Multifocal Dysembryoplastic Neuroepithelial Tumour with Intradural Spinal Cord Lipomas: Report of a Case

White, Richard D.; Kanodia, Avinash Kumar; Sammler, Esther; Brunton, John N.; Heath, Craig

doi:10.1155/2011/734171

Cited by 8 publications

(7 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…To the best of our knowledge, there are only seven reports of multifocal DNT in the English literature. [3][4][5][6][7][8][9][10] The present case was mostly cortical with no prominent mass effect and almost unchanged in size and configuration in MRI studies during two years follow up. DNTs are usually cortical tumors with neither mass journals.sbmu.ac.ir/Neuroscience http effect nor significant peritumoral edema, and these are essential differentiating factors between DNTs and diffuse infiltrating gliomas.…”

Section: Discussionsupporting

confidence: 48%

Diffuse Multifocal Bilateral Dysembryoplastic Neuroepithelial Tumor: A Very Unusual Case Report

Mohammadi

Moradi

Rahimian

et al. 2020

Int Clin Neurosci J

View full text Add to dashboard Cite

Dysembryoplastic neuroepithelial tumor (DNT) considered as a benign cortical glioneuronal neoplasm of children or young adults, typically present with drug-resistant focal epilepsy. DNTs are usually located in the temporal lobe but can found in any part of the supratentorial brain cortex. Multifocal DNTs have rarely reported. Here we present an eight years old boy with two years follow up, having a somewhat stable diffuse multinodular DNT of the most significant spatial extent that may have reported, involving cortical and subcortical left temporo-occipital lobe, bilateral basal ganglia and thalamus, presenting with headache, short stature, and behavioral disorder.

show abstract

Section: Discussionsupporting

confidence: 48%

Diffuse Multifocal Bilateral Dysembryoplastic Neuroepithelial Tumor: A Very Unusual Case Report

Mohammadi

Moradi

Rahimian

et al. 2020

Int Clin Neurosci J

View full text Add to dashboard Cite

show abstract

“…Multifocal and cerebellar DNET are rare and based on our review of reported cases, there appears to be a relationship between DNET multifocality, cerebellar location, and presence of an underlying genetic syndrome. Including our patient, there have been 12 multifocal cases reported [Leung et al, ; Lellouch‐Tubiana et al, ; Whittle et al, ; Fujimoto et al, ; Krossnes et al, ; Schittenhelm et al, ; White et al, ; Krishna et al, ; Delisle et al, ; Yang et al, ], seven of which involved the cerebellum [Leung et al, ; Whittle et al, ; Fujimoto et al, ; Krossnes et al, ; Krishna et al, ; Yang et al, ]. Of the 12 multifocal cases, five patients had genetic disorders: one with Klinefelter syndrome, one with NF1, and three with NS [Lellouch‐Tubiana et al, ; Krossnes et al, ; Krishna et al, ; Delisle et al, ].…”

Section: Discussionmentioning

confidence: 99%

Occurrence of DNET and other brain tumors in Noonan syndrome warrants caution with growth hormone therapy

McWilliams

SantaCruz

Hart

et al. 2015

American J of Med Genetics Pt A

View full text Add to dashboard Cite

Noonan syndrome (NS) is an autosomal dominant developmental disorder caused by mutations in the RAS-MAPK signaling pathway that is well known for its relationship with oncogenesis. An 8.1-fold increased risk of cancer in Noonan syndrome has been reported, including childhood leukemia and solid tumors. The same study found a patient with a dysembryoplastic neuroepithelial tumor (DNET) and suggested that DNET tumors are associated with NS. Herein we report an 8-year-old boy with genetically confirmed NS and a DNET. Literature review identified eight other reports, supporting the association between NS and DNETs. The review also ascertained 13 non-DNET brain tumors in individuals with NS, bringing to 22 the total number of NS patients with brain tumors. Tumor growth while receiving growth hormone (GH) occurred in our patient and one other patient. It is unknown whether the development or progression of tumors is augmented by GH therapy, however there is concern based on epidemiological, animal and in vitro studies. This issue was addressed in a 2015 Pediatric Endocrine Society report noting there is not enough data available to assess the safety of GH therapy in children with neoplasia-predisposition syndromes. The authors recommend that GH use in children with such disorders, including NS, be undertaken with appropriate surveillance for malignancies. Our case report and literature review underscore the association of NS with CNS tumors, particularly DNET, and call attention to the recommendation that clinicians treating NS patients with GH do so with awareness of the possibility of increased neoplasia risk.

show abstract

“…Those with multifocal involvement are rare, with only four cases reported in which DNETs occurred in infra-and supra-tentorial locations (Leung et al, 1994;Krossnes et al, 2005;Schittenhelm et al, 2007;White et al, 2011). Typically located in the temporal lobes, DNETs have also been reported within the periventricular white matter, basal ganglia, thalamus, brainstem, and cere-bellum.…”

mentioning

confidence: 99%

Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy

Yang

Khawaja

Ballester-Fuentes

et al. 2014

Epileptic Disorders

View full text Add to dashboard Cite

Dysembryoplastic neuroepithelial tumours (DNET) are a common cause of tumour‐associated epilepsy, and are usually located in the temporal lobes. We present a case of multifocal DNETs in both infra‐ and supra‐tentorial locations, in a 23‐year‐old man with a coincident Type I Chiari malformation, presenting with medically refractory focal seizures. The extensive anatomical distribution of the lesions suggests a genetic component in their tumourigenesis.

show abstract

Multifocal Dysembryoplastic Neuroepithelial Tumour with Intradural Spinal Cord Lipomas: Report of a Case

Cited by 8 publications

References 14 publications

Diffuse Multifocal Bilateral Dysembryoplastic Neuroepithelial Tumor: A Very Unusual Case Report

Diffuse Multifocal Bilateral Dysembryoplastic Neuroepithelial Tumor: A Very Unusual Case Report

Occurrence of DNET and other brain tumors in Noonan syndrome warrants caution with growth hormone therapy

Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy

Contact Info

Product

Resources

About