Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy

Yang, Andrew; Khawaja, Ayaz; Ballester-Fuentes, Leo; Pack, Svetlana D.; Abdullaev, Ziedulla; Patronas, Nicholas J.; Inati, Sara K.; Theodore, William H.; Quezado, Martha; Zaghloul, Kareem A.

doi:10.1684/epd.2014.0680

Cited by 15 publications

(5 citation statements)

References 11 publications

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“…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: 47%

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

Mohammadi

Moradi

Rahimian

et al. 2020

Int Clin Neurosci J

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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.

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Section: Discussionsupporting

confidence: 47%

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

Mohammadi

Moradi

Rahimian

et al. 2020

Int Clin Neurosci J

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“…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

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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.

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“…Multifocality is also an atypical presentation for DNETs, as these tumors tend to be solitary. However, there have been rare case reports of multifocal DNETs [8,[12][13][14][15][16][17], which was first described by Leung et al in 1994 [13]. Furthermore, multifocal presentation has been reported in association with hereditary conditions such as Jacob's syndrome (47,XYY) [14] or neurofibromatosis type 1 (NF1) [18].…”

Section: Discussionmentioning

confidence: 99%

A Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature

Tuohy¹,

Lane²,

Abendroth³

et al. 2022

Cureus

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Dysembryoplastic neuroepithelial tumors (DNETs) are rare, generally benign, mixed neuronal-glial neoplasms occurring most often between 10 and 14 years of age. These lesions are classically cortically based and solitary, found preferentially in the temporal lobe, and most commonly present with seizures. On magnetic resonance imaging (MRI), these lesions are generally cystic and have variable contrast enhancement, which, when present, often involves the periphery. Rarely, lesions followed radiographically may demonstrate delayed contrast enhancement. Here, we present a case of multifocal DNETs involving the cerebellum that demonstrated delayed contrast enhancement. In addition, these occurred in a patient with Noonan syndrome (NS), a "RASopathy" disorder associated with low-grade glial and glioneuronal tumors. We present a summary of all previously reported cases of cerebellar DNETs. Our patient was successfully treated surgically and is doing well clinically, now one year status post his last procedure, and is being closely monitored with serial MRIs for progression. Gross total resection is often curative without adjuvant therapy for most DNETs. Our case emphasizes the importance of radiographic surveillance, as multifocality and recurrence may necessitate more than one procedure. Lastly, clinicians should be suspicious for DNETs and other low-grade glial tumors when treating patients with NS, acknowledging their predisposition for multifocal involvement and atypical presentations.

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Multifocal dysembryoplastic neuroepithelial tumours associated with refractory epilepsy

Cited by 15 publications

References 11 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

A Constellation of Atypical Findings in a Nine-Year-Old Child With Dysembryoplastic Neuroepithelial Tumors: A Case Report and Review of the Literature

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