Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report

Abstract Background Glioblastoma multiform with primitive neuronal component (GBM-PNC) has been recently defined as a rare variant of glioblastoma multiform (GBM), which shows characteristically pathological pattern of less differentiated areas with small blue cell morphology and neuroectodermic imm...

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Autores principales: Santiago Valbuena, Alejandro Ortega, Macarena Centeno, Jordi Manuel Rimbau
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Publicado: SpringerOpen 2021
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Acceso en línea:https://doaj.org/article/57832d8800f44fe6be56d47c7a61b62c
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spelling oai:doaj.org-article:57832d8800f44fe6be56d47c7a61b62c2021-11-28T12:27:17ZGlioblastoma multiform with primitive neuronal component, radiological and histology features: a case report10.1186/s41984-021-00135-92520-8225https://doaj.org/article/57832d8800f44fe6be56d47c7a61b62c2021-11-01T00:00:00Zhttps://doi.org/10.1186/s41984-021-00135-9https://doaj.org/toc/2520-8225Abstract Background Glioblastoma multiform with primitive neuronal component (GBM-PNC) has been recently defined as a rare variant of glioblastoma multiform (GBM), which shows characteristically pathological pattern of less differentiated areas with small blue cell morphology and neuroectodermic immunophenotype. New studies emphasize its characteristics and differences, which have become vitally important due to the changes in therapeutic management. Case presentation We present the case of 57-year-old male patient who onset symptoms were secondarily widespread partial seizures and expression aphasia. Brain magnetic resonance imaging (MRI) reported left enhanced temporal infiltrating lesion, requiring surgery twice throughout two years. At first surgery, pathological samples revealed embryonic tumor of the central nervous system (grade IV, WHO 2016), so PACKER protocol consisting of CSRT (craniospinal radiation) plus weekly vincristine followed by 8 cycles of cisplatin, lomustine and vincristine usually used for medulloblastomas or other primitive neuroectodermal tumors was started. However, due to reappearance of symptoms and progression in MRI, reoperation was performed with definitive diagnosis of GBM-PNC (Grade IV, WHO 2016) and switched to STUPP protocol. Conclusions It is important to take into account the chance of this entity when histological, radiological and intraoperative findings orient toward a primitive neural tumor since the presence of GBM could be overlooked leading to mistakes in diagnosis and the therapeutic orientation.Santiago ValbuenaAlejandro OrtegaMacarena CentenoJordi Manuel RimbauSpringerOpenarticleGlioblastoma multiformPrimitive neuroectodermal tumorBrain neoplasmNeurosurgeryDifferential diagnosisSurgeryRD1-811Neurosciences. Biological psychiatry. NeuropsychiatryRC321-571ENEgyptian Journal of Neurosurgery, Vol 36, Iss 1, Pp 1-5 (2021)
institution DOAJ
collection DOAJ
language EN
topic Glioblastoma multiform
Primitive neuroectodermal tumor
Brain neoplasm
Neurosurgery
Differential diagnosis
Surgery
RD1-811
Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
spellingShingle Glioblastoma multiform
Primitive neuroectodermal tumor
Brain neoplasm
Neurosurgery
Differential diagnosis
Surgery
RD1-811
Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
Santiago Valbuena
Alejandro Ortega
Macarena Centeno
Jordi Manuel Rimbau
Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
description Abstract Background Glioblastoma multiform with primitive neuronal component (GBM-PNC) has been recently defined as a rare variant of glioblastoma multiform (GBM), which shows characteristically pathological pattern of less differentiated areas with small blue cell morphology and neuroectodermic immunophenotype. New studies emphasize its characteristics and differences, which have become vitally important due to the changes in therapeutic management. Case presentation We present the case of 57-year-old male patient who onset symptoms were secondarily widespread partial seizures and expression aphasia. Brain magnetic resonance imaging (MRI) reported left enhanced temporal infiltrating lesion, requiring surgery twice throughout two years. At first surgery, pathological samples revealed embryonic tumor of the central nervous system (grade IV, WHO 2016), so PACKER protocol consisting of CSRT (craniospinal radiation) plus weekly vincristine followed by 8 cycles of cisplatin, lomustine and vincristine usually used for medulloblastomas or other primitive neuroectodermal tumors was started. However, due to reappearance of symptoms and progression in MRI, reoperation was performed with definitive diagnosis of GBM-PNC (Grade IV, WHO 2016) and switched to STUPP protocol. Conclusions It is important to take into account the chance of this entity when histological, radiological and intraoperative findings orient toward a primitive neural tumor since the presence of GBM could be overlooked leading to mistakes in diagnosis and the therapeutic orientation.
format article
author Santiago Valbuena
Alejandro Ortega
Macarena Centeno
Jordi Manuel Rimbau
author_facet Santiago Valbuena
Alejandro Ortega
Macarena Centeno
Jordi Manuel Rimbau
author_sort Santiago Valbuena
title Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
title_short Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
title_full Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
title_fullStr Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
title_full_unstemmed Glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
title_sort glioblastoma multiform with primitive neuronal component, radiological and histology features: a case report
publisher SpringerOpen
publishDate 2021
url https://doaj.org/article/57832d8800f44fe6be56d47c7a61b62c
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AT alejandroortega glioblastomamultiformwithprimitiveneuronalcomponentradiologicalandhistologyfeaturesacasereport
AT macarenacenteno glioblastomamultiformwithprimitiveneuronalcomponentradiologicalandhistologyfeaturesacasereport
AT jordimanuelrimbau glioblastomamultiformwithprimitiveneuronalcomponentradiologicalandhistologyfeaturesacasereport
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