RESUMEN
OBJECTIVE: To evaluate for disparities in surgical care among US children with hepatoblastoma (HB) and hepatocellular carcinoma (HCC). STUDY DESIGN: In this retrospective National Cancer Database study (2004-2015), children aged <18 years with HB or HCC were included. Multivariable mixed-effects logistic regression was used to evaluate the association of sociodemographic factors (age, sex, race and ethnicity, insurance status, income, proximity to treating hospital) with the odds of undergoing surgical treatment after adjusting for disease-related factors (tumor size, metastasis, comorbidities) and hospital-level effects. Subgroup analyses by tumor histology were performed. RESULTS: A total of 811 children were included (HB: 80.9%; HCC: 19.1%), of which 610 (75.2%) underwent surgical treatment. Following adjustment, decreased odds of undergoing surgical treatment were associated with Black race (OR: 0.46 vs White, 95% CI [95% CI]: 0.26-0.80, P = .01), and having Medicaid (OR: 0.58 vs private, 95% CI: 0.38-0.88, P = .01) or no insurance (OR: 0.33 vs private, 95% CI: 0.13-0.80, P = .02). In children with HB, Black race was associated with decreased odds of undergoing surgical treatment (OR: 0.47 vs White, 95% CI: 0.25-0.89, P = .02). In children with HCC, Medicaid (OR: 0.10 vs private, 95% CI: 0.03-0.35, P < .001), or no insurance status (OR: 0.10 vs private, 95% CI: 0.01-0.83, P = .03) were associated with decreased odds of undergoing surgical treatment. Other than metastatic disease, no additional factors were associated with likelihood of surgical treatment in any group. CONCLUSIONS: Black race and having Medicaid or no insurance are independently associated with decreased odds of surgical treatment in children with HB and HCC, respectively. These children may be less likely to undergo curative surgery for their liver cancer.
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Carcinoma Hepatocelular , Disparidades en Atención de Salud , Hepatoblastoma , Neoplasias Hepáticas , Humanos , Hepatoblastoma/cirugía , Carcinoma Hepatocelular/cirugía , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/patología , Masculino , Femenino , Niño , Estudios Retrospectivos , Preescolar , Lactante , Estados Unidos , Disparidades en Atención de Salud/estadística & datos numéricos , Disparidades en Atención de Salud/etnología , Adolescente , Factores Sociodemográficos , Medicaid/estadística & datos numéricos , Factores Socioeconómicos , Bases de Datos FactualesRESUMEN
Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein's anomaly following Starnes procedure. The patient's postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.
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Anomalía de Ebstein , Hepatoblastoma , Neoplasias Hepáticas , Corazón Univentricular , Recién Nacido , Humanos , Anomalía de Ebstein/diagnóstico , Anomalía de Ebstein/cirugía , Anomalía de Ebstein/complicaciones , Hepatoblastoma/diagnóstico , Hepatoblastoma/cirugía , Hepatoblastoma/complicaciones , Corazón Univentricular/complicaciones , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/complicacionesRESUMEN
BACKGROUND: Surgical resection remains the cornerstone of treatment for hepatoblastoma in children and offers the best chance of disease-free survival. We aimed to analyze the 30 day outcomes of hepatic resection for hepatoblastoma stratified by extent using the National Surgical Quality Improvement Program-Pediatric (NSQIP-P). METHODS: We queried NSQIP-P for children undergoing resection of Hepatoblastoma from 2012 to 2021. Relevant clinical characteristics and outcomes were extracted for multivariate logistic regression to identify predictors of common adverse outcomes. RESULTS: We included 458 children with a median age of 1.90 years. Overall complications were rare, and perioperative blood transfusion (64.2%) and postoperative ventilation > 48 h (10.0%) were the only adverse events prevalent in more than 5% of patients. Median transfusion volume was 15.7 ml/kg. On multivariate regression, only patients undergoing Trisectionectomy (aOR = 3.387, 95% C.I. = 1.348-8.510) had higher odds of receiving > 75th percentile blood transfusion. Furthermore, only perioperative transfusion and postoperative ventilation > 48 h were statistically more common in patients undergoing extended versus standard resections. CONCLUSIONS: Outcomes following resection of hepatoblastoma are excellent, with low rates of postoperative adverse events. Although children undergoing trisectionectomy likely require greater transfusion volume, extended hepatic resections do not appear to have worse 30 day outcomes despite greater operative complexity and duration.
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Bases de Datos Factuales , Hepatectomía , Hepatoblastoma , Neoplasias Hepáticas , Complicaciones Posoperatorias , Mejoramiento de la Calidad , Humanos , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Femenino , Lactante , Hepatectomía/métodos , Masculino , Preescolar , Niño , Complicaciones Posoperatorias/epidemiología , Resultado del Tratamiento , Estudios Retrospectivos , Transfusión Sanguínea/estadística & datos numéricos , Estados UnidosRESUMEN
PURPOSE: Recently, children with trisomy 18 have been receiving more active treatment for malignancies. We report herein seven cases complete resection was achieved, and discuss multidisciplinary treatment for hepatoblastoma in patients with trisomy 18. METHOD: The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed. RESULT: Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free. CONCLUSION: Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery.
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Hepatoblastoma , Neoplasias Hepáticas , Síndrome de la Trisomía 18 , Humanos , Hepatoblastoma/genética , Hepatoblastoma/cirugía , Síndrome de la Trisomía 18/complicaciones , Síndrome de la Trisomía 18/genética , Síndrome de la Trisomía 18/cirugía , Masculino , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/genética , Femenino , Lactante , Preescolar , Estudios Retrospectivos , Hepatectomía/métodos , Niño , Resultado del Tratamiento , Trisomía/genéticaRESUMEN
Hepatoblastoma (HB) is the most common liver malignancy in children, accounting for approximately 60 % of liver tumors in this population. However, the exact cause of HB remains unclear. The combination of surgery and neoadjuvant chemotherapy has significantly improved the overall survival rate of children with HB, increasing it from 40 % in the past to over 70 %. The concept of precise hepatectomy, which aims to achieve the best rehabilitation outcomes with minimal trauma and maximum liver protection, has been widely accepted by hepatobiliary surgeons. This article provides a comprehensive review of the recent advancements in surgical treatment of HB, focusing on digital surgery and minimally invasive techniques.
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Hepatectomía , Hepatoblastoma , Neoplasias Hepáticas , Procedimientos Quirúrgicos Mínimamente Invasivos , Humanos , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Niño , Hepatectomía/métodos , Terapia NeoadyuvanteRESUMEN
Hepatoblastoma, a common extracranial malignant solid tumor in childhood, is often detected at an advanced stage and is difficult to treat surgically. Despite the availability of multiple comprehensive treatments that can be combined with surgery, hepatoblastoma treatment outcomes remain poor. Surgery is the main treatment strategy for hepatoblastoma, but it faces many challenges, including tumor attachment to surrounding tissues, tumor wrapping or invading of vital organs and tissues, the presence of giant or multiple tumors, distant metastasis, the formation of a tumor thrombus, and significant surgical trauma. In this review, we discuss recent research advances and propose potential strategies for overcoming these challenges. Such strategies may improve the rate of hepatoblastoma resection and local control in children, as well as reduce complications and trauma.
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Hepatoblastoma , Neoplasias Hepáticas , Hepatoblastoma/cirugía , Hepatoblastoma/patología , Humanos , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/patología , Hepatectomía/métodos , Hepatectomía/tendencias , Resultado del Tratamiento , NiñoRESUMEN
OBJECTİVE: Classical transverse, vertical abdominal or thoracoabdominal incisions inpediatric patients are frequently used to removelarge abdominal tumors such as hepatoblastomaand neuroblastoma. We present our initial experiences on our patients who was operated by modifiedMakuuchi incision.MATERİALS AND METHODS: We used this incisionin 6 cases with large abdominal tumors (1 hepatoblastoma and 5 neuroblastoma and/or ganglioneuroma)between January 2019 and August 2020.RESULTS: These patients had previously receivedchemotherapy according to appropiate protocol. Theexposure of surgical field was perfect with this incisionand dissection of the tumors was easily performed.Complete removal of large abdominal tumors was successfully achieved in the patients although the masseshave close proximity and adhesions with importantstructures and organs. There was serous collection in2 patients and it resolved spontaneously. No wound infection, hernia or wound dehiscence was observedduring a mean follow-up of 9.6 months (ranged from3 to18 months).CONCLUSİON: According to our preliminary experiences, the Modified Makuuchi incision provides a niceexposure for removal of large abdominal tumors to thesurgeons and is well tolerated by children. (AU)
OBJETIVO: Las incisiones clásicastransversa, abdominal vertical o toracoabdominal enpacientes pediátricos son utilizadas frecuentementeen la escisión de tumores abdominales de gran tamaño como el hepatoblastoma y el neuroblastoma.Presentamos nuestra experiencia inicial en pacientesoperados usando la incision de Makuuchi modificada.MATERIALES Y MÉTODOS: Usamos esta incision en6 casos con tumores abdominales de gran tamaño (1hepatoblastoma y 5 neuroblastomas y/o ganglioneuroma) entre Enero 2019 y Agosto 2020.RESULTADOS: Los pacientes recibieron quimioterapia neoadyuvante según protocolo. La exposicióndel campo quirúrgico, así como la disección del tumor,fue perfecta con esta incisión. Se logró remover la totalidad de los tumores con éxito a pesar de su proximidad y adherencias a órganos vecinos. Dos pacientespresentaron colecciónes serosas que se resolvieronespontáneamente. No se observaron infecciones de herida, hernias o dehiscencia de heridas durante elperíodo de seguimiento con una media de 9.6 meses(rango de 3-18 meses).CONCLUSIONES: Según nuestra experiencia preliminar, la incisión de Makuuchi modificada ofrece alcirujano una buena exposición del campo quirúrgicopara la extirpación de tumores abdominales de grantamaño, además de ser bien tolerada por los niños. (AU)
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Humanos , Preescolar , Niño , Adolescente , Hepatoblastoma/cirugía , Ganglioneuroma/cirugía , Neoplasias Abdominales/cirugía , Resultado del TratamientoRESUMEN
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.
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Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Hepatoblastoma/cirugía , Hepatoblastoma/tratamiento farmacológico , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/tratamiento farmacológico , Análisis de Supervivencia , Estudios de Seguimiento , Resultado del Tratamiento , Quimioterapia Adyuvante , Medición de Riesgo , EcuadorRESUMEN
No disponible
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Humanos , Hepatoblastoma/cirugía , Neoplasias de las Glándulas Suprarrenales/secundario , Neoplasias Hepáticas/cirugíaRESUMEN
El hepatoblastoma (HB) en el adulto (HBA) es un tumor muy infrecuente del que se han descrito 45 casos hasta junio de 2012. A diferencia del HB en la infancia (HBI), tiene un pronóstico fatal. Presentamos el caso de una mujer de 37 años, asintomática, que consulta por una masa hepática de 12 cm de diámetro que ocupa los segmentos 5 y 6 del hígado, alfa-fetoproteína de 1.556,30 UI/ml. Se realizó bisegmentectomía. La histología confirmó el diagnóstico de HB con presencia de células epiteliales formando túbulos y estructuras trabecu - lares con marcadores para hepatocitos y colangiocitos. La enferma falleció a los 10 meses por progresión de la enfermedad. Recientemente se ha confirmado la mutación en la vía de señalización Wnt/β- Catenina en el HBI y su relación con un pronóstico peor. Debido a su mal pronóstico, parece lógico aplicar las pautas terapéuticas descritas en la población infantil, en la que se obtienen mejores resultados(AU)
Adult hepatoblastoma (AHB) is a very rare tumor, having been described 45 cases up to June 2012. In contrast to HB in infancy (IHB), it has poor prognosis. We present the case of a 37-year-old asymptomatic woman who consulted for a large 12 cm diameter mass involving segments 5 and 6 of the liver, and alfa-fetoprotein of 1,556,30 UI/mL. A bisegmentectomy was carried out. The microscopic study confirmed the AHB diagnosis, revealing the presence of epithelial cells forming clusters, trabecular patterns and tubules. The patient died on the 10th postoperative month due to progression disease. The Wnt/β-Catenin signaling pathway mutation has been reported and associated with a poor prognosis in IHB. Due to the AHB poor prognosis, seems reasonable to introduce the therapeutic regimens described in children who have a better outcome(AU)
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Humanos , Adulto , Hepatoblastoma/complicaciones , Hepatoblastoma/diagnóstico , Hepatoblastoma/fisiopatología , Hepatoblastoma/cirugía , HepatoblastomaRESUMEN
Background: malignant hepatic tumors (Mht) are rare in children. Among them hepatoblastoma (HB) is the most common. Aim: to report the results of the multidisciplinary management in 6 consecutive children: five HB and one metastatic Wilms tumor (MWT). Patients and methods: the mean age of patients was 42 months. All HB patients had elevated serum alfafetoprotein (median 150,000 ng/ml). All patients received preoperative chemotherapy: HB patients received carboplatin/doxorrubicin alternating with cisplatin, and the MWT patient, vincristine alone. Surgery included two formal right and two formal left hepatectomies, one extensive central resection with partial left segmentectomy, and one lateral segmentectomy. Extracorporeal circulation was used in the child with atrial involvement. All patients received postoperative chemotherapy. Results: all tumors had variable regresion on preoperative chemotherapy. Complete resection with negative margins was achieved in all patients. The degree of tumor necrosis on histology ranged from 60 percent to 90 percent. Alfafetoprotein levels fell to under 10 ng/ml in all HB cases, one to three months after surgery. All patients survive free of disease at a median follow up of 19 months. Conclusion: a multidisciplinary approach including the well timed used of chemotherapy and surgery is highly effective in the management of pediatric malignant tumors
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Humanos , Niño , Masculino , Femenino , Preescolar , Lactante , Neoplasias Primarias Secundarias , Hepatoblastoma/diagnóstico , Neoplasias Hepáticas/diagnóstico , Complicaciones Posoperatorias , Quimioterapia Adyuvante , Hepatoblastoma/cirugía , Hepatoblastoma/patología , Hepatoblastoma/tratamiento farmacológico , Hepatectomía , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/tratamiento farmacológico , alfa-Fetoproteínas , Tiempo de InternaciónRESUMEN
Objetivo. Analizar nuestros resultados con el trasplante hepático (TxH) en tumores primitivos hepáticos malignos irresecables. Discutirlas indicaciones controvertidas en base a nuestra experiencia. Pacientes. Doce niños (edad 6 meses-14 años) con hepatoblastoma (11)y hepatocarcinoma (1) sin cirrosis. El TxH se planteó como tratamiento primario en 10 casos (catalogados como PRETEXT IV o con cualquier grado si tenían extensión a cava retrohepática, a 3 venas hepáticaso a tronco de la porta o sus 2 ramas principales) y como tratamiento de rescate tras recidiva (1) o constancia de persistencia de restos (..) (AU)
Objective. To analyse our results on liver transplantation(LTX) in primitive malignant unresectable liver tumours in children and discussing its controversial indications in order to our experience. Methods/Patients. We report 12 patients with ages ranging from 6 months to 14 years old. They had hepatoblastoma (11) and fibrolamellar hepatocellular carcinoma (1) without cirrhosis. LTX was considered as primary treatment in 10 patients (PRETEXT IV or any grade if extension to retrohepaticcava vein, 3 hepatic veins or porta vein were assessed) and as rescuetherapy after recurrence (1) or persistence of unresectable macroscopicrests (2). One of the patients who underwent a LTX as primary therapy had lung metastases previously resolved with chemotherapy. We usedentire liver (5), left lateral segment from cadaveric donor (3), live related donor (3, 2 segments II-III and 1 right liver) and left lateral segment (..) (AU)
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Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Adolescente , Neoplasias Hepáticas/cirugía , Carcinoma Hepatocelular/cirugía , Hepatoblastoma/cirugía , Carcinoma/cirugía , Trasplante de Hígado , Resultado del TratamientoRESUMEN
Introducción: el hepatoblastoma es el principal tumor maligno primario del hígado en niños. El objetivo del presente trabajo es analizar el perfil clínico, tipo de cirugía, complicaciones postoperatorias y sobrevida libre de enfermedad de acuerdo a la modalidad quirúrgica empleada, en niños con hepatoblastoma. Material y métodos: se revisaron los expedientes de pacientes con hepatoblastoma atendidos entre enero de 1985 y diciembre de 1995. Se registraron edad, sexo, manifestaciones clínicas, exámenes de laboratorio, alfa-feto proteína pre y postoperatoria, estudios de gabinete, tipo de procedimiento quirúrgico, complicaciones trans y postoperatorias variedad histológica, estudio, tipo de quimioterapia y sobrevida libre de enfermedad. Resultados: se analizaron 20 pacientes de cuatro meses a 13 años de edad. La principal e invariable manifestación clínica fue una masa abdominal. En once pacientes se encontraron niveles de alfa-feto proteína entre 100-10,000 ng/mL. En 12 pacientes se practicó hemihepatectomía; en cuatro, trisegmentectomía y en el resto, biopsia incisional diagnóstica. Hubo sangrado masivo en cuatro pacientes durante el transoperatorio; ninguno falleció. Todos los pacientes en estadio IV fallecieron. El tipo de resección, completa o incompleta, no fue estadísticamente significativo (p=0.49) para la sobrevida; ésta fue 7/20 con un seguimiento de cuatro a 104 meses. Conclusiones: 1. los niveles de alfa-feto proteína son útiles para la viginalcia y el diagnóstico de pacientes con hepatoblastoma. 2. La mayor sobrevida ocurre en pacientes operados y que recibieron quimioterapia. 3. Un segundo abordaje quirúrgico es preferible después de la citorreducción con quimioterapia en pacientes con tumores muy voluminosos o multicéntricos. 4. La conducta actual para el tratamiento del hepatoblastoma debe ser cirugía más quimioterapia independientemente del estadio