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Dysembryoplastic Neuroepithelial Tumors.

Suh YL - J Pathol Transl Med (2015)

Bottom Line: 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.The histological and cytological differential diagnoses for this lesion, especially the nonspecific variant, will be discussed.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

Ultrastructural findings of dysembryoplastic neuroepithelial tumors. (A) Oligodendroglioma-like cells (OLCs) show oval nuclei with small indentation, marginal aggregates of heterochromatin, and their cytoplasm with scanty organelles (×7,000). (B, C) Neuropil-like network of cellular process with a synaptic contact (arrow) (B, ×17,000) and scant dense core granules (arrow) (C, ×30,000) indicates neuronal differentiation of OLCs. (D) A few OLCs contain ribosome-lamellae complex inclusions (arrow) (×7,000).
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f6-jptm-2015-10-05: Ultrastructural findings of dysembryoplastic neuroepithelial tumors. (A) Oligodendroglioma-like cells (OLCs) show oval nuclei with small indentation, marginal aggregates of heterochromatin, and their cytoplasm with scanty organelles (×7,000). (B, C) Neuropil-like network of cellular process with a synaptic contact (arrow) (B, ×17,000) and scant dense core granules (arrow) (C, ×30,000) indicates neuronal differentiation of OLCs. (D) A few OLCs contain ribosome-lamellae complex inclusions (arrow) (×7,000).

Mentions: Ultrastucturally, OLCs exhibit round to oval invaginated nuclei with evenly dispersed chromatin, marginally condensed heterochromatin, and frequently a single small nucleolus (Fig. 6A). The cytoplasm of OLCs has scanty numbers of organelles, such as rough endoplasmic reticulum, mitochondria, microtubules, and clear vesicles. Unlike oligodendroglial cells, OLCs do not show abundant microtubules, although oligodendroglial differentiation such as pericellular lamination of cell processes has been demonstrated in one case [32]. OLCs may show features of neuronal differentiation [33]. The neuronal features include scant dense core granules (45–60 µm), clear vesicles, synaptic junctions, or neuropil-like cellular processes (Fig. 6B, C). OLCs may show astrocytic features with small numbers of intermediate filaments. Ganglion cells are also observed but never exhibit the dysplastic features or abundant dense core granules of gangliogliomas. Ribosome-lamellae complexes can be found in a few OLCs (Fig. 6D). They are cylindrical and resemble “laboratory tubes with cone-like endings [33].” The presence of these inclusions in OLCs suggests an astrocytic differentiation, because they have been demonstrated in tumors of astrocytic lineage, including glioblastomas.


Dysembryoplastic Neuroepithelial Tumors.

Suh YL - J Pathol Transl Med (2015)

Ultrastructural findings of dysembryoplastic neuroepithelial tumors. (A) Oligodendroglioma-like cells (OLCs) show oval nuclei with small indentation, marginal aggregates of heterochromatin, and their cytoplasm with scanty organelles (×7,000). (B, C) Neuropil-like network of cellular process with a synaptic contact (arrow) (B, ×17,000) and scant dense core granules (arrow) (C, ×30,000) indicates neuronal differentiation of OLCs. (D) A few OLCs contain ribosome-lamellae complex inclusions (arrow) (×7,000).
© Copyright Policy
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4696533&req=5

f6-jptm-2015-10-05: Ultrastructural findings of dysembryoplastic neuroepithelial tumors. (A) Oligodendroglioma-like cells (OLCs) show oval nuclei with small indentation, marginal aggregates of heterochromatin, and their cytoplasm with scanty organelles (×7,000). (B, C) Neuropil-like network of cellular process with a synaptic contact (arrow) (B, ×17,000) and scant dense core granules (arrow) (C, ×30,000) indicates neuronal differentiation of OLCs. (D) A few OLCs contain ribosome-lamellae complex inclusions (arrow) (×7,000).
Mentions: Ultrastucturally, OLCs exhibit round to oval invaginated nuclei with evenly dispersed chromatin, marginally condensed heterochromatin, and frequently a single small nucleolus (Fig. 6A). The cytoplasm of OLCs has scanty numbers of organelles, such as rough endoplasmic reticulum, mitochondria, microtubules, and clear vesicles. Unlike oligodendroglial cells, OLCs do not show abundant microtubules, although oligodendroglial differentiation such as pericellular lamination of cell processes has been demonstrated in one case [32]. OLCs may show features of neuronal differentiation [33]. The neuronal features include scant dense core granules (45–60 µm), clear vesicles, synaptic junctions, or neuropil-like cellular processes (Fig. 6B, C). OLCs may show astrocytic features with small numbers of intermediate filaments. Ganglion cells are also observed but never exhibit the dysplastic features or abundant dense core granules of gangliogliomas. Ribosome-lamellae complexes can be found in a few OLCs (Fig. 6D). They are cylindrical and resemble “laboratory tubes with cone-like endings [33].” The presence of these inclusions in OLCs suggests an astrocytic differentiation, because they have been demonstrated in tumors of astrocytic lineage, including glioblastomas.

Bottom Line: 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.The histological and cytological differential diagnoses for this lesion, especially the nonspecific variant, will be discussed.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus