchymal types. SIOPEL risk stratification was done, which showed 40% of the children to be of high risk. Three children had PRETEXT 1, 2, and 4, respectively. Conclusion: Our study is significant with respect to the information on PRETEXT staging, risk status, and histological favorability. In developing countries with limited resources and low-socioeconomic status, it is important to have a multidisciplinary team approach and tailor treatment to manage these patients effectively and improve the overall survival.

Hepatoblastoma: 5-Year Retrospective Study. Pediatric Oncological Surgery Service "GENERAL Oncology Hospital.Solon Espinosa Ayala" Solca Quito- Ecuador

Objectives:Describe the demographic variables, types of liver tumor, surgeries performed and survival children diagnosed with liver tumor undergoing surgical treatment.Method: A retrospective analysis was performed in patients with pediatric liver tumors undergoing surgical treatment, from January 2010 to December 2015 at the "General Oncology Hospital. Solon Espinoza Ayala”.Results:Data from our study reported a diagnosis of hepatoblastoma in 51.85% of all pediatric liver tumors; 50% are routine controls without evidence of disease, 14.28% have been completed clinical treatment, 21.42% died from a second primary diagnosis with metastasis, and another 14.28% (only surgery) who were not followed up because they were transfers from another health system; with respect to global survival it was 64%. The ages ranged from 0 to 15 years old with an average of 5.5.Conclusion: It is very important a timely detection and adequate treatment by a specialized center and trained professionals, liver surgery is a very important chapter for the treatment of liver tumors. The surgical approach with tumor-free resection along with multidisciplinary treatment is the goal for healing.

Focal nodular hyperplasia of the liver in children: A report of 2 cases

Focal nodular hyperplasia (FNH) is a benign non-neoplastic lesion of the liver usually found in adults. It is uncommon in children, comprising 2-10% of all pediatric liver tumours. In children, it can occur at all ages, with increased frequency between 6-10 years. We present two cases of FNH in childhood- the first being that of a 5-month-old infant, and the second in a 6-year-old boy. The possibility of congenital FNH had been excluded in the first case. The second case posed diagnostic difficulty initially and was wrongly treated for hepatoblastoma by neoadjuvant chemotherapy, but later correctly diagnosed to be FNH. Both the children are doing well on follow-up. Paediatric FNH though rare, should be kept in mind while dealing with a hepatic mass. Radiological features can be variable and needle sampling may not be sufficient to reach to a diagnosis. Histological examination with glutamine synthetase immunostaining should be performed in doubtful cases to differentiate FNH from other paediatric liver masses, as management differs.

Correlation between IN1 gene expression levels and clinical characteristics and prognosis of hepatoblastoma in childhood / 中华实用儿科临床杂志

Objective To study on the mRNA expression level of IN1 gene associated with the clinical efficacy and prognosis of hepatoblastoma(HB) in children and to elucidate the early warning value of IN1 gene for prognosis and chemotherapy sensitivity.Methods Forty HB patients were selected based on primary diagnosis and treated from January 2015 to May 2018 in Tongren Hospital Affiliated to Capital Medical University.The expression level of INI1 gene was detected by using fluorescence immuno-PCR.Clinical data of children with HB were collected,including staging,grouping,risk,efficacy and prognosis of chemotherapy.The relationship between the mRNA expression of IN1 gene and clinical data was analyzed by SPSS 21.0 software.Results (1) Clinical characters:the medium age of 40 HB patients was (30.50 ± 2.39) months,male 29 cases (72.5 ％),and female 11 cases (27.5 ％).There was 1 case (2.5％) of stage Ⅱ HB,13 cases (32.5％) of stage Ⅲ HB,and 26 cases (65.0％) of stage Ⅳ.The low-and intermediate-risk group had 12 cases (30.0％) of HB,and the high-risk group had 28 cases (70.0％) of HB.(2) Clinical efficacy and prognosis:by July 1st,2018,the medium following-up time was (12.2 ± 10.1) months,4 patients (10％) were treated by chemotherapy without surgery,and 36 patients (90％) were treated by surgery.All patients (100％) received chemotherapy.The average cycle of chemotherapy lasted (13.17 ± 0.02).Ten patients were dead and 30 patients survived till following-up.The overall survival (OS) rates were 75％ and the event-free survival rates were 50％ (20 patients with HB).(3) IN1 gene mRNA expression:The average PCR quantitative of IN1 gene mRNA of 40 HB patients (2-△CT) was 0.31 ± 0.70.The average mRNA quantitative of HB patients (2-△CT) with high-risk and low/intermediate-risk group was 0.23 ± 0.43 and 0.48 ± 1.13 (t =6.363,P =0.05).According to histology diagnosis,the average mRNA quantitative of small cell undifferentiated (SCU) type (12 cases)and other type (25 cases) was 0.09 ± 0.11 and 0.43 ± 0.86 (t =4.533,P =0.04).The average mRNA quantitative of patients with or without radical surgery was 0.04 ± 0.03 and 0.34 ± 0.74 (t =2.935,P =0.022).The data of children with poor chemotherapy sensitivity(19 cases)and those sensitive to chemotherapy (21 cases) were 0.03 ± 0.04 and 0.30 ± 0.82,and the difference was statistically significant (t =5.688,P =0.018).Conclusions Poor therapeutic effect results in low mRNA expression of IN1 gene in children of HB.The IN1 gene expression could be an early warning factor for treatment sensitivity and prognosis.

A Case of Congenital Hepatoblastoma Presented with Hepatic Osteodystrophy Complicated by Multiple Bone Fractures / 임상소아혈액종양

Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone fractures despite adequate supplementation of fat-soluble vitamins including vitamin D. He was born by Caesarean section because of a 7 cm–sized abdominal mass detected by prenatal ultrasonography. The pathologic diagnosis was hepatoblastoma, PRETEXT staging III or IV. Whole body bone scan at the time of diagnosis showed no abnormal uptake. Oral vitamin D3 of 2,000 IU/day was administered with other fat-soluble vitamins. Serum direct bilirubin level gradually increased up to 28.9 mg/dL at postnatal 6 days and was above 5 mg/dL until 110 days of age. Bony changes consistent with rickets became apparent in left proximal humerus since 48 days of age, and multiple bone fractures developed thereafter. With resolving cholestasis by chemotherapy, his bony lesions improved gradually after add-on treatment of bisphosphonate and parenteral administration of vitamin D with calcium. High level of suspicion and prevention of osteodystrophy is needed in patients with hepatoblastoma, especially when cholestasis persists.

Correlation between IN1 gene expression levels and clinical characteristics and prognosis of hepatoblastoma in childhood / 中华实用儿科临床杂志

Objective@#To study on the mRNA expression level of IN1 gene associated with the clinical efficacy and prognosis of hepatoblastoma(HB) in children and to elucidate the early warning value of IN1 gene for prognosis and chemotherapy sensitivity.@*Methods@#Forty HB patients were selected based on primary diagnosis and treated from January 2015 to May 2018 in Tongren Hospital Affiliated to Capital Medical University. The expression level of INI1 gene was detected by using fluorescence immuno-PCR. Clinical data of children with HB were collected, including staging, grouping, risk, efficacy and prognosis of chemotherapy. The relationship between the mRNA expression of IN1 gene and clinical data was analyzed by SPSS 21.0 software.@*Results@#(1)Clinical characters: the medium age of 40 HB patients was(30.50±2.39) months, male 29 cases (72.5%), and female 11 cases (27.5%). There was 1 case (2.5%) of stage Ⅱ HB, 13 cases (32.5%) of stage Ⅲ HB, and 26 cases (65.0%) of stage Ⅳ. The low-and intermediate- risk group had 12 cases (30.0%) of HB, and the high-risk group had 28 cases (70.0%) of HB.(2)Clinical efficacy and prognosis: by July 1st, 2018, the medium following-up time was (12.2±10.1) months, 4 patients (10%) were treated by chemotherapy without surgery, and 36 patients (90%) were treated by surgery .All patients (100%) received chemotherapy.The average cycle of chemotherapy lasted(13.17±0.02). Ten patients were dead and 30 patients survived till following-up.The overall survival (OS) rates were 75% and the event-free survival rates were 50% (20 patients with HB). (3) IN1 gene mRNA expression: The average PCR quantitative of IN1 gene mRNA of 40 HB patients(2-ΔCT)was 0.31±0.70.The average mRNA quantitative of HB patients (2-ΔCT)with high-risk and low/intermediate-risk group was 0.23±0.43 and 0.48±1.13(t=6.363, P=0.05). According to histology diagnosis, the average mRNA quantitative of small cell undifferentiated (SCU) type (12 cases)and other type (25 cases) was 0.09±0.11 and 0.43±0.86(t=4.533, P=0.04). The average mRNA quantitative of patients with or without radical surgery was 0.04±0.03 and 0.34±0.74 (t=2.935, P=0.022). The data of children with poor chemotherapy sensitivity(19 cases)and those sensitive to chemotherapy (21 cases) were 0.03±0.04 and 0.30±0.82, and the difference was statistically significant (t=5.688, P=0.018).@*Conclusions@#Poor therapeutic effect results in low mRNA expression of IN1 gene in children of HB.The IN1 gene expression could be an early warning factor for treatment sensitivity and prognosis.