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]

• Suggestibility

  The presence of occasional astrocytic cell processes covered by basal laminae strongly suggests that some of the constituent astrocytic cells were of subpial astrocytic origin. [ncbi.nlm.nih.gov]

• 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

  […] impairments, or if epileptic spasms or generalized seizure types are seen. [epilepsydiagnosis.org]

  Our patient presented several risk factors: generalized seizures, lower age of onset of seizures, duration of seizures longer than 10 years, age between 20 and 40 years and a poorly controlled disorder. [jmedicalcasereports.biomedcentral.com]

  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]

  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] Simple DNTs more frequently manifest generalized 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 [en.wikipedia.org]

• Gait Ataxia

  On neurologic examination, the only abnormality was gait ataxia. Magnetic resonance imaging (MRI) demonstrated a midbrain tumor involving the tectum. It was hypointense on T1-weighted images and featured an iso- to hyperintense nodule at its center. [ncbi.nlm.nih.gov]

  The post-operative course was characterised by VI cranial nerve palsy with diplopia and gait ataxia with an improvement of these troubles at three months follow-up. [giornalechirurgia.it]

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]

• Lymphocytic Infiltrate

  They show neoplastic ganglion cells and perivascular lymphocytic infiltrate. [ncbi.nlm.nih.gov]

  In order to exclude any confusion of nonspecific histological form of DNTs with gangliogliomas, we excluded from our study any tumor with lymphocytic infiltrates, which are typically absent in DNTs, but nearly always present in gangliogliomas (see discussion [em-consulte.com]

• 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]

Surgical excision is the most common approach to the treatment of dysembryoplastic neuroepithelial tumor. [wikidoc.org]

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]

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]

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]

[Figure 1], [Figure 2], [Figure 3] This article has been cited by 1 Dysembryoplastic Neuroectodermal Tumor: An Analysis from the Surveillance, Epidemiology, and End Results Program, 2004–2013 World Neurosurgery. 2017; 103: 380 [Pubmed] | [DOI] [neurologyindia.com]

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]

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]

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]

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]

After the surgical removal of this tumor (arrow) shown on the above left image, the patient's troubling seizures went away, and the medications taken to prevent them were successfully discontinued. [stechisonneurosurgery.com]

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]