Glioblastoma Multiforme in the Posterior Cranial Fossa in a Patient with Neurofibromatosis Type I

Broekman, Marike L. D.; Risselada, Roelof; Engelen-Lee, Joo-Yeon; Spliet, Wim G.M.; Verweij, Bon H.

doi:10.1155/2009/757898

Cited by 21 publications

(22 citation statements)

References 23 publications

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“…Glioblastoma multiforme may also occur in the course of genetic diseases: tuberous sclerosis [ 15 ], Turcot syndrome [ 16 ], multiple endocrine neoplasia type IIA [ 17 ] and neurofibromatosis type I, NF1 [ 18 ]. In addition, acquired head injuries, which occurred as a result of a brain contusion, may predispose to the onset of glioblastoma [ 19 , 20 ].…”

Section: Etiologymentioning

confidence: 99%

Review Glioblastoma multiforme – an overview

Urbańska¹,

Sokołowska²,

Szmidt³

et al. 2014

225

217

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Glioblastoma multiforme is a central nervous system tumor of grade IV histological malignancy according to the WHO classification. Over 90% of diagnosed glioblastomas multiforme cases are primary gliomas, arising from normal glial cells through multistep oncogenesis. The remaining 10% are secondary gliomas originating from tumors of lower grade. These tumors expand distinctly more slowly. Although genetic alterations and deregulations of molecular pathways leading to both primary and secondary glioblastomas formation differ, morphologically they do not reveal any significant differences. Glioblastoma is a neoplasm that occurs spontaneously, although familial gliomas have also been noted. Caucasians, especially those living in industrial areas, have a higher incidence of glioblastoma. Cases of glioblastoma in infants and children are also reported. The participation of sex hormones and viruses in its oncogenesis was also suggested. Progression of glioblastoma multiforme is associated with deregulation of checkpoint G1/S of a cell cycle and occurrence of multiple genetic abnormalities of tumor cells. Metastases of glioblastoma multiforme are rarely described. Treatment of glioblastoma multiforme includes tumor resection, as well as radiotherapy and chemotherapy. Drugs inhibiting integrin signaling pathways and immunotherapy are also employed. Treatment modalities and prognosis depend on the tumor localization, degree of its malignancy, genetic profile, proliferation activity, patient's age and the Karnofsky performance scale score. Although the biology of glioblastoma multiforme has recently been widely investigated, the studies summarizing the knowledge of its development and treatment are still not sufficient in terms of comprehensive brain tumor analysis.

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Section: Etiologymentioning

confidence: 99%

Review Glioblastoma multiforme – an overview

Urbańska¹,

Sokołowska²,

Szmidt³

et al. 2014

225

217

View full text Add to dashboard Cite

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“…The study suggested close follow-up of brain lesions in NF1 patients for potential development of high-grade gliomas. The Patient received standard surgical and chemo-radiational therapy but showed signs of tumor metastasis in right frontal lobe and died 6 months after the treatment [26]. In another report from India, a 6year-old NF1 patient was diagnosed with cerebellar GBM and despite surgical intervention and chemoradiotherapy died after 4 months [27].…”

Section: Discussion and Evaluationsmentioning

confidence: 97%

Cerebellar glioblastoma multiforme in an adult patient with neurofibromatosis type 1: an extremely rare report with review of literature

Derakhshan

Azadeh

Saffarian

et al. 2019

Egypt J Neurol Psychiatry Neurosurg

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Background: Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder with autosomal dominant inheritance which predisposes the affected individuals to increased risk of developing certain benign and malignant central nervous system (CNS) tumors. NF1 patients are most notably prone to develop low-grade optic pathway, brainstem, and cerebellar astrocytoma. Current literature suggests that brain tumors in patients with NF1 tend to be less aggressive compared to sporadic ones. Glioblastoma multiforme (GBM) is a high-grade glioma which is relatively rare in patients with NF1 and is most commonly seen in supratentorial regions of the brain. Case presentation: A 33-year-old patient was admitted in neurosurgery ward with acute hydrocephalus caused by a cerebellar mass lesion. On primary assessment, the patient was diagnosed with NF1. He was followed for 2 months and underwent surgical resection of the mass due to worsening symptoms. The pathology report revealed the malignant nature of the lesion. Patient received adjuvant chemo-radiotherapy with diagnosis of cerebellar GBM. Up to 19 months following surgery, he had gained a relatively well ability to walk and talk again.

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“…NF1 confers at least a fivefold increased risk for developing gliomas, including World Health Organization (WHO) grade IV astrocytomas (such as glioblastomas) (Fig 10) (11,59). Glioblastomas usually occur in young adults, in whom the overall prognosis is poor.…”

Section: Neurofibromatosis Typementioning

confidence: 99%

Genetic Syndromes Associated with Central Nervous System Tumors

Vijapura¹,

Aldin²,

Capizzano³

et al. 2017

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Several genetic tumor syndromes have associated central nervous system (CNS) neoplasms. The spectrum of syndromes that have intracranial tumor manifestations includes ataxia telangiectasia, Cowden syndrome, familial adenomatous polyposis, hereditary non-polyposis-related colorectal cancer, Li-Fraumeni syndrome, Gorlin syndrome, neurofibromatosis types 1 and 2, multiple endocrine neoplasia type 1, tuberous sclerosis complex, von Hippel-Lindau disease, and Turcot syndrome. Many of these disorders are inherited in an autosomal dominant fashion, and identification of the associated genetic defects has led to improved understanding of the molecular pathways involved in tumorigenesis, helping pave the way to the emergence of molecularly targeted therapeutics. Recognition of individuals and families at risk for such tumors is critical to improve clinical care and optimize proper genetic counseling. To contribute effectively, the radiologist should recognize the common varieties of tumors and characteristic neuroimaging manifestations seen in each familial syndrome. A fundamental understanding of the genetics and molecular pathogenesis of these tumors is critical in understanding the development of specific and unique tumors in each entity. In this article, we review the most common genetic tumor syndromes with associated intracranial neoplasms, with emphasis on recent genetic and molecular biology data, clinical manifestations, and management as well as the controversies and current recommendations for screening and surveillance. A detailed overview of all the major and pertinent CNS imaging features will be elucidated, including computed tomography, magnetic resonance imaging, and, in relevant cases, magnetic resonance spectroscopy. RSNA, 2016.

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Glioblastoma Multiforme in the Posterior Cranial Fossa in a Patient with Neurofibromatosis Type I

Cited by 21 publications

References 23 publications

Review Glioblastoma multiforme – an overview

Review Glioblastoma multiforme – an overview

Cerebellar glioblastoma multiforme in an adult patient with neurofibromatosis type 1: an extremely rare report with review of literature

Genetic Syndromes Associated with Central Nervous System Tumors

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