Symptoma

Dysembryoplastic Neuroepithelial Tumor

DNET

A cystic swelling of the right frontal lobe was present at autopsy, histopathological examination of the swelling revealed to be Dysembryoplastic Neuroepithelial Tumour (DNT). [ncbi.nlm.nih.gov]

  • Epilepsy

    Ganglioglioma and dysembryoplastic neuroepithelial tumor are both low-grade glioneuronal neoplasms that most commonly occur in association with chronic epilepsy. [ncbi.nlm.nih.gov]

  • Fishing

    However, KIAA1549-BRAF fusion gene was identified by fluorescence in situ hybridization (FISH), supporting for the diagnosis of PA. DNT-like PA (WHO grade I). The tumor was totally removed via a right temporal-occipital craniotomy. [ncbi.nlm.nih.gov]

    FISH, Copy Number Variation assays and Sanger sequencing were used to validate the findings. Supporting evidence for functional defects was obtained by in silico modelling of novel FGFR1p.R661P variant. [cancerres.aacrjournals.org]

    Raymond AA, Halpin SF, Alsanjari N, Cook MJ, Kitchen ND, Fish DR, et al: Dysembryoplastic neuroepithelial tumor. Features in 16 patients. Brain 1994;117:461–475. [karger.com]

    Raymond AA, Halpin SFS, Alsamjari N, Cook MJ, Kitchen ND, Fish DR, Stevens JN, Harding BN, Scaravilli F, Kendall B, Shorvon SD, Kendall B, Shorvon SD Neville BGR (1994) Dysembryoplastic neuroepithelial tumor: feature in 16 patients. [link.springer.com]

  • Intravenous Administration

    In 45 cases preoperative CT studies were obtained before and after intravenous administration of contrast medium. Eight patients had only an unenhanced CT examination, but three of them had an enhanced MRI examination. [em-consulte.com]

  • Pseudotumor

    ", keywords "Calcifying pseudotumor, Collision tumor, DNT, Seizure", author "X. Yan and Marsh, {W. R.} and Scheithauer, {B. W. [mayoclinic.pure.elsevier.com]

  • Behavior Disorder

    Case 2 is that of a 10-year-old girl with intractable complex partial seizures and behavioral disorder. Postmortem examination in case 1 showed multifocal tumor in the left temporal lobe, third ventricle, and basal ganglia. [ncbi.nlm.nih.gov]

    Three patients presented with behavior disorders (cases 2, 5, and 8) and two with mental retardation (cases 8 and 9). [ajnr.org]

  • Seizure

    All DNT patients who underwent resection of the epileptogenic cortex associated with lesionectomy became seizure free or achieved a 90% reduction in seizures. [ncbi.nlm.nih.gov]

  • Headache

    Patient 1 experienced headache and worsening dizziness; Patient 2 experienced headache and worsening dizziness and also had partial seizures. [ncbi.nlm.nih.gov]

  • Generalized Seizure

    Twenty-seven patients (93.1%) had complex partial seizures, one (3.44%) had simple partial seizures, and one patient had generalized seizures. [ncbi.nlm.nih.gov]

    seizure types are seen. [epilepsydiagnosis.org]

    The presence of secondary generalized seizures, an extratemporal irritative zone and a structural lesion in extratemporal regions correlate with sudden unexplained death in epilepsy (SUDEP). [jmedicalcasereports.biomedcentral.com]

    All patients had partial seizures, 24 patients also had secondarily generalized seizures and none presented primary generalized seizures. Seizure duration at the time of surgery ranged from one to 37 years (mean 11.4 years). [em-consulte.com]

    seizures. [2] In children, DNTs are considered to be the second leading cause of epilepsy. [3] A headache is another common symptom. [2] Other neurological impairments besides seizures are not common. [2] Pathogenesis [ edit ] Dysembryoplastic neuroepithelial [en.wikipedia.org]

  • Convulsions

    A 3-year-old girl had a generalized convulsion as the initial symptom, without other neurological deficits. [ncbi.nlm.nih.gov]

    Physical examination may be remarkable for: Memory loss Aphasia Jerking movements Convulsions Muscle rigidity Sensory loss Ataxia Laboratory Findings There are no specific laboratory findings associated with dysembryoplastic neuroepithelial tumor. [wikidoc.org]

  • Truncal Ataxia

    A 34-year-old woman with known cystic cerebellar lesions presented with increased frequency of vertigo and new onset of truncal ataxia. [ncbi.nlm.nih.gov]

Five years later, a recurrent tumor at the edge of the resection cavity was removed, and the tissue underwent an intensive diagnostic workup. [ncbi.nlm.nih.gov]

  • Abnormal Spikes

    Abnormal spikes recorded adjacent to the tumor disappeared immediately after total removal. Histological examination showed a multinodular, multicystic structure, satisfying the criteria for the diagnosis of dysembryoplastic neuroepithelial tumor. [ncbi.nlm.nih.gov]

  • White Matter Lesions

    In this report, a rare case of dysembryoplastic neuroepithelial tumor (DNET) initially presented as a small white matter lesion with calcification adjacent to the lateral ventricle and extending to the frontal cortex after 7 years. [ncbi.nlm.nih.gov]

It is suggested that GKRS may be an effective and less invasive alternative treatment for the patients in whom surgical treatment is difficult. [ncbi.nlm.nih.gov]

Children’s Minnesota treats the majority of children with cancer and blood disorders in Minnesota and provides patients access to a variety of clinical trials using ground-breaking new treatments. [childrensmn.org]

CONCLUSION: Unlike diffuse gliomas, such as oligodendrogliomas and central neurocytomas, DNTs are benign lesions with a favorable prognosis after surgical resection. [ncbi.nlm.nih.gov]

Prognosis A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. [braintumour.ca]

Dysembryoplastic neuroepithelial tumors (DNETs) are typically benign World Health Organization (WHO) grade I tumors of the cortical or deep gray matter with a favorable prognosis. [jhu.pure.elsevier.com]

The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors. [ncbi.nlm.nih.gov]

They are frequently found to co-occur with adjacent focal cortical dysplasia (FCD IIIb), suggesting a common developmental etiology for both structural abnormalities. [epilepsydiagnosis.org]

[…] hyperintense on T2, giving a 'bubbly' appearance, and a mixed signal, with a bright rim in the Fluid attenuated inversion recovery images Click here to view A repeat neurologist opinion was sought to rule out the possibility of temporal lobe epilepsy as the etiology [ijpm.info]

The MIB-1 labeling indexes in both components were low ( The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors. [thedoctorsdoctor.com]

Epidemiology DNET represents 1.2% of all neuroepithelial tumors in patients under age 20 and 0.2% of those aged more than 20 years. The peak incidence is in children and young adults in their second and third decades of life. [prod.wiki.cns.org]

Neuroepithelial Tumor from Other Diseases Dysembryoplastic neuroepithelial tumor must be differentiated from other tumors that cause seizures, such as: [2] Pleomorphic xanthoastrocytoma Ganglioglioma Oligodendroglioma Desmoplastic infantile ganglioglioma Epidemiology [wikidoc.org]

Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst. 2001; 17:503–11. [ CrossRef ] [ Google Scholar ] 17. Spalice A, Ruggieri M, Grosso S, et al. [e-sciencecentral.org]

From the epidemiologic point of view, incidence is between six and 35 years old, with an average of 21.5 years and an equal sex distribution. [jmedicalcasereports.biomedcentral.com]

Epidemiology [ edit ] Children are much more prone to exhibit these dysembryoplastic neuroepithelial tumours than adults. [1] The mean age of onset of seizures for children with DNTs is 8.1 years old. [1] Few other neurological deficits are associated [en.wikipedia.org]

Pathophysiology Intracortical tumors: DNETs are intracortically localized tumors, with a relatively sharp margin between tumor and adjacent brain. [ispn.guide]

[…] glioneuronal element (SGNE) Glial nodules and a multinodular architecture Component of associated focal cortical dysplasia is common (Blumcke classification IIIb) Same clinical and neuroimaging features as complex DNE No specific glioneuronal element (SGNE) Pathophysiology [wikidoc.org]

In this case, intraoperative electrocorticography was very useful to identify the possible focus and prevent unnecessary resection of the adjacent tissue. Total removal of the tumor resulted in a dramatic reduction of seizure activity. [ncbi.nlm.nih.gov]

Prevention Primary Prevention There are no known primary preventive measures for dysembryoplastic neuroepithelial tumor. [2] Secondary Prevention Secondary prevention strategies following diagnosis and treatment of dysembryoplastic neuroepithelial tumor [wikidoc.org]

Careful hemostasis after tumor resection is important in prevention. Routine imaging with CT or MRI 24 hours after surgery can allow the timely detection of clinically present but not yet symptomatic hematoma. [ispn.guide]

Earlier intervention can prevent the physical and psychosocial damage resulting from chronic seizures and can improve the prognosis for these young patients. The optimal time for surgical intervention in children with these tumors remains unclear. [astrocyte.in]