CASE REPORT

Astrocitoma Difuso de Mesencéfalo e Diencéfalo: um relato de caso de localização rara

Diffuse Astrocytoma in Mesencephalon and Diencephalon: a case report of rare localization

  • Luís Irajá Nogueira de Sá Neto (1)
  • Letícia Gusso Scremin (2)
  • Silvia Cristiane Gusso Scremin (3)
  • Augusto Amato Neto (4)
  • Amylcar Edemilson Dvilevicius (5)
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Resumo

O astrocitoma difuso grau II, IDH-mutante, é um tumor de crescimento relativamente lento com um tempo médio de sobrevida de 3,9 a 10,8 anos. Contudo, sua infiltração no parênquima cerebral dificulta a ressecção total, cuja taxa bruta é em apenas 14 a 17% dos casos. Estes tumores apresentam desdiferenciação para tumores de alto grau com facilidade, com alto índice de mortalidade. A localização em tronco e diencéfalo constitui evento raro, visto que 90% das manifestações são supratentoriais. Há uma suscetibilidade genética ao desenvolvimento dos astrocitomas de baixo grau, e em 66% destes casos há mutações no gene p53. Os fatores de mau prognóstico destes tumores são: idade avançada, status de desempenho de Karnofsky (KPS) <70, déficits neurológicos anteriores, tumor maior que 6 cm, tumor cruzando a linha média e tumores não hemisféricos. Apresentamos relato de caso de paciente feminina, 54 anos, que procurou atendimento por quadro de cefaleia intensa e progressiva hemicraniana direita, tipo pulsátil, de início recente, associado a vertigem e soluços, sem outros déficits neurológicos focais. Posterior progressão do quadro clínico com estrabismo convergente e diplopia, disartria severa, disfagia grave, paresia grau 2 de predomínio proximal em dimídio direito, tremor em repouso, clônus esgotável em membros inferiores e Babinski positivo. Exames de neuroimagem com evidência de lesão glial em tronco e diencéfalo, com predomínio mesencefálico. A localização em tronco cerebral inviabiliza a total ressecção da lesão dado conjunto de estruturas nobres locais. Desta forma, a paciente é submetida à biópsia sendo evidenciado astrocitoma difuso IDH-mutante grau II (OMS). Apesar dos avanços no tratamento dos gliomas difusos, com estudos comparativos mostrando ganho de 5,5 anos de sobrevida com associação de quimioterapia e radioterapia, não há terapias curativas, podendo haver progressão tumoral e desfechos desfavoráveis.

Palavras-chave

Astrocitoma difuso; Astrocitoma de diencéfalo; Glioma de linha média

Abstract

Grade II diffuse astrocytoma (IDH-mutant) is a relatively slow-growing tumor with median survival time of 3.9 to 10.8 years. However, its infiltration into the brain parenchyma makes total resection difficult, and gross rate is only 14 to 17% of cases. These tumors dedifferentiate to high-grade tumors easily, with a high mortality rate. The location in the brainstem and diencephalon is a rare event, since 90% of the manifestations are supratentorial. There is a genetic susceptibility to the development of low-grade astrocytomas, and in 66% of these cases there are mutations in the p53 gene. Poor prognostic factors for these tumors are advanced age, Karnofsky performance status (KPS) <70, previous neurological deficits, tumor larger than 6 cm, tumor crossing the midline, and non-hemispheric tumors. We present a case report of a 54-year-old female patient who was presented due to a recent onset of intense and progressive right hemicranial headache, pulsatile, associated with vertigo and hiccups, without other focal neurological deficits. Later, progression of the clinical picture with convergent strabismus and diplopia, severe dysarthria, severe dysphagia, grade 2 paresis predominantly in the proximal right side, resting tremor, exhaustible clonus in the lower limbs, positive Babinski. Neuroimaging exams with evidence of glial lesion in the brainstem and diencephalon, with mesencephalic predominance. The location in the brainstem makes the total resection of the lesion impossible, given the set of local noble structures, in this way, the patient is submitted to a biopsy, which shows diffuse IDH-mutant astrocytoma grade II (WHO). Despite advances in the treatment of diffuse gliomas, with comparative studies showing a 5.5-year gain in survival with the combination of chemotherapy and radiotherapy, there are no curative therapies, and there may be tumor progression and unfavorable outcomes.

Keywords

Diffuse astrocytoma; Diencephalon astrocytoma; Midline glioma

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1 MD, MR, Medical Residency Program of Neurosurgery, Neurosurgery Service, Angelina Caron Hospital, Campina Grande do Sul, PR, Brazil.

2 Medical student, Medicine Course, Positivo University, Curitiba, PR, Brazil.

3 MD, Radiologist, Angelina Caron Hospital, Campina Grande do Sul, PR, Brazil.

4 MD, Radiologist, UNICAMP, Campinas, SP, Brazil.

5 MD, Neurosurgeon, Chairman of Neurosurgery Service, Angelina Caron Hospital, Campina Grande do Sul, PR, Brazil.

 

Received May 2, 2022

Accepted May 18, 2022

JBNC  Brazilian Journal of Neurosurgery

  •   ISSN (print version): 0103-5118
  •   e-ISSN (online version): 2446-6786

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