RESUMO
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.
Assuntos
Carcinoma Hepatocelular , Hepatoblastoma , Neoplasias Hepáticas , Idoso , Brasil/epidemiologia , Carcinoma Hepatocelular/epidemiologia , Carcinoma Hepatocelular/cirurgia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Hepatoblastoma/epidemiologia , Hepatoblastoma/patologia , Hepatoblastoma/terapia , Hospitais Pediátricos , Humanos , Lactente , Neoplasias Hepáticas/epidemiologia , Neoplasias Hepáticas/cirurgia , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVE: The aim of this study was to summarize the experience of a tertiary center in treating hepatoblastoma for the last 21 years. PATIENTS AND METHODS: Fifty-eight cases were included. The tumor extent and prognosis were assessed using the PRETEXT system. The following data were analyzed: age at diagnosis, comorbidities, prematurity, treatment modalities, histopathological findings, surgical details and complications, treatment outcomes, chemotherapy schedules, side effects and complications. Treatment outcomes included the occurrence of local or distant recurrence, the duration of survival and the cause of death. The investigation methods were ultrasonography, CT scan, serum alpha-fetoprotein level measurement and needle biopsy. Chemotherapy was then planned, and the resectability of the tumor was reevaluated via another CT scan. RESULTS: The mean numbers of neoadjuvant cycles and postoperative cycles of chemotherapy were 6±2 and 1.5±1.7, respectively. All children except one were submitted for surgical resection, including 50 partial liver resections and 7 liver transplantations. Statistical comparisons demonstrated that long-term survival was associated with the absence of metastasis (p=0.04) and the type of surgery (resection resulted in a better outcome than transplantation) (p=0.009). No associations were found between vascular invasion, incomplete resection, histological subtype, multicentricity and survival. The overall 5-year survival rate of the operated cases was 87.7%. CONCLUSION: In conclusion, the experience of a Brazilian tertiary center in the management of hepatoblastoma in children demonstrates that long survival is associated with the absence of metastasis and the type of surgery. A multidisciplinary treatment involving chemotherapy, surgical resection and liver transplantation (including transplantations using tissue from living donors) led to good outcomes and survival indexes.
Assuntos
Hepatectomia/métodos , Hepatoblastoma/terapia , Neoplasias Hepáticas/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Brasil , Criança , Pré-Escolar , Cisplatino/administração & dosagem , Doxorrubicina/administração & dosagem , Feminino , Seguimentos , Hepatectomia/mortalidade , Hepatectomia/estatística & dados numéricos , Hepatoblastoma/mortalidade , Hepatoblastoma/patologia , Humanos , Lactente , Recém-Nascido , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Transplante de Fígado/mortalidade , Transplante de Fígado/estatística & dados numéricos , Masculino , Prontuários Médicos , Terapia Neoadjuvante , Complicações Pós-Operatórias , Taxa de Sobrevida , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
OBJECTIVE: The aim of this study was to summarize the experience of a tertiary center in treating hepatoblastoma for the last 21 years. PATIENTS AND METHODS: Fifty-eight cases were included. The tumor extent and prognosis were assessed using the PRETEXT system. The following data were analyzed: age at diagnosis, comorbidities, prematurity, treatment modalities, histopathological findings, surgical details and complications, treatment outcomes, chemotherapy schedules, side effects and complications. Treatment outcomes included the occurrence of local or distant recurrence, the duration of survival and the cause of death. The investigation methods were ultrasonography, CT scan, serum alpha-fetoprotein level measurement and needle biopsy. Chemotherapy was then planned, and the resectability of the tumor was reevaluated via another CT scan. RESULTS: The mean numbers of neoadjuvant cycles and postoperative cycles of chemotherapy were 6±2 and 1.5±1.7, respectively. All children except one were submitted for surgical resection, including 50 partial liver resections and 7 liver transplantations. Statistical comparisons demonstrated that long-term survival was associated with the absence of metastasis (p=0.04) and the type of surgery (resection resulted in a better outcome than transplantation) (p=0.009). No associations were found between vascular invasion, incomplete resection, histological subtype, multicentricity and survival. The overall 5-year survival rate of the operated cases was 87.7%. CONCLUSION: In conclusion, the experience of a Brazilian tertiary center in the management of hepatoblastoma in children demonstrates that long survival is associated with the absence of metastasis and the type of surgery. A multidisciplinary treatment involving chemotherapy, surgical resection and liver transplantation (including transplantations using tissue from living donors) led to good outcomes and survival indexes. .
Assuntos
Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Hepatectomia/métodos , Hepatoblastoma/terapia , Neoplasias Hepáticas/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Brasil , Cisplatino/administração & dosagem , Doxorrubicina/administração & dosagem , Seguimentos , Hepatectomia/mortalidade , Hepatectomia/estatística & dados numéricos , Hepatoblastoma/mortalidade , Hepatoblastoma/patologia , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Transplante de Fígado/mortalidade , Transplante de Fígado/estatística & dados numéricos , Prontuários Médicos , Terapia Neoadjuvante , Complicações Pós-Operatórias , Taxa de Sobrevida , Centros de Atenção Terciária , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
We report a case of hepatoblastoma that developed in a child with Sotos syndrome, an overgrowth syndrome with an increased risk of neoplasms. Genome-wide analysis of copy number alterations showed a gain of chromosome 2, uniparental disomy of 18q, and microdeletion of 5q35.
Assuntos
Anormalidades Múltiplas/patologia , Transtornos do Crescimento/patologia , Hepatoblastoma/genética , Peptídeos e Proteínas de Sinalização Intracelular/genética , Neoplasias Hepáticas/genética , Proteínas Nucleares/genética , Anormalidades Múltiplas/genética , Aneuploidia , Deleção Cromossômica , Transtornos do Crescimento/complicações , Transtornos do Crescimento/genética , Hepatoblastoma/diagnóstico , Hepatoblastoma/terapia , Histona Metiltransferases , Histona-Lisina N-Metiltransferase , Humanos , Lactente , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapia , Masculino , Polimorfismo de Nucleotídeo Único/genética , SíndromeAssuntos
Hepatoblastoma/diagnóstico , Hepatoblastoma/terapia , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/sangue , Terapia Combinada , Hepatectomia , Hepatoblastoma/genética , Hepatoblastoma/patologia , Hepatoblastoma/secundário , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido , Neoplasias Hepáticas/genética , Neoplasias Hepáticas/patologia , Transplante de Fígado , Neoplasias Pulmonares/secundário , Estadiamento de Neoplasias/métodos , Síndromes Neoplásicas Hereditárias/genética , Trissomia , alfa-Fetoproteínas/análise , beta Catenina/genéticaRESUMO
OBJECTIVE: To review our experience in the management of children who present with hepatoblastoma. STUDY DESIGN: Thirty patients treated for hepatoblastoma at a single institution were reviewed. RESULTS: Ten patients presented with stage I to stage II disease and underwent resection. Seventeen presented with stage III disease; two underwent initial resection of which one required rescue transplantation. The remaining 15 underwent biopsies, which were followed by chemotherapy. Nine patients had a reduction in tumor size and underwent conventional resection. One required rescue transplantation for residual disease. Five patients underwent primary transplantation for unresectable disease. One patient expired during chemotherapy. Three patients presented with stage IV disease and underwent biopsies, which were followed by chemotherapy. One patient responded but required "rescue" transplantation after conventional resection. Seven patients underwent aggressive conventional resection (trisegmentectomy or central liver resection); three had positive surgical margins and underwent transplantation. One developed recurrent disease. Five-year survival was 82.5% +/- 7.1%. There was no operative mortality during surgical therapy. All transplant recipients were tumor free, but one died from lymphoma 7 years post-transplant. CONCLUSION: Chemotherapy may reduce tumor size, allowing for conventional resection. If aggressive resection is necessary or bi-lobar disease persists, primary transplantation is recommended.
Assuntos
Hepatoblastoma/terapia , Neoplasias Hepáticas/terapia , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Estudos Transversais , Feminino , Hepatoblastoma/tratamento farmacológico , Hepatoblastoma/cirurgia , Humanos , Lactente , Recém-Nascido , Fígado/efeitos dos fármacos , Fígado/patologia , Fígado/cirurgia , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Masculino , Estadiamento de Neoplasias , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
Pediatric solid tumors represent a distinct set of malignancies of embryonal origin whose incidence peaks in the first years of life. Specific genetic anomalies with pathogenic significance, which have helped to define the diagnosis better and to improve the prognosis of children with these tumors, recently have been discovered. Survival of children with solid tumors also has improved significantly because of effective multidisciplinary care, which, in this case, always involves chemotherapy and surgery. These favorable results require that children with these diseases are referred and treated at institutions that have multidisciplinary teams and the infrastructure and expertise for caring for these children. Diagnostic and therapeutic principles for the most common childhood solid tumors are discussed in this article, with an emphasis on surgical procedures.
Assuntos
Neoplasias , Adolescente , Neoplasias Ósseas/cirurgia , Criança , Hepatoblastoma/terapia , Humanos , Neoplasias Renais/cirurgia , Neoplasias Hepáticas/terapia , Estadiamento de Neoplasias , Neoplasias/diagnóstico , Neoplasias/tratamento farmacológico , Neoplasias/radioterapia , Neoplasias/cirurgia , Neuroblastoma/terapia , Osteossarcoma/cirurgia , Equipe de Assistência ao Paciente , Rabdomiossarcoma/terapiaRESUMO
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/doxorubicin 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% to 90%. 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.