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Craniofaringioma : caracterização da série pediátrica do Hospital de Santa Maria (CHLN)
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Os craniofaringiomas são tumores primários da região selar e suprasselar de natureza histológica benigna. São neoplasias pouco frequentes e apresentam uma distribuição bimodal com um pico de incidência nos grupos etários dos 5-14 anos e dos 50-74 anos, sem predileção por género. A apresentação clínica é caracterizada por sintomas relacionados com o aumento da pressão intracraniana (cefaleias, náuseas e vómitos) e por sintomas provocados pela compressão das estruturas adjacentes ao tumor (alterações visuais e endócrinas). A abordagem terapêutica clássica deste tumor implica uma cirurgia extensa com remoção total da lesão. Apesar das elevadas taxas de sobrevida, esta abordagem está associada a uma alta taxa de complicações por disfunção do eixo hipotálamo-hipofisário. Deste modo, nos últimos anos têm sido defendidas abordagens mais conservadoras com o objectivo de reduzir a morbilidade a longo prazo e melhorar a qualidade de vida. Neste trabalho foram analisadas as modalidades de tratamento utilizadas e os seus resultados a longo prazo em pacientes em idade pediátrica com diagnóstico de craniofaringioma tratados no Hospital de Santa Maria do Centro Hospitalar Lisboa Norte (HSM-CHLN), entre Janeiro de 2000 e Dezembro de 2017. Da análise desta série conclui-se que o contexto epidemiológico encontrado é semelhante ao esperado e a apresentação clínica e características radiológicas e histológicas tumorais observadas vão de encontro ao descrito na literatura. Tal como noutros centros na Europa e América do Norte, o tratamento do craniofaringioma no HSM tem vindo a sofrer alterações nos últimos anos, com a adoção de uma abordagem mais conservadora.
Craniopharyngiomas are primary tumors of the sellar and suprasellar region with benign histological characteristics. They are uncommon neoplasms and show a bimodal age distribution with peak incidence in children from 5 to 14 years old and in adults from 50 to 74 years old, with no gender differences. The clinical presentation is characterized by symptoms related to increased intracranial pressure (headache, nausea and vomiting) and symptoms caused by the compression of structures adjacent to the tumor (visual impairment and endocrine deficits). For many years, extensive surgery with complete resection was the method of choice for the treatment of craniopharyngioma. Despite the high survival rates, this approach resulted in multiple long-term complications due to dysfunction of the hypothalamic-pituitary axis. In recent years less invasive surgical approaches have been advocated with the aim of reducing long-term morbidity and improving quality of life. In this work, the treatment modalities and their long-term results were analysed in pediatric patients with the diagnosis of craniopharyngioma, treated at Hospital de Santa Maria (HSM-CHLN) between January 2000 and December 2017. From the analysis of this series we concluded that our cohort has a similar epidemiology when compared to other series in the literature. The clinical presentation, radiological and histological tumor characteristics observed are also similar to those described in the literature. As in other centers in Europe and North America, the treatment of craniopharyngioma at Hospital de Santa Maria has been changing to a more conservative approach.
Craniopharyngiomas are primary tumors of the sellar and suprasellar region with benign histological characteristics. They are uncommon neoplasms and show a bimodal age distribution with peak incidence in children from 5 to 14 years old and in adults from 50 to 74 years old, with no gender differences. The clinical presentation is characterized by symptoms related to increased intracranial pressure (headache, nausea and vomiting) and symptoms caused by the compression of structures adjacent to the tumor (visual impairment and endocrine deficits). For many years, extensive surgery with complete resection was the method of choice for the treatment of craniopharyngioma. Despite the high survival rates, this approach resulted in multiple long-term complications due to dysfunction of the hypothalamic-pituitary axis. In recent years less invasive surgical approaches have been advocated with the aim of reducing long-term morbidity and improving quality of life. In this work, the treatment modalities and their long-term results were analysed in pediatric patients with the diagnosis of craniopharyngioma, treated at Hospital de Santa Maria (HSM-CHLN) between January 2000 and December 2017. From the analysis of this series we concluded that our cohort has a similar epidemiology when compared to other series in the literature. The clinical presentation, radiological and histological tumor characteristics observed are also similar to those described in the literature. As in other centers in Europe and North America, the treatment of craniopharyngioma at Hospital de Santa Maria has been changing to a more conservative approach.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2018
Palavras-chave
Craniofaringioma Epidemiologia Tratamento cirúrgico Resultados a longo prazo Neurocirurgia