Diffuse form of dysembryoplastic neuroepithelial tumour: the histological and immunohistochemical features of a distinct entity showing transition to dysembryoplastic neuroepithelial tumour and ganglioglioma

Bódi, István; Selway, Richard; Bannister, P.; Doey, Lawrence J.; Mullatti, Nandini; Elwes, Robert; Honavar, Mrinalini

doi:10.1111/j.1365-2990.2011.01225.x

Cited by 30 publications

(33 citation statements)

References 35 publications

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“…Higher expression levels of nestin, MAP2 and CD34, stem cell markers, in nonspecific rather than simple or complex DNTs suggests an earlier developmental origin of the nonspecific DNTs. 8,30 Because DNTs share CD34 expression, BRAF V600E mutations, and chromosomal copy number profiles with gangliogliomas, and occurrence of composite tumors with ganglioglioma and DNT, both tumors may represent different morphological variants of the same tumor entity. …”

Section: Histogenesismentioning

confidence: 99%

“…5 However, several studies demonstrated three histologic forms of DNTs under the different terms "multinodular, solitary nodular, and diffuse" or "simple, complex, and diffuse." [6][7][8] The frequent immunoreactivity of CD34, nestin, and microtubule-associated protein 2 (MAP2) 9 and the identification of BRAF V600E mutation in nonspecific DNTs 10 suggest that DNTs exhibit a broad spectrum of histopathology from simple to nonspecific forms.A large series of DNTs demonstrated intrinsic epileptogenicity of DNTs in three histologic subtypes by intralesional recordings and simple and complex types showed localization of the epileptogenicity to the tumor, but non-specific temporal DNTs showed more extensive areas with the epileptogenicity. In their series, FCD was found in approximately two-thirds of patients with DNTs and was more common in nonspecific (85%) than in complex types (47%).…”

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confidence: 99%

“…Different expression patterns of nestin and MAP2 in three subtypes of DNTs have also been demonstrated. 8,30 Combined analysis of CD34 and MAP2 is useful in differential diagnosis between nonspecific DNTs and diagnostically challenging mimickers, which will be discussed in the section on differential diagnosis. The Ki-67 labeling index is generally low, below 1% or 2%.…”

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Dysembryoplastic Neuroepithelial Tumors

Keating¹,

Goodrich²,

Packer³

2013

Tumors of the Pediatric Central Nervous System

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Dysembryoplastic neuroepithelial tumor (DNT) is a benign glioneuronal neoplasm that most commonly occurs in children and young adults and may present with medically intractable, chronic seizures. Radiologically, this tumor is characterized by a cortical topography and lack of mass effect or perilesional edema. Partial complex seizures are the most common presentation. Three histologic subtypes of DNTs have been described. Histologically, the recognition of a unique, specific glioneuronal element in brain tumor samples from patients with medically intractable, chronic epilepsy serves as a diagnostic feature for complex or simple DNT types. However, nonspecific DNT has diagnostic difficulty because its histology is indistinguishable from conventional gliomas and because a specific glioneuronal element and/or multinodularity are absent. This review will focus on the clinical, radiographic, histopathological, and immunohistochemical features as well as the molecular genetics of all three variants of DNTs. The histological and cytological differential diagnoses for this lesion, especially the nonspecific variant, will be discussed. Journal of Pathology and Translational Medicine 2015; 49: 438-449 http://dx.doi.org/10.4132/jptm.2015.10.05 ▒ REVIEW ▒ This tumor was originally recognized in patients who underwent surgery for treatment of medically intractable seizures; however, recent progress in neuro-imaging has allowed for increased detection of dysembryoplastic neuroepithelial tumors (DNTs) in patients with a single episode of epilepsy or in older patients. 1 The original report in 1988 by describes morphologically unique features, including intracortical multi-nodularity, a specific glioneuronal (GN) element, and association with focal cortical dysplasia (FCD). The term "dysembryoplastic neuroepithelial tumor" was introduced for this unique tumor because tumors have a number of clinicopathological features, strongly suggesting a dysembryoplastic origin such as early onset of seizure, presence of FCD in the adjacent cortex, and deformity of the overlying skull. 2 The generic term "neuroepithelial tumors" was used so that a large range of morphologic variants could be integrated into this entity. 2The complex type of DNTs was first categorized as neuronal and mixed neuronal-glial tumors in the 1993 World Health Organization (WHO) classification of central nervous system (CNS) tumors. Additionally, simple and nonspecific histologic variants of DNTs have been described. The simple types of DNTs consist of only specific GN elements and the complex types are characterized by specific GN elements associated with glial nodules and/or FCD. Nonspecific types of DNTs lack multinodular architecture and specific GN elements, but show similar histologic findings to those seen in glial nodules of complex DNTs. 3,4 Nonspecific DNTs have been controversial because their histology is indistinguishable from conventional gliomas and because of the lack of a specific GN element and/or multinodularity of typical DNTs. The 2007 WHO ...

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Section: Histogenesismentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Dysembryoplastic Neuroepithelial Tumors

Keating¹,

Goodrich²,

Packer³

2013

Tumors of the Pediatric Central Nervous System

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“…The last form was created based on the statement that the diffuse form of DNT corresponds to a non-specific variant [8,19,29]. However, the diagnosis and definition of the non-specific type of DNT is controversial.…”

Section: Histopathologymentioning

confidence: 99%

Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Sontowska¹,

Matyja²,

Malejczyk³

et al. 2017

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Molecular pathology of paediatric central nervous system tumours

Chiang

Ellison

2016

The Journal of Pathology

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Advances in our understanding of the biology of paediatric central nervous system (CNS) tumours have encouraged pathologists to use molecular markers alongside histopathological analysis for disease classification or prognostication and treatment stratification. In this article, we review molecular genetic alterations in paediatric CNS tumours, including those in low-grade and high-grade gliomas, ependymomas, and embryonal tumours. Some of these molecular changes with clinicopathological utility have been used for the first time in the most recent edition of the World Health Organization (WHO) classification of CNS tumours to define entities like ependymoma, RELA fusion-positive or diffuse midline glioma, H3 K27M-mutant. The classification of paediatric CNS tumours is entering a new era when histopathologists must work with molecular genetic data and their molecular pathology colleagues to provide an optimal diagnostic evaluation for their patients and clinical colleagues.

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Diffuse form of dysembryoplastic neuroepithelial tumour: the histological and immunohistochemical features of a distinct entity showing transition to dysembryoplastic neuroepithelial tumour and ganglioglioma

Cited by 30 publications

References 35 publications

Dysembryoplastic Neuroepithelial Tumors

Dysembryoplastic Neuroepithelial Tumors

Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Molecular pathology of paediatric central nervous system tumours

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