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Advisor(s)
Abstract(s)
O Glioblastoma Multiforme (GBM) é o tumor maligno mais comum e agressivo encontrado
no sistema nervoso central, altamente letal com uma taxa média de sobrevida de menos de
15 meses e grande recorrência. São tumores cerebrais intrínsecos originários de célulastronco neurogliais ou células progenitoras. Mais de 90% dos glioblastomas são tumores do
tipo IDH (isocitrato desidrogenase) wild type. A incidência aumenta com a idade e os
homens são mais afetados. Apesar de alguns casos raros de predisposição genética e
exposição à irradiação, não há fatores de risco conhecidos para este tipo de tumor.
Desde 2005 que o tratamento para o GBM é uma abordagem multidisciplinar que envolve
cirurgia, radioterapia e quimioterapia (nomeadamente com temozolomida). No entanto, é
importante notar que o tratamento do GBM ainda é um grande desafio clínico devido à
agressividade do tumor, à sua natureza infiltrativa e à sua tendência em recidivar.
É importante notar que, apesar dos esforços significativos na pesquisa e no
desenvolvimento de novas terapias, o GBM continua também sendo um tumor cerebral
desafiador de tratar. A participação em ensaios clínicos pode ser uma opção para alguns
pacientes que desejam acessar tratamentos experimentais em busca de melhores
resultados. A pesquisa continua avançando na busca por tratamentos mais eficazes e,
eventualmente, uma cura para o glioblastoma.
Apresentam-se dois casos clínicos de dois adultos, do sexo masculino, o caso um com
quarenta e dois anos de idade e o caso 2 com cinquenta e dois anos, com diagnóstico clínico
e histológico, de glioblastoma multiforme grau 4 em Junho de 2019 e Julho de 2016
respetivamente. O sintoma inicial do caso 1 foi a sonolência excessiva, que impactava a vida
profissional do paciente. A zona cerebral atingida foi o lobo frontal, o que levou a alterações
de comportamento ao longo da progressão do tumor, nomeadamente de cariz sexual. O
paciente apresentava ecolalia severa e manteve este tipo de conduta até aparecerem
sintomas de falta de controle de esfíncteres, deterioração das funções motoras e falência
multiorgânica. O utente acabaria por falecer na unidade de cuidados paliativos (CP) da
cidade do Fundão a 16 de Janeiro de 2020. No caso 2, o sintoma inicial foi também
sonolência excessiva e apresentou-se no Serviço de Urgência (SU) com cefaleias
holocranianas e vómitos. A zona acometida inicialmente foi o lobo parietal, sem grande
sintomatologia ao longo de sua evolução. Foi submetido a várias cirurgias e tratamentos,
tendo vindo a falecer no domicílio a 02 de Abril de 2021. Com a presente revisão bibliográfica pretende-se reunir a informação atualizada sobre os
aspetos mais relevantes relacionados com os glioblastomas, especialmente em estadio 4 e
apresentar dois casos clínicos da patologia em questão, com evoluções distintas.
O objetivo desta dissertação é aumentar e promover o conhecimento científico
relativamente a esta patologia agressiva ainda sem tratamento de intuito curativo, através
destes casos clínicos reais. Pretende-se também fomentar a atenção dada aos doentes, pela
vasta e variada apresentação dos sintomas desta patologia.
Para suporte teórico vai ser realizada uma revisão bibliográfica, através de bases de dados
Pub MED com as palavras-chave “glioblastoma multiforme”, “malignidade”, “tumor
cerebral primário”, “mortalidade”, “nova classificação” podendo incluir outras fontes de
informação como por exemplo o site da WHO e revistas de neurologia.
Glioblastoma multiforme (GBM) is the most common and aggressive malignant tumor found in the central nervous system, highly lethal with an average survival rate of less than 15 months and high recurrence. They are intrinsic brain tumors originating from neuroglial stem cells or progenitor cells. More than 90% of glioblastomas are IDH (isocitrate dehydrogenase) wild-type tumors. The incidence increases with age and men are more affected. Despite some rare cases of genetic predisposition and exposure to irradiation, there are no known risk factors for this type of tumor. Since 2005, treatment for GBM has been a multidisciplinary approach involving surgery, radiotherapy and chemotherapy (namely temozolomide). However, it is important to note that the treatment of GBM is still a major clinical challenge due to the aggressiveness of the tumor, its infiltrative nature and its tendency to recur. It is important to note that despite significant efforts in research and development of new therapies, GBM also remains a challenging brain tumor to treat. Participation in clinical trials may be an option for some patients who wish to access experimental treatments in search of better results. Research continues to advance in the search for more effective treatments and, eventually, a cure for glioblastoma. Two clinical cases of two male adults are presented, case one at forty-two years of age and case 2 at fifty-two years of age, with a clinical and histological diagnosis of grade 4 glioblastoma multiforme in June 2019 and July 2016 respectively. The initial symptom of case 1 was excessive drowsiness, which impacted the patient's professional life. The brain area affected was the frontal lobe, which led to behavioral changes throughout the progression of the tumor, particularly of a sexual nature. The patient had severe echolalia and continued this type of behavior until symptoms of lack of sphincter control, deterioration of motor functions and multiorgan failure appeared. The user ended up dying in the palliative care unit in the city of Fundão on January 16, 2020. In case 2, the initial symptom was also excessive drowsiness and he presented to the Emergency Department with holocranial headaches and vomiting. The initially affected area was the parietal lobe, without major symptoms throughout its evolution. He underwent several surgeries and treatments and died at home on April 2, 2021. With this bibliographical review, we intend to gather updated information on the most relevant aspects related to glioblastomas, especially in stage 4, and to present two clinical cases of the pathology in question, with distinct evolutions. The objective of this dissertation is to increase and promote scientific knowledge regarding this aggressive pathology that still has no curative treatment, through these real clinical cases. It is also intended to encourage the attention given to patients, due to the vast and varied presentation of the symptoms of this pathology. For theoretical support, a bibliographical review will be carried out, using Pub MED databases with the keywords “glioblastoma multiforme”, “malignancy”, “primary brain tumor”, “mortality”, “new classification” and may include other sources of information, such as the WHO website and neurology journals.
Glioblastoma multiforme (GBM) is the most common and aggressive malignant tumor found in the central nervous system, highly lethal with an average survival rate of less than 15 months and high recurrence. They are intrinsic brain tumors originating from neuroglial stem cells or progenitor cells. More than 90% of glioblastomas are IDH (isocitrate dehydrogenase) wild-type tumors. The incidence increases with age and men are more affected. Despite some rare cases of genetic predisposition and exposure to irradiation, there are no known risk factors for this type of tumor. Since 2005, treatment for GBM has been a multidisciplinary approach involving surgery, radiotherapy and chemotherapy (namely temozolomide). However, it is important to note that the treatment of GBM is still a major clinical challenge due to the aggressiveness of the tumor, its infiltrative nature and its tendency to recur. It is important to note that despite significant efforts in research and development of new therapies, GBM also remains a challenging brain tumor to treat. Participation in clinical trials may be an option for some patients who wish to access experimental treatments in search of better results. Research continues to advance in the search for more effective treatments and, eventually, a cure for glioblastoma. Two clinical cases of two male adults are presented, case one at forty-two years of age and case 2 at fifty-two years of age, with a clinical and histological diagnosis of grade 4 glioblastoma multiforme in June 2019 and July 2016 respectively. The initial symptom of case 1 was excessive drowsiness, which impacted the patient's professional life. The brain area affected was the frontal lobe, which led to behavioral changes throughout the progression of the tumor, particularly of a sexual nature. The patient had severe echolalia and continued this type of behavior until symptoms of lack of sphincter control, deterioration of motor functions and multiorgan failure appeared. The user ended up dying in the palliative care unit in the city of Fundão on January 16, 2020. In case 2, the initial symptom was also excessive drowsiness and he presented to the Emergency Department with holocranial headaches and vomiting. The initially affected area was the parietal lobe, without major symptoms throughout its evolution. He underwent several surgeries and treatments and died at home on April 2, 2021. With this bibliographical review, we intend to gather updated information on the most relevant aspects related to glioblastomas, especially in stage 4, and to present two clinical cases of the pathology in question, with distinct evolutions. The objective of this dissertation is to increase and promote scientific knowledge regarding this aggressive pathology that still has no curative treatment, through these real clinical cases. It is also intended to encourage the attention given to patients, due to the vast and varied presentation of the symptoms of this pathology. For theoretical support, a bibliographical review will be carried out, using Pub MED databases with the keywords “glioblastoma multiforme”, “malignancy”, “primary brain tumor”, “mortality”, “new classification” and may include other sources of information, such as the WHO website and neurology journals.
Description
Keywords
Glioblastoma Multiforme Malignidade Mortalidade Nova Classificação Tumor Cerebral Primário