RESUMO
Introduction: In Europe and North America, the majority of children with high-risk neuroblastoma survive the disease. Elsewhere, the treatment outcomes are poor. Methods: A retrospective review of children treated for high-risk neuroblastoma in a single institution in Singapore from 2007 to 2019 was carried out. Treatment consisted of intensive chemotherapy, surgery aimed at gross total resection of residual disease after chemotherapy, consolidation with high-dose therapy followed by autologous stem cell rescue, and radiotherapy to the primary and metastatic sites followed by maintenance treatment with either cis-retinoic acid or anti-disialoganglioside monoclonal antibody therapy. Survival data were examined on certain clinical and laboratory factors. Results: There were 57 children (32 male) treated for high-risk neuroblastoma. Their mean age was 3.9 (range 0.7-14.9) years. The median follow-up time was 5.5 (range 1.8-13.0) years for the surviving patients. There were 31 survivors, with 27 patients surviving in first remission, and the five-year overall survival and event-free survival rates were 52.5% and 47.4%, respectively. On log-rank testing, only the group of 17 patients who were exclusively treated at our centre had a survival advantage. Their five-year overall survival rate compared to patients whose initial chemotherapy was done elsewhere was 81.6% versus 41.1% (P = 0.011), and that of event-free survival was 69.7% versus 36.1% (P = 0.032). Published treatment results were obtained from four countries in Southeast Asia with five-year overall survival rates from 13.5% to 28.2%. Conclusion: Intensified medical and surgical treatment for high-risk neuroblastoma proved to be effective, with superior survival rates compared to previous data from Southeast Asia.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Neuroblastoma , Criança , Humanos , Masculino , Lactente , Pré-Escolar , Adolescente , Intervalo Livre de Doença , Neuroblastoma/tratamento farmacológico , Neuroblastoma/patologia , Transplante de Células-Tronco Hematopoéticas/métodos , Resultado do Tratamento , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Sudeste Asiático/epidemiologia , Terapia CombinadaAssuntos
Angina Instável/etiologia , Neoplasias Cardíacas/complicações , Pericárdio , Sarcoma Sinovial/complicações , Adulto , Angina Instável/diagnóstico , Angina Instável/cirurgia , Quimiorradioterapia Adjuvante , Ponte de Artéria Coronária , Evolução Fatal , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Humanos , Masculino , Pericárdio/diagnóstico por imagem , Pericárdio/patologia , Pericárdio/cirurgia , Sarcoma Sinovial/diagnóstico por imagem , Sarcoma Sinovial/patologia , Sarcoma Sinovial/cirurgia , Resultado do Tratamento , Recusa do Paciente ao TratamentoAssuntos
Doenças Autoimunes/diagnóstico , Infecções por HIV/diagnóstico , Mielofibrose Primária/diagnóstico , Biópsia , Medula Óssea/patologia , Diagnóstico Diferencial , Infecções por HIV/complicações , Infecções por HIV/tratamento farmacológico , Humanos , Lactente , Masculino , Mielofibrose Primária/complicações , Mielofibrose Primária/imunologiaRESUMO
We report a case of 46,XY pure gonadal dysgenesis (Swyer syndrome) in a phenotypically normal 12-year-old girl with a history of vaginal bleeding and early breast development, with ovarian choriocarcinoma as the first manifestation. The clues leading to the diagnosis included the failure to establish any relationship between normal menstrual cycles postoperatively and a small remaining contralateral ovary. The correct diagnosis is important for cancer prophylaxis and hormonal replacement therapy. Prepubertal and peripubertal girls presenting with gonadal germ cell tumors should be carefully evaluated for the possibility of underlying gonadal dysgenesis. A history of vaginal bleeding or early signs of puberty does not exclude the diagnosis.
