Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Iwona Sontowska; Ewa Matyja; Jacek Malejczyk; Wieslawa Grajkowska

doi:10.5114/fn.2017.66708

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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vol. 55

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abstract:

Review paper

Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Iwona Sontowska

,

Ewa Matyja

,

Jacek Malejczyk

,

Wieslawa Grajkowska

Folia Neuropathol 2017; 55 (1): 1-13

DOI: https://doi.org/10.5114/fn.2017.66708

Online publish date: 2017/03/27

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Dysembryoplastic neuroepithelial tumour (DNT) is categorized as a benign glioneuronal neoplasm affecting children and young adults with chronic epileptic seizures. It is characterized by predominant intracortical localization and nodular architecture. Dysembryoplastic neuroepithelial tumour usually demonstrates a distinctive morphological pattern with a specific glioneuronal element but occasionally, its morphological picture is heterogeneous and unspecific. Thus, considering the morphology of DNT, three different histopathological subtypes are distinguished: simple, complex, and non-specific and diffuse. The DNT lesions are often related with focal cortical dysplasia (FCD) type IIIb, which is postulated to play a role in epileptogenicity. Moreover, the accompanying inflammation process might be implicated in DNT-related epileptogenesis.

Dysembryoplastic neuroepithelial tumour is generally characterized by favourable prognosis and good results of surgical treatment. The pathogenesis and molecular mechanisms involved in DNT development remain uncertain. The main molecular findings are connected with BRAF alterations and activation of RAS/ERK, PI3K/AKT and mTOR signalling pathways.

The present review summarizes the clinical, histopathological and molecular findings of DNT. The classification controversy, morphological heterogeneity and diagnostic problems are also discussed.

keywords:

Dysembryoplastic neuroepithelial tumour: insight into the pathology and pathogenesis

Iwona Sontowska , Ewa Matyja , Jacek Malejczyk , Wieslawa Grajkowska

DNT, dysembryoplastic neuroepithelial tumour, histopathological variants, BRAF, mTOR, DNT – pathology and pathogenesis

Iwona Sontowska

,

Ewa Matyja

,

Jacek Malejczyk

,

Wieslawa Grajkowska