Cases Of Papillary Glioneuronal Tumor Research Articles

Papillary glioneuronal tumor: A rare case with recent updates as per 2021 CNS WHO 5th classification.

The papillary glioneuronal tumor is a WHO grade 1, rare neuronal-glial tumor and comprises 0.02% of all CNS tumors. Histologically, it is a mixture of glial and neuronal components showing a pseudopapillary pattern with hyalinized vessels. PGNT is considered a low-grade neoplasm, and surgical excision has been curative in most cases. In this paper, we report a new case of papillary glioneuronal tumor in a 44-year-old male having a divergent presentation, to analyze it due to the rarity of its occurrence as per the latest classification.

被误诊的乳头状胶质神经元肿瘤一例

被误诊的乳头状胶质神经元肿瘤一例

Clinicopathological study of recently added glioneuronal tumors

Background: Glioneuronal tumors are pathologically heterogeneous group of tumors containing both glial and neural components or glial tumors with neural differentiation. In the year 2007, three new entities have been added to the repertoire of glioneuronal tumors by the World Health Organization (WHO) which include papillary glioneuronal tumor (PGNT) (WHO Grade I), rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) (WHO Grade I), and glioneuronal tumor with neuropil-like islands (GTNIs) (WHO grade II/III). The present study summarizes the clinical and neuropathological features of these three glioneuronal neoplasms. Materials and Methods: This study included seven cases of newer glioneuronal tumors (four cases of PGNT, two cases of RGNT, and one case of GTNI) which were reviewed. Results: The clinical presentations (patient characters), radiology, squash preparations, histology, and immunohistochemical findings of these cases are discussed including some of the morphological variations and follow-up. Conclusion: Glioneuronal tumors show considerable morphological and clinical diversity with some unique features. As these neoplasms are low grade and well manageable, the knowledge of their clinical presentation and histological diagnosis is essential for treatment. The present study is an attempt toward this.

SLC44A1–PRKCA fusion in papillary and rosette-forming glioneuronal tumors

We investigated the fused protein of solute carrier family 44 choline transporter member 1 (SLC44A1) and protein kinase C alpha (PRKCA) in three patients with papillary glioneuronal tumors (PGNT). PGNT and rosette-forming glioneuronal tumors (RGNT) are recently identified, unusual glioneuronal tumor variants which were categorized as novel tumor entities in the 2007 World Health Organization classification system. The molecular background of these tumors remains poorly understood due to the paucity of studies. The SLC44A1–PRKCA fusion was recently detected in three cases of PGNT. We invesitgated for the SLC44A1–PRKCA fusion protein in the three PGNT patients and a further two with RGNT using fluorescence in situ hybridization. Two out of the three PGNT patients had a fused signal (paired red–green signal) representing a rearrangement on chromosomes 9 and 17. A normal signal pattern was observed in the third PGNT patient. Neither of the two RGNT patients demonstrated a fused signal. This suggests that the SLC44A1–PRKCA fusion is a characteristic alteration in PGNT but not RGNT. Therefore, it is a potential biomarker of PGNT. The paired red–green signal that was observed in the PGNT patients implies the presence of a different breakpoint than that previously reported in the 9q31 and 17q24 genes.

Analysis of a Novel Translocation, T(9;17)(Q31;Q24), in Rosette-Forming Glioneuronal Tumors

ABSTRACT Aim: Papillary glioneuronal tumor (PGNT) and rosette-forming glioneuronal tumor (RGNT) were recently established unusual glioneuronal phenotypes and were categorized as a novel tumor entity in the 2007 World Health Organization classification. The molecular background of these glioneuronal tumors remains poorly understood due to a paucity of reports. Recently, the SLC44A1-PRKCA fusion has been identified in 3 cases of PGNT. Methods: The aim of this study was to confirm it in our 3 cases of PGNT and analyze the presence of specific fusion in 2 cases of RGNT, using fluorescence in situ hybridization. Results: Two out of three PGNT cases showed one adjacent red-green signal pair representing the rearrangement on chromosome 9 and 17, but not a complete fused yellow signal. Normal signal pattern was observed in the other PGNT case. Neither of two RGNT cases revealed adjacent red-green signal pair or yellow signal. Conclusions: The SLC44A1-PRKCA fusion is a characteristic alteration in PGNT but not in RGNT, and is suggested as a potential biomarker of PGNT. One adjacent red-green signal pair observed in the PGNTs may imply the presence of different breakpoints from those previously reported in the 9q31 and 17q24 genes. Disclosure: All authors have declared no conflicts of interest.

Papillary Glioneuronal Tumor: Unexplored Entity

Papillary glioneuronal tumors represent a new and rare entity of an uncommon morphologic subtype of low-grade mixed neuronal-glial neoplasms with an unclear etiology. They are described as benign lesions with extraventricular localization. We report the second case of papillary glioneuronal tumor with recurrent nature after gross-total resection, and the third case of this tumor with intraventricular localization. While conventional magnetic resonance imaging of papillary glioneuronal tumors is well described in literature, there are no data based on advanced magnetic resonance techniques. The present article represents a review of clinicopathological and both conventional and advanced magnetic resonance imaging characteristics of papillary glioneuronal tumors, with focus on 2 cases with atypical course and localization.

Papillary Glioneuronal Tumors

This study was designed to evaluate 4 new cases of papillary glioneuronal tumors (PGNTs), 2 of which had atypical histologic features, provides extensive IHC characterization, performed comparative genomic hybridization in 2 of our cases, and reviews the recent literature. The study group comprised 3 women and 1 man, with ages ranging from 12 to 75 years. Patients presented with seizures (n = 3) or muscle spasm (n = 1), and the tumors were located in the supratentorial region of the brain (3 in the frontal and 1 in the parietotemporal lobe). The 2 atypical tumors showed a moderately high mitotic rate (4 and 7/10 HPF, each), vascular endothelial hyperplasia, and necrosis. Tumor cells expressed both neuronal and glial markers, but the degree of neuronal versus glial expression was varied. None of the tumors expressed p53, EGFR wild type/vIII, IDH1, or CD34; however, nestin, galectin-3, and S100 were positive in the tumor cells. No EGFR gene amplification or 1p/19q deletion was found by fluorescence in situ hybridization. Half of the cases revealed PTEN loss by immunohistochemistry, and MGMT methylation was positive in 3 cases by MGMT methylation-specific polymerase chain reaction. Ultrastructurally, either astrocytic or neuronal differentiation was observed, but we could not identify any hybrid cells. An array-based comparative genomic hybridization study revealed loss of 1q, 6p, 8p, 9p, 9q, and 16q and gain of 2q, 3p, 5q, 6p, 7q, 10q, 16q, 19p, and 22q in 2 cases simultaneously. The first patient, who underwent subtotal resection, died because of progression of the tumor within 9 months after surgery; however, 2 patients were symptom free and progression free at 34 and 48 months after gross total resection (the patient 2: plus radiotherapy, the patient 3: no adjuvant chemo- or radiotherapy). The last patient developed seizures after a long symptom-free period (40 mo) with no evidence of tumor recurrence. Our 4 new cases, in conjunction with the literature review, reinforce that PGNTs are tumors that usually occur in young adults (mean age, 24 y); they are most often cystic with a mural nodule or are cystic/solid, supratentorial, closely located with the ventricle, molecularly genetically different from astrocytic or oligodendroglial tumors, and indolent in behavior. Cases (2 of 4 in our study) with atypical histologic features or that occur in advanced age (75 y), and sporadic reports of histologically or biologically aggressive PGNTs, serve to remind pathologists that the full spectrum of PGNTs is as yet unknown.

Papillary Glioneuronal Tumor: Radiological Evidence of a Newly Established Tumor Entity

Papillary glioneuronal tumor (PGNT) was newly classified as a Grade I neuronal-glial tumor by 2007 revision of the World Health Organization (WHO) classification of tumor of central nervous system (CNS) because of its characteristic pseudopapillae and diphase differentiation features. Previous literature has laid particular emphasis on pathology manifestations, and radiological features were only briefly mentioned. The purpose of this study was to describe magnetic resonance imaging (MRI) features through reporting 2 cases of PGNT and literature review. MRI findings and pathology features in 2 cases of PGNT were reported and the literature was reviewed. Both patients were confirmed as PGNT by surgery and pathology. Seizure was the main clinical manifestation. Histopathological examination revealed characteristic pseudopapillary structure with astrocytes and neurons. Both lesions were located in the temporal lobe, and one case was closely related with the lateral ventricle. MRI showed cystic-solid mass or cystic lesion with mural nodule. The solid component enhanced strikingly after contrast agent administration. MRI findings of PGNT present certain characteristics. Most cases showed cystic mass with mural nodule and periventricular location was common. Although radiological findings can provide some evidence for this newly established tumor entity, differential diagnosis is still needed.

Papillary glioneuronal tumor – contribution to a new tumor entity and literature review

Papillary glioneuronal tumor (PGNT) is a recently identified low-grade mixed glial-neuronal neoplasm of juvenile and young adult patients. The WHO classification does not categorize this tumor as a separate entity, but rather considers it as a variant of ganglioglioma. We present a new case of this rare entity, representing the 3rd case of this lesion in Chinese patients and review the findings in 34 patients from different case reports found in the international literature. This report describes a histologically similar-appearing lesion arising in the left frontoparietal lobe of a 23-year-old man. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells which eventually admixed with ganglioid and ganglion cells. Sporadic Rosenthal fibers, foci of calcification, areas of hemosiderin deposition were identified. The mean Ki67 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation or necrosis were consistently lacking. It is important that every new case of PGNT is reported to allow its recognition and classification. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular low malignant potential neoplasm, whose presentation may allow for consideration as an entity.

Papillary glioneuronal tumor

Papillary glioneuronal tumor (PGNT) is a rare, recently recognized tumor type histologically characterized by pseudopapillary architecture associated with compact areas composed of neuronal elements in different maturation states. The histogenesis of this tumor type is still unclear. The immunophenotype of PGNT comprises expression of several glial and neuronal proteins. Recently, immunohistochemical expression of Olig2 in a fraction of tumor cells has been reported, suggesting an additional oligodendroglial or at least oligodendroglia-like tumor cell component. We report a further case of papillary glioneuronal tumor in a 12-year-old boy with immunohistochemical expression of PDGFRalpha, Olig2 and Nestin in support of a postulated origin of this tumor type from common progenitor cells in the subependymal plate. Nevertheless, further studies are needed to clarify the histogenesis of PGNT.

Papillary glioneuronal tumor radiologically mimicking a cavernous hemangioma with hemorrhagic onset

Papillary glioneuronal tumor is a recently identified low-grade brain neoplasm classified as variant of ganglioglioma. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells, eventually admixed with ganglioid and ganglion cells. We present a case of papillary glioneuronal tumor occurring in a 15-year-old female with an unusual hemorrhagic onset. The clinical, morphological and immunohistochemical features are discussed and the published literature is reviewed. This article proposes that papillary glioneuronal tumor should be included in the differential diagnosis of patients with tumoral related brain hemorrhage.

Papillary Glioneuronal Tumor

Papillary glioneuronal tumor is a recently described neoplasm composed of gliovascular pseudopapillae associated with intervening neuronal cells ranging from neurocytes to ganglion cells. This tumor is not currently included in the WHO classification of tumors of the central nervous system. We describe a new case of papillary glioneuronal tumor and analyze the data for a series of further 15 patients from international literature. A 27-year-old man presented to us for generalized seizure. CT and MRI showed a cystic tumor with mural nodule in the left frontal lobe. Frontal craniotomy with gross total removal of the tumor was performed. Histopathological examination was positive for papillary glioneuronal tumor. The clinical, radiologic, and pathological features of our case are strikingly similar to those of the previous reported cases. A review of the literature disclosed only 15 other cases of these tumors. It is important that every new case of PGNT is reported to allow its recognition and classification.

Papillary Glioneuronal Tumor

Papillary Glioneuronal TumorT. Stosic-Opincal1, V. Peric1, S. Gavrilovic1, M. Gavrilov1, Z. Markovic1 and R. N. Sener2Audio Available | Share

A distinct pattern of Olig2-positive cellular distribution in papillary glioneuronal tumors: a manifestation of the oligodendroglial phenotype?

Mixed neuronal-glial tumors of the central nervous system display a wide spectrum of differentiation. Among them, the papillary glioneuronal tumor (PGNT) is characterized by pseudopapillary structures composed of astroglial cells covering hyalinized vessels, and by neurocytic, ganglioid and ganglion cells. In addition, a "nonspecific" cell type, not similar to either astrocytes or neurocytes, has been recognized since the initial reports. Recently, minigemistocytic cells and a population immunostained by anti-Olig2 antibody have also been recognized in PGNT. Olig2 is a transcription factor that is specific for the cellular phenotype of oligodendrocytes. The aim of this study was to further investigate the histological diversity of PGNT. We examined six cases of PGNT, each of which showed Olig2 immunopositivity. Minigemistocytes were encountered in three cases at close proximity to the Olig2-positive area. Olig2-positive cells were negative for glial fibrillary acidic protein (GFAP) and neuronal nuclear antigen by double immunostaining, and mainly occupied the interpapillary area laterally adjacent to the GFAP-positive cells. They had relatively small, round and vesicular nuclei, and were formerly regarded as neurocytic cells or nonspecific cellular elements. Fluorescence in situ hybridization targeting chromosome 1p failed to demonstrate any deletion. This study disclosed an additional cellular component of PGNT that is characterized by Olig2 positivity, suggestive of oligodendroglial phenotype, and the results also encourage us to investigate oligodendroglial participation in various glioneuronal tumors.