Mixed glioneuronal tumor: A dysembryoplastic neuroepithelial tumor with rosette‐forming glioneuronal tumor component

2012 ◽  
Vol 33 (4) ◽  
pp. 431-435 ◽  
Author(s):  
Ji Xiong ◽  
LiLi Ding ◽  
Hong Chen ◽  
Haixia Chen ◽  
Yin Wang
Keyword(s):  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor

Rosette-forming glioneuronal tumors of the posterior fossa

2010 ◽  
Vol 5 (1) ◽  
pp. 98-103 ◽  
Author(s):  
Manish N. Shah ◽  
Jeffrey R. Leonard ◽  
Arie Perry
Keyword(s):  
Posterior Fossa ◽  
Fourth Ventricle ◽  
Complete Resection ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Tumor Type ◽  
Who Grade ◽  
Pathological Characteristics ◽  
The Mean ◽  
Glioneuronal Tumors

Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a rare, recently described WHO Grade I neoplasm. The authors report 6 examples of RGNT arising primarily from the cerebellar vermis. All the patients were female, and the mean age of presentation was 24.8 years. The original diagnoses included pilocytic astrocytoma, ependymoma, cerebellar dysembryoplastic neuroepithelial tumor (DNT), and oligodendroglioma. The cases showed classic pathological characteristics, although in 2 cases the lesions included DNT-like “floating neurons” involving Purkinje cells, a feature which has not been previously reported to the authors' knowledge. The clinical outcome was excellent with no recurrences after complete resection. These cases expand the known clinical and histological spectrum of this rare tumor type. Given the lack of fourth ventricle involvement in most of these cases, the authors suggest revising the name to RGNT of the posterior fossa.

scholarly journals A Rare Case of Dysembryoplastic Neuroepithelial Tumor

2012 ◽  
Vol 2 ◽  
pp. 60 ◽  
Author(s):  
Harsha Guduru ◽  
Jun K. Shen ◽  
Harish S. Lokannavar
Keyword(s):  
Rare Case ◽  
Intractable Epilepsy ◽  
Mass Effect ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Peritumoral Edema ◽  
Imaging Diagnosis ◽  
Common Presentation ◽  
The Central Nervous System ◽  
Children And Young Adults

We present a rare case of dysembryoplastic neuroepithelial tumor, a rare benign glioneuronal tumor of the central nervous system. It generally occurs in the supratentorial region and the temporal cerebral cortex in children and young adults. The most common presentation is epilepsy. The supratentorial tumor without any signs of mass effect or peritumoral edema is the conventionally accepted diagnostic criteria. In this case of a 19-year-old male with intractable epilepsy, atypical features such as the location of the tumor and the presence of mass effect and peritumoral edema made imaging diagnosis difficult. Diagnosis was confirmed through histopathology. Due to its recent discovery and relatively rare occurrence it is important for radiologists to recognize this disease entity.

scholarly journals Frequent FGFR1 hotspot alterations in driver-unknown low-grade glioma and mixed neuronal-glial tumors

2022 ◽  
Author(s):  
Sophie Engelhardt ◽  
Felix Behling ◽  
Rudi Beschorner ◽  
Franziska Eckert ◽  
Patricia Kohlhof ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Mapk Pathway ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Low Grade ◽  
Sequencing Analysis ◽  
Diffuse Astrocytoma ◽  
Glial Tumors ◽  
Hotspot Mutations ◽  
Fgfr1 Mutation

Abstract Purpose Low-grade gliomas (LGG) and mixed neuronal-glial tumors (MNGT) show frequent MAPK pathway alterations. Oncogenic fibroblast growth factor receptor 1 (FGFR1) tyrosinase kinase domain has been reported in brain tumors of various histologies. We sought to determine the frequency of FGFR1 hotspot mutations N546 and K656 in driver-unknown LGG/MNGT and examined FGFR1 immunohistochemistry as a potential tool to detect those alterations. Methods We analyzed 476 LGG/MNGT tumors for KIAA-1549-BRAF fusion, IDH1/2, TERT promotor, NF1, H3F3A and the remaining cases for FGFR1 mutation frequency and correlated FGFR1 immunohistochemistry in 106 cases. Results 368 of 476 LGG/MNGT tumors contained non-FGFR1 alterations. We identified 9 FGFR1 p.N546K and 4 FGFR1 p.K656E mutations among the 108 remaining driver-unknown samples. Five tumors were classified as dysembryoplastic neuroepithelial tumor (DNT), 4 as pilocytic astrocytoma (PA) and 3 as rosette-forming glioneuronal tumor (RGNT). FGFR1 mutations were associated with oligodendroglia-like cells, but not with age or tumor location. FGFR1 immunohistochemical expression was observed in 92 cases. FGFR1 immunoreactivity score was higher in PA and DNT compared to diffuse astrocytoma, but no correlation between FGFR1 mutation in tumors and FGFR1 expression level was observed. Conclusion FGFR1 hotspot mutations are the fifth most prevailing alteration in LGG/MNGT. Performing FGFR1 sequencing analysis in driver-unknown low-grade brain tumors could yield up to 12% FGFR1 N546/K656 mutant cases.

Complex Dysembryoplastic Neuroepithelial Tumor With Pilocytic Astrocytoma Component

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S44-S45
Author(s):  
Sandhyarani Dasaraju ◽  
Krutika Patel ◽  
Shalla Akbar ◽  
Osama Elkadi
Keyword(s):  
Contrast Enhancement ◽  
Pilocytic Astrocytoma ◽  
Early Recognition ◽  
Complex Form ◽  
Surgical Excision ◽  
Biological Behavior ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Who Grade ◽  
Complete Surgical Excision

Abstract Objectives Dysembryoplastic neuroepithelial tumor (DNET) is an uncommon mixed glioneuronal tumor. DNET is classified as WHO grade I neoplasm and commonly seen in the temporal lobe of children and young adults with features of pharmacoresistant seizures. The term complex DNET defines those tumors associated with additional features, including a pilocytic astrocytoma (PA) component, a finding that is rarely described in the literature. Methods Here we report a 16-year-old male who presented with seizures. On imaging, a 5.5 × 2.2 × 2.1-cm multiloculated mass was centered in the left frontoparietal gray matter with solid and cystic components and focal areas of contrast enhancement. Results Microscopic examination revealed a noninfiltrative glioneuronal neoplasm composed of a bland population of round to oval cells arranged around pools of mucinous material. Occasional large neuronal cells were noted. Focal areas composed of elongated astrocytic cells demonstrating piloid morphology were also present. No high-grade features were demonstrated. The findings are consistent with DNET, complex form in addition to a second component with piloid features resembling pilocytic astrocytoma. Conclusion The early recognition of DNET is important as it has an excellent prognosis on complete surgical excision without use of adjunct therapies. Unresected or incompletely removed DNET and secondary radiochemotherapy may give rise to recurrence and malignancy. The development of the secondary neoplasm challenges the stability in terms of biological behavior and highlights the importance of complete surgical excision and close follow-up. Our patient had neither undergone previous surgery nor received any adjuvant therapy, and hence this case is a de novo development of PA in a DNET. The association of contrast enhancement with DNET is an unusual finding in this case.

MULTIFOCAL COMPLEX GLIONEURONAL TUMOR IN AN ELDERLY MAN

Neurosurgery ◽  
2009 ◽  
Vol 64 (6) ◽  
pp. E1193-E1195 ◽  
Author(s):  
Jian-Qiang Lu ◽  
Bernd W. Scheithauer ◽  
Pranshu Sharma ◽  
James N. Scott ◽  
Ian F. Parney ◽  
...  
Keyword(s):  
Obstructive Hydrocephalus ◽  
External Ventricular Drain ◽  
Third Ventricle ◽  
Glioneuronal Tumor ◽  
Third Ventriculostomy ◽  
Magnetic Resonance Imaging Scan ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Histological Features ◽  
The Third ◽  
Glioneuronal Tumors

Abstract OBJECTIVE The clinicopathological spectra of a dysembryoplastic neuroepithelial tumor (DNT) and a rosette-forming glioneuronal tumor (RGNT) are expanding. We report here the autopsy findings of a case of complex glioneuronal tumor with combined histological features of both a DNT and an RGNT. CLINICAL PRESENTATION A 79-year-old man presented with a 1-month history of confusion and gait difficulties. A magnetic resonance imaging scan revealed obstructive hydrocephalus attributed to a mass in the posterior third ventricle. INTERVENTION A third ventriculostomy was performed. Postoperatively, the mass remained unchanged in size for more than 14 months. Thirty-eight months after his initial manifestations, he experienced minor head trauma and was then hospitalized. Despite placement of an external ventricular drain and other supportive treatment, he deteriorated and died. A full autopsy was performed, with emphasis on the brain. The mass lesion and a few independent microfoci situated primarily around the third ventricle showed histological features of pilocytic astrocytoma with recurrent hemorrhage. Far more numerous were microfoci with histological features of a DNT, including floating neurons, as well as typical RGNT-associated, synaptophysin-positive rosettes and perivascular pseudorosettes. CONCLUSION The advanced age of the patient, the coexisting histological features of the DNT and RGNT, and the distinctive anatomic distribution of the lesions, being centered on the third ventricle, may lend insight into the histogenetic relationship of a DNT, an RGNT, and mixed glioneuronal tumors.

Challenges in the Intraoperative Consultation of Low-Grade Epilepsy-Associated Neuroepithelial Tumors by Cytomorphology in Squash Preparations

2022 ◽  
pp. 1-7
Author(s):  
Olcay Kurtulan ◽  
Burçak Bilginer ◽  
Figen Soylemezoglu
Keyword(s):  
Ganglion Cells ◽  
Case Reports ◽  
Daily Practice ◽  
Individual Case ◽  
Glioneuronal Tumor ◽  
Dysembryoplastic Neuroepithelial Tumor ◽  
Low Grade ◽  
Diagnostic Challenge ◽  
Neuroepithelial Tumors ◽  
Tumor Types

<b><i>Introduction:</i></b> Low-grade epilepsy-associated neuroepithelial tumors (LEATs) create a diagnostic challenge in daily practice and intraoperative pathological consultation (IC) in particular. Squash smears are extremely useful in IC for accurate diagnosis; however, the knowledge on cytopathologic features of LEATs is based on individual case reports. Here, we discuss the 3 most common and well-established entities of LEATs: ganglioglioma (GG), dysembryoplastic neuroepithelial tumor (DNT), and papillary glioneuronal tumor (PGNT). <b><i>Methods:</i></b> Thirty patients who underwent surgery for GG, DNT, and PGNT between 2001 and 2021 were collected. Squash smears prepared during intraoperative consultation were reviewed by 1 cytopathologist and an experienced neuropathologist. <b><i>Results:</i></b> Among the 30 tumors, 16 (53.3%) were GG, 11 (36.6%) DNT, and 3 (10%) PGNT. Cytomorphologically, all of the 3 tumor types share 2 common features such as dual cell population and vasculocentric pattern. GG smears were characteristically composed of dysplastic ganglion cells and piloid-like astrocytes on a complex architectural background of thin- to thick-walled vessels. DNT, on the other hand, showed oligodendroglial-like cells in a myxoid thin fibrillary background associated with a delicate capillary network. Common cytological features of PGNT were hyperchromatic cells with narrow cytoplasm surrounding hyalinized vessels forming a pseudopapillary pattern and bland cells with neuroendocrine nuclei dispersed in a neuropil background. <b><i>Conclusion:</i></b> A higher diagnostic accuracy can be obtained when squash smears are applied with frozen sections. However, it is important to integrate clinical and radiologic features of the patient as well as to know the cytopathologic features of the LEAT spectrum in the context of differential diagnosis to prevent misinterpretation in the IC.

Glioneuronal tumor with neuropil-like islands

2000 ◽  
Vol 31 (11) ◽  
pp. 1435-1438
Author(s):  
Richard A. Prayson ◽  
Caroline M. Abramovich
Keyword(s):  
Glioneuronal Tumor
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