Abstract

Introduction Glioblastoma multiforme (GBM), the most common primary malignant brain tumor in adults, is characterized by extensive heterogeneity in its clinicopathological presentation. A primary brain tumor with both astrocytic differentiation and neuronal immunophenotype features is rare. Here, we report a long-term survival patient who presented this rare form of GBM in the disease course. Presentation of Case A 23-year-old woman, presenting with rapidly progressive headache and right-side weakness, was diagnosed with brain tumor over the left basal ganglion. She underwent the first craniectomy for tumor removal, and histopathology revealed classic GBM. Tumor recurrence occurred 8 years later. Another gross total resection was performed and pathology revealed GBM with the oligodendroglioma component (GBM-O). Due to disease progression, she received debulking surgery the following year. The third pathology revealed glioblastoma with primitive neuroectodermal tumor-like component (GBM-PNET). Discussion GBM-PNETs are collision tumors with both neuronal and glial components. They are rare, and a few case reports have suggested that these tumors are associated with favorable outcomes but a higher risk of cerebrospinal fluid dissemination. Conclusion We report a patient who developed the distinct pathologic variants of classic GBM, GBM-O, and GBM-PNET, throughout the disease course. Young age, aggressive surgical resection, and pathologic and genetic features may have contributed to the long-term survival of the patient.

Highlights

  • Glioblastoma multiforme (GBM), the most common primary malignant brain tumor in adults, is characterized by extensive heterogeneity in its clinicopathological presentation

  • GBM shows a great degree of histological variability and numerous morphologic subtypes, some of which may be associated with specific genetic alterations or clinical behaviors [2]

  • In contrast to GBMs, those previously designated as PNETs, incorporated into the term embryonal tumor with multilayered rosettes (ETMR) in the 2016 WHO classification system for CNS tumors, are aggressive neoplasms, with medulloblastoma-like histology, and mostly affect the pediatric population

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Summary

Introduction

Glioblastoma multiforme (GBM) is the most malignant form of astrocytoma and the most frequent primary tumor of the brain in adults. With a median survival of 12.1–14.6 months despite current multimodal treatment [1]. GBM shows a great degree of histological variability and numerous morphologic subtypes, some of which may be associated with specific genetic alterations or clinical behaviors [2]. Glioblastoma with primitive neuronal component, which has been referred to as glioblastoma with the primitive neuroectodermal tumor-like component (GBM-PNET) in the literature and which is one of the emerging GBM subtypes, is a rare tumor with the combined features of malignant glioma and PNET. We report a long-term survival patient who presented this rare collision tumor and different histopathological components in the disease course

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