A Case of Diffuse Multicentric Metachronous Astrocytoma of Temporoinsular and Intramedullary Location

Multiple gliomas are rare glial tumors with a histology that is typically consistent with high-grade gliomas. A distinction is made between multifocal and multicentric gliomas according to criteria of anatomical continuity, as well as between synchronous and metachronous gliomas according to chronological time of onset. We present the case of a professional saxophonist with a left temporoinsular lesion who underwent awake craniotomy with monitoring of verbal and musical ability as well as primary sensory and motor cortices. Histopathological study revealed an isocitrate dehydrogenase 1 (IDH)-mutant diffuse astrocytoma. After 4 years of complete oncological remission, the patient developed impaired proprioception in all four extremities. An intramedullary lesion was detected at the level of C4 consistent with an IDH wild-type diffuse astrocytoma. We highlight the singularity of this case as it involved two low-grade glial lesions, separated in time (metachronous) and location (multicentric), as well as genetic differences between both lesions (IDH mutant and wild type).

Hemorragia cerebral espontânea como manifestação inicial de glioma multicêntrico / Spontaneous cerebral haemorrhage as an initial manifestation of multicentric glioma

Introdução / Objetivos: Os gliomas multicêntricos são neoplasias malignas raras e importantes no diagnóstico diferencial de lesões cerebrais múltiplas. São representados por lesões múltiplas, localizadas em lobos ou hemisférios cerebrais distintos, com ausência de contiguidade entre as lesões e de vias de disseminação anatômica ou formações satélites. Apresentam manifestação clínica heterogênea a depender da área cometida. A ressonância magnética é o recurso utilizado para investigação diagnóstica, onde pode-se avaliar a ausência de contiguidade entre as lesões. O objetivo do presente estudo é relatar o caso de um paciente adulto portador de glioma multicêntrico, cuja manifestação inicial se deu após episódio de hemorragia tumoral. Relato de caso: paciente masculino de 62 anos, sem diagnóstico de lesão cerebral conhecida, foi diagnosticado com hemorragia cerebral espontânea. Após algumas semanas, tal hemorragia revelou tratar-se de glioma multicêntrico, confirmado por biopsia. Resultados: após a biopsia, com a confirmação do diagnóstico, o paciente foi encaminhado para tratamento adjuvante. No entanto, faleceu por progressão da doença. Conclusão: Os gliomas multicêntricos são raros e apresentam sintomatologia semelhante aos demais processos neoplásicos cerebrais, sendo que manifestações hemorrágicas são incomuns nesse tipo de neoplasia.Descritores: Glioma, Glioblastoma, Neoplasias encefálicas, Diagnóstico por imagemAbstractIntroduction / Purpose: Multicentric gliomas are rare malignant neoplasms and important in the differential diagnosis of multiple brain lesions. They are represented by multiple lesions, located in distinct lobes or cerebral hemispheres, with absence of contiguity between the lesions and of anatomical dissemination pathways or satellite formations. They present heterogeneous clinical manifestation depending on the committed area. Magnetic resonance imaging is the resource used for diagnostic investigation, in which the absence of contiguity between the lesions can be evaluated. The objective of this paper is to report the case of an adult patient with multicentric glioma, whose initial manifestation occurred after an episode of tumor hemorrhage. Case report: A 62-year-old male patient, without a known brain injury diagnosis, was diagnosed with spontaneous cerebral hemorrhage. After a few weeks, such bleeding revealed to be multicenter, biopsy-confirmed glioma. Results: After biopsy, with confirmation of diagnosis, the patient was referred for adjuvant treatment. However, he died of disease progression. Conclusion: Multicentric gliomas are rare and present similar symptomatology to other brain neoplastic processes, and hemorrhagic manifestations are uncommon in this type of neoplasia.Keywords: Glioma, Glioblastoma, Brain Neoplasms, Diagnostic Imaging

Metachronous, multicentric glioma of pilocytic astrocytoma with oligodendroglioma-like component and oligodendroglioma through distinct genetic aberrations

This patient presented with a rare case of metachronous, multicentric gliomas first manifesting as headache and nausea in 1983 when he was an 8-year-old boy. Computed tomography revealed a cerebellar tumor and the tumor was subtotally resected. The histological diagnosis was pilocytic astrocytoma, and radiation therapy to the posterior fossa and chemotherapy consisting of nimustine hydrochloride and fluorouracil were performed. In 1989, at age 14 years, the patient presented with local recurrence. He underwent gross-total resection of the tumor, and histological examination revealed that the tumor consisted of classic pilocytic astrocytoma with a biphasic pattern and a small oligodendroglioma-like component. In 2011, at age 36 years, he presented with seizure. Magnetic resonance imaging revealed a mass lesion in the right middle frontal gyrus. Gross-total resection of the tumor was performed, and the histological diagnosis was oligodendroglioma. Genetic analyses revealed amplification of the BRAF gene in both the primary cerebellar pilocytic astrocytoma and the recurrent tumor with biphasic features, as well as a BRAF V600E missense mutation in the oligodendroglioma-like component. On the other hand, the IDH1 R132H mutation, instead of aberrations of the BRAF gene, was identified in the oligodendroglioma arising in the right frontal lobe. Different types of aberrations of the BRAF gene in the classic and oligodendroglioma-like component in the recurrent pilocytic astrocytoma suggest that they had different cell origins or that amplification of BRAF was negatively selected under the de novo BRAF V600E mutation. In addition, the aberration profiles of IDH1 and BRAF suggest that the oligodendroglioma arose independent of cerebellar pilocytic astrocytoma.