Epithelioid glioblastoma presenting as multicentric glioma: A case report and review of the literature

Background: Epithelioid glioblastoma is a rare aggressive variant of glioblastoma multiforme (GBM), which was formally recognized by the World Health Organization classification of the central nervous system in 2016. Clinically, epithelioid GBMs are characterized by aggressive features, such as metastases and cerebrospinal fluid dissemination, and an extremely poor prognosis. A rare case of epithelioid GBM that was discovered as a multicentric glioma with different histopathology is reported. Case Description: A 78-year-old man was admitted to our hospital with mild motor weakness of the right leg. Neuroimaging showed small masses in the left frontal and parietal lobes on magnetic resonance imaging. The abnormal lesion had been increasing rapidly for 3 weeks, and a new lesion appeared in the frontal lobe. 11C-methionine positron emission tomography (PET) showed abnormal uptake corresponding to the lesion. To reach a definitive diagnosis, surgical excision of the right frontal mass lesion was performed. Histological findings showed diffuse astrocytoma. Only radiotherapy was planned, but the left frontal and parietal tumors progressed further within a short period. Therefore, it was thought that these tumors were GBM, and a biopsy of the left parietal tumor was performed. The histological diagnosis was epithelioid GBM. Immunohistochemistry showed that most tumor cells were negatively stained for p53 and isocitrate dehydrogenase 1. BRAF V600E mutations were not identified, but TERT promoter mutations were identified. Immediately after surgery, the patient was given chemotherapy using temozolomide, extended local radiotherapy and then bevacizumab. After 6 months, he showed no signs of recurrence. Conclusion: Epithelioid GBM is one of the rarest morphologic subtypes of GBM and has a strongly infiltrative and aggressive nature. Therefore, careful identification of preoperative imaging studies and detailed evaluation of genetic studies are necessary to select the appropriate treatment for epithelioid GBM.

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