Extracranial Metastases From Glioblastoma With Primitive Neuronal Components on FDG PET/CT

Kay, Marguerite M. B.; Pariury, Holly; Perry, Arie; Winegar, Blair A.; Kuo, Phillip H.

doi:10.1097/rlu.0000000000002896

Cited by 15 publications

(17 citation statements)

References 9 publications

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“…Goodwin and colleagues reviewed 28 cases of GBM metastases to the vertebra, with a mean age at presentation of 38.4 years and an average overall survival of 26 months, of which seven patients received surgery for vertebral metastases (34). After a systematic search on Medline, Embase, and Google Scholar, we retrieved 25 cases of GBM-PNC with extracranial metastases from 14 reports (Table 2) (7,16,17,22,(35)(36)(37)(38)(39)(40)(41)(42)(43)(44). CSF dissemination occurred in 88% (22/25) of the patients, whereas only 4 patients (16%) suffered bone metastases.…”

Section: Discussionmentioning

confidence: 99%

“…In dealing with spinal metastasis, early detection can lead to a better prognosis. Therefore, in addition to brain MRI, surveillance MRI of the total spine is recommended, and a further PET/CT under the condition of positive findings on MRI, given the high risk of CSF dissemination in GBM-PNC ( 36 , 62 ). A comprehensive evaluation based on the neurologic, oncologic, mechanical instability, and systemic disease (NOMS) decision framework is indispensable ( 63 ).…”

Section: Discussionmentioning

confidence: 99%

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A Rare Manifestation of a Presumed Non-Osteophilic Brain Neoplasm: Extensive Axial Skeletal Metastases From Glioblastoma With Primitive Neuronal Components

et al. 2021

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BackgroundGlioblastoma multiforme (GBM) is the most common malignant tumor of the central nervous system. GBM with primitive neuronal component (GBM-PNC) is an aggressive variant identified in 0.5% of GBMs. Extracranial metastasis from GBM-PNC is a rare and challenging situation.MethodsA special case of early-onset GBM with systemic bone metastasis was enrolled. Clinical data, including patient characteristics, disease course, and serial radiological images were retrieved and analyzed. Tumor tissues were obtained by surgical resections and were made into formalin-fixed paraffin-embedded sections. Histopathological examinations and genetic testing were performed for both the primary and metastatic tumor specimens.ResultsA 20-year-old man suffered from GBM with acute intratumoral hemorrhage of the left temporal lobe. He was treated by gross total resection and chemoradiotherapy following the Stupp protocol. Seven months later, he returned with a five-week history of progressive neck pain and unsteady gait. The radiographic examinations identified vertebral collapse at C4 and C6. Similar osteolytic lesions were also observed at the thoracolumbar spine, pelvic, and left femur. Anterior spondylectomy of C4 and C6 was performed. The resected vertebral bodies were infiltrated with greyish, soft, and ill-defined tumor tissue. One month later, he developed mechanical low-back pain and paraplegia caused by thoracolumbar metastases. Another spine surgery was performed, including T10 total en-bloc spondylectomy, T7-9, L2-3, and L5-S1 laminectomy. After the operation, the patient’s neurological function and spinal stability remained stable. However, he finally succumbed to the rapidly increased tumor burden and died 15 months from onset because of cachexia and multiple organ failure. In addition to typical GBM morphology, the histological examinations identified monomorphic small-round cells with positive immunohistochemical staining of synaptophysin and CD99, indicating the coexistence of PNC. The next-generation sequencing detected pathogenic mutations in TP53 and DNMT3A. Based on above findings, a confirmed diagnosis of systemic metastases from GBM-PNC (IDH-wild type, WHO grade IV) was made.ConclusionsThe present case highlights the occurrence and severity of extensive axial skeletal metastases from GBM-PNC. This rare variant of GBM requires aggressive multimodal treatment including surgery and chemoradiotherapy targeting PNC. The pathological screening of PNC is recommended in patients with early-onset GBM and intratumoral hemorrhage. Surgery for spinal metastasis is appropriate in patients with chemoradioresistance and relatively good general status, with the objectives of restoring spinal stability and relieving spinal cord compression.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Rare Manifestation of a Presumed Non-Osteophilic Brain Neoplasm: Extensive Axial Skeletal Metastases From Glioblastoma With Primitive Neuronal Components

et al. 2021

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“…14 b No mention of whether there was a decrease or not. 14 ependymal region. Tumor margin characteristics were associated with the survival prognosis.…”

Section: Discussionmentioning

confidence: 99%

“…After excluding 41 articles and including one article extracted from the citation review of each relevant article (no case with pathologically proven DHG-G34m, 25 articles; no radiological findings or images, 14 articles; and insufficient patient-specific information, 2 articles), 12 articles met the selection criteria for the systematic review. 1,[6][7][8][9][10][11][12][13][14][15][16] The year of publication of the selected articles ranged from 2017 to 2021. With the additional three cases from our hospital, the final study cohort included 59 cases of DHG-G34m.…”

Section: Study Selectionmentioning

confidence: 99%

Neuroimaging features of diffuse hemispheric glioma, H3 G34‐mutant: A case series and systematic review

Kurokawa

Baba

Kurokawa

et al. 2021

Journal of Neuroimaging

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Background and Purpose: Diffuse hemispheric gliomas, H3 G34-mutant (DHGs-G34m), are newly recognized malignant brain tumors characterized by histone gene mutations.However, the neuroradiologic characteristics of these tumors require elucidation. We reviewed the demographic, clinical, and neuroradiological features of DHGs-G34m.Methods: Data were extracted using a database search in MEDLINE, SCOPUS, and Google Scholar in June 2021. Studies assessing pathologically proven DHGs-G34m with each patient's information and neuroradiological findings were included. After screening and reviewing 332 abstracts, 12 articles including 56 cases met the criteria. We also added the findings for three patients evaluated in our hospital. Two board-certified radiologists reviewed all demographic, clinical, and neuroradiological findings of each study. One board-certified pathologist reviewed all pathological data of each study. Kaplan-Meier analyses with log-rank tests were performed to compare the survival between patients with different tumor margin characteristics (well-delineated and ill-defined). Results:The median patient age at diagnosis was 19 years (range, 6-66 years), and 31/59 patients (52.5%) were men. Supratentorial tumors were observed in all patients (59/59, 100%). Frequent contact with leptomeninges (92.3%) and ependymal regions (87.5%) was observed. The 1-and 2-year survival rates after initial surgery were 66.7% and 40.0%, respectively. DHGs-G34m with ill-defined and well-delineated margins showed significant differences in survival (p = .04).Conclusions: DHGs-G34m occur most often in the supratentorial regions of adolescents.Prognosis varies among patients. Evaluation of tumor margins may provide prognostic value.

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“…GBM-PNC is a unique variant of GBM at molecular level with high frequency of TP53, PIK3CA, PIK3R1 or PTEN mutation [4]. Unlike GBM, primitive neuronal component (PNC) tumor has a better response to therapy and higher survival rate of approximately 38% in 4 years, although it has an increased risk of spread to cerebrospinal fluid with extracranial metastases [5][6][7]. Its treatment differs from GBM protocol, because after surgical resection needs craniospinal irradiation and platinum base land chemotherapy to prevent dissemination [8].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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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.

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Extracranial Metastases From Glioblastoma With Primitive Neuronal Components on FDG PET/CT

Cited by 15 publications

References 9 publications

A Rare Manifestation of a Presumed Non-Osteophilic Brain Neoplasm: Extensive Axial Skeletal Metastases From Glioblastoma With Primitive Neuronal Components

A Rare Manifestation of a Presumed Non-Osteophilic Brain Neoplasm: Extensive Axial Skeletal Metastases From Glioblastoma With Primitive Neuronal Components

Neuroimaging features of diffuse hemispheric glioma, H3 G34‐mutant: A case series and systematic review

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

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