Tumores da região da glândula pineal em crianças e adolescentes: aspectos neurocirurgicos
Data
2023-04-05
Autores
Valsechi, Linoel Curado 
Orientadores
Cavalheiro, Sergio
Tipo
Dissertação de mestrado
Título da Revista
ISSN da Revista
Título de Volume
Resumo
Objetivos: Analisar o perfil clinico-epidemiológico dos tumores da região da glândula pineal em crianças e adolescentes; analisar o papel da neuroendoscopia no manejo dos tumores nesta região; analisar os resultados cirúrgicos, na ressecção dos tumores e no tratamento da hidrocefalia, e comparar as diferentes técnicas em relação ao grau de ressecção, eficácia, morbi-mortalidade e sobrevida livre de doença; e identificar os fatores de pior prognóstico de cada tipo histológicos. Métodos: Foram analisados 151 prontuários de crianças e adolescentes entre 0 e 21 anos tratadas no período de 1991 a 2020 na Escola Paulista de Medicina e no Instituto de Oncologia Pediátrica de São Paulo (IOP / GRAACC). O manejo de tais lesões consistia em coletar marcadores tumorais em todos os pacientes; se positivo, realizava-se quimioterapia, se negativo, biópsia preferencialmente endoscópica ou diretamente para cirurgia. Cirurgia ressectiva também era utilizada quando havia lesão residual pós quimioterapia ou quando o resultado anatomopatológico evidenciava que a lesão não era da linhagem germinativa. Resultados: A distribuição por tipo histológico encontrada foi: germinoma 33,1%; tumores de células germinativas não germinomatosos (TCGNG) 27,1%; pinealoblastoma 22,5%; glioma 12,5%; tumor embrionário (Teratóide rabdóide atípico) 3,3% e miscelânia em 1,32%. 97 pacientes foram submetidos à ressecção cirúrgica com ressecção total em 64% dos casos, sendo maior nos tumores de células germinativas (74%) e menor nos gliomas (30%). O acesso mais utilizado foi a via infratentorial supracerebelar (53,6%) seguido pelo occipital transtentorial (24,7%). Biopsia da lesão foi realizada em 70 pacientes com uma acurácia de 94%. A sobrevida geral por tipo histológico em 12, 24 e 60 meses foi, respectivamente, de: 93,7; 93,7 e 88% para germinomas; 84,5; 63,5; 40,7% para pinealoblastomas; 89,4; 80,8 e 67,2% para os TCGNG; 89,4; 78,2 e 72,6% para os gliomas e 40; 0 e 0% para os tumores embrionários (p < 0001). A sobrevida geral em 60 meses foi significativamente superior no grupo que atingiu ressecção completa (69,7%), em relação à ressecção subtotal (40,8%) (p=0,04). A sobrevida livre de doença em 5 anos foi: 77% para os germinomas; 72,6% para os gliomas; 50,8% para os TCGNG, e 38,9% para os pinealoblastomas. Em relação aos fatores prognósticos, nos germinomas o sexo feminino foi único fator de pior prognóstico. Nos tumores de células germinativas não germinomatosas (TCGNG), metástase ao diagnóstico, lesão residual e ausência de radioterapia foram relacionados a pior prognostico. Nos pinealoblastomas o sexo masculino foi o único fator de pior prognóstico, com tendência a pior desfecho nos pacientes < 3 anos e com metástase ao diagnóstico. Os gliomas de alto grau foram relacionados a pior prognostico. Tumor teratoide rabdoide foi visto em 3,3% e todos foram a óbito num período máximo de 19 meses. Conclusão: Tumores da região da pineal são mais prevalentes no sexo masculino, sobretudo da linhagem de células germinativa. Síndrome de hipertensão intracraniana é a clinica mais frequente. Os tipos histológicos pinealoblastoma e ATRT tiveram pior desfecho e acometeram crianças em média abaixo de 7 anos. Neuroendoscopia é uma ferramenta indispensável no manejo das lesões cuja acurácia da biopsia foi de 91,4%; apresentou menores taxas de complicação para tratamento de hidrocefalia, comparado a DVP, com taxas semelhantes de sucesso; contribuiu para ampliar a ressecção cirugica e inspecionar presença de metástase. Não houve diferença entre os acessos cirúrgicos utilizados em relação morbi-mortalidade e grau de ressecção. A ressecção cirúgica completa, em geral, relacionou-se a melhor prognóstico. Foram identificados os seguintes fatores de pior prognósticos nos tipos histológicos: sexo feminino nos germinomas; lesão residual, ausência radioterapia e metástase nos TCGNG, sexo masculino nos pinealoblastomas; lesão de alto grau nos gliomas.
Objective: Analyzing demographic data of pineal region tumors in children and adolescents; analyzing the role of neuroendoscopic in management of tumors in this region; analyzing surgical results, in the tumor’s resection and hydrocephalus’ treatment, and to compare differents approaches regarding to resection’s degree, efficacy, morbidity and mortality, and progression-free survival; and to identify prognostic factors of each histological type. Methods: We analyzed 151 medical records of children and adolescents aged between 0 and 21 years treated between 1991 and 2020 at Escola Paulista de Medicina and Instituto de Oncologia Pediátrica of São Paulo (IOP / GRAACC). The management of such lesions consisted of collecting tumor markers in all patients; if positive, chemotherapy was performed, if negative, preferably endoscopic biopsy or directly for surgery. Surgery was also used when there was residual lesion after chemotherapy or when the biopsy showed other type of lesion excepted germ line tumor. Results: The distribution by histological type found was: germinoma 33.1%; non-germinomatous germ cell tumors (NGGCT) 27.1%; pinealoblastoma 22.5%; glioma 12.5%; embryonary tumor (atypical teratoid rhabdoid tumor) 3.3 % and miscellany 1,32%. 97 patients underwent surgical resection with total resection in 64%, the highest in germ cell tumors (74%) and lowest in gliomas (30%). The most used approach was the infratentorial supracerebellar (53.6%) followed by the transtentorial occipital (24.7%). Biopsy was performed in 70 patients with an accuracy of 94%. Overall survival by histological type at 12, 24 and 60 months was, respectively, 93.7; 93.7 and 88% for germinomas; 84.5; 63.5; 40.7% for pinealoblastomas; 89.4; 80.8 and 67.2% for NGGCT; 89.4; 78.2 and 72.6% for gliomas and 40; 0 and 0% for embryonary tumors (p < 0001). Overall survival at 60 months was significantly higher in the group that achieved total resection (69.7%) compared to subtotal resection (40.8%) (p=0.04). Progression-free survival at 5 years was: 77% for germinomas; 72.6% for gliomas; 50.8% for TCGNG, and 38.9% for pinealoblastomas. Regarding prognostic factors, in germinomas, female was the only factor with worse prognosis. In NGGCT, metastasis at diagnosis, residual lesion and absence of radiotherapy were related to worse prognosis. In pinealoblastoma, male was the only factor with worse prognosis, with a tendency to worse outcome in patients < 3 years and with metastasis at diagnosis. High-grade gliomas were related to worse prognosis. Atypical teratoid rhabdoid tumor was seen in 3.3% and all died within a maximum period of 19 months. Conclusion: Pineal region tumors are more prevalent in males, specially germ cell tumors. Intracranial hypertension syndrome is the most frequent symptoms. Pineoblastoma and ATRT had the worst outcome and affected children under 7 years on average. Neuroendoscopy is a pivotal tool in the management of this lesion, and biopsy accuracy was 91,4%; ETV showed lower complication rates for hydrocephalus than VPS and similar success rates; it contributed to enlarge surgical resection and inspected the presence of metastasis. There was no difference between surgical approaches regarding mobidity, mortality and degree of resection. Gross total resection, in general, was related to a better overall survival. The following prognostic factores were related to a worst outcome: female gender in germinoma; residual lesion, absence of radiotherapy and metastasis in NGGCT; male gender in pineoblastoma and high grade lesion in glioma.
Objective: Analyzing demographic data of pineal region tumors in children and adolescents; analyzing the role of neuroendoscopic in management of tumors in this region; analyzing surgical results, in the tumor’s resection and hydrocephalus’ treatment, and to compare differents approaches regarding to resection’s degree, efficacy, morbidity and mortality, and progression-free survival; and to identify prognostic factors of each histological type. Methods: We analyzed 151 medical records of children and adolescents aged between 0 and 21 years treated between 1991 and 2020 at Escola Paulista de Medicina and Instituto de Oncologia Pediátrica of São Paulo (IOP / GRAACC). The management of such lesions consisted of collecting tumor markers in all patients; if positive, chemotherapy was performed, if negative, preferably endoscopic biopsy or directly for surgery. Surgery was also used when there was residual lesion after chemotherapy or when the biopsy showed other type of lesion excepted germ line tumor. Results: The distribution by histological type found was: germinoma 33.1%; non-germinomatous germ cell tumors (NGGCT) 27.1%; pinealoblastoma 22.5%; glioma 12.5%; embryonary tumor (atypical teratoid rhabdoid tumor) 3.3 % and miscellany 1,32%. 97 patients underwent surgical resection with total resection in 64%, the highest in germ cell tumors (74%) and lowest in gliomas (30%). The most used approach was the infratentorial supracerebellar (53.6%) followed by the transtentorial occipital (24.7%). Biopsy was performed in 70 patients with an accuracy of 94%. Overall survival by histological type at 12, 24 and 60 months was, respectively, 93.7; 93.7 and 88% for germinomas; 84.5; 63.5; 40.7% for pinealoblastomas; 89.4; 80.8 and 67.2% for NGGCT; 89.4; 78.2 and 72.6% for gliomas and 40; 0 and 0% for embryonary tumors (p < 0001). Overall survival at 60 months was significantly higher in the group that achieved total resection (69.7%) compared to subtotal resection (40.8%) (p=0.04). Progression-free survival at 5 years was: 77% for germinomas; 72.6% for gliomas; 50.8% for TCGNG, and 38.9% for pinealoblastomas. Regarding prognostic factors, in germinomas, female was the only factor with worse prognosis. In NGGCT, metastasis at diagnosis, residual lesion and absence of radiotherapy were related to worse prognosis. In pinealoblastoma, male was the only factor with worse prognosis, with a tendency to worse outcome in patients < 3 years and with metastasis at diagnosis. High-grade gliomas were related to worse prognosis. Atypical teratoid rhabdoid tumor was seen in 3.3% and all died within a maximum period of 19 months. Conclusion: Pineal region tumors are more prevalent in males, specially germ cell tumors. Intracranial hypertension syndrome is the most frequent symptoms. Pineoblastoma and ATRT had the worst outcome and affected children under 7 years on average. Neuroendoscopy is a pivotal tool in the management of this lesion, and biopsy accuracy was 91,4%; ETV showed lower complication rates for hydrocephalus than VPS and similar success rates; it contributed to enlarge surgical resection and inspected the presence of metastasis. There was no difference between surgical approaches regarding mobidity, mortality and degree of resection. Gross total resection, in general, was related to a better overall survival. The following prognostic factores were related to a worst outcome: female gender in germinoma; residual lesion, absence of radiotherapy and metastasis in NGGCT; male gender in pineoblastoma and high grade lesion in glioma.