Papillary glioneuronal tumors: Distinctive cytological characteristics and cyto-histologic correlation

Puzyrenko, Andrii; Cochran, Elizabeth J.; Giorgadze, Tamara; Nomani, Laila

doi:10.1016/j.anndiagpath.2021.151757

Cited by 5 publications

(7 citation statements)

References 8 publications

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“…PGNT usually presents with nonspecific clinical manifestations [ 7 ]. Headache, nausea, and vomiting caused by increased intracranial pressure are common initial symptoms, followed by seizures.…”

Section: Discussionmentioning

confidence: 99%

Imaging manifestations of papillary glioneuronal tumors

Du,

He,

et al. 2024

Neurosurg Rev

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To analyse the imaging findings of papillary glioneuronal tumors (PGNTs), in order to improve the accuracy of preoperative diagnosis of this tumor. The clinical and imaging manifestations of 36 cases of PGNT confirmed by pathology were analyzed retrospectively. A total of 17 males and 19 females, averaging 22.47 (± 11.23) years. Initial symptoms included epilepsy in ten, headache in seven, and others in 19 cases. 97.2% (35/36) of the lesions were located in the supratentorial area, and 80.5% (29/36) in the intraventricular or deep white matter adjacent to the lateral ventricles. Twenty-four of the lesions (66.7%) were mixed cystic and solid, four (11.1%) were cystic with mural nodules, four (11.1%) were cystic, and four (11.1%) were solid. Four cases of PGNT of cystic imaging showed a “T2-FLAIR mismatch” sign. 69.4% (25/36) had septations. Nine lesions (25%) were accompanied by edema, and 9 (25%) of the mixed cystic and solid lesions were accompanied by hemorrhage. Among the 18 patients who underwent computed tomography (CT) or susceptibility-weighted imaging (SWI), nine had lesions with calcification. PGNTs mostly manifest as cystic mass with mural nodules or mixed cystic and solid mass in the white matter around the supratentorial ventricle, and the cystic part of the lesion is mostly accompanied by septations. Pure cystic lesions may exhibit the sign of “T2-FLAIR mismatch”. PGNT is rarely accompanied by edema but sometimes by calcification and hemorrhage. Patients often present with seizures, headaches, and mass effect symptoms.

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Section: Discussionmentioning

confidence: 99%

Imaging manifestations of papillary glioneuronal tumors

Du,

He,

et al. 2024

Neurosurg Rev

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“…Approximately just under 140 papillary glioneuronal tumor cases were revealed by reviewing the literature until 2017, and less than 20 patients with PGNTs have been recorded from 2017 to 2021 [6][7][8][9][10][11]. It mostly affects young adults in age around 25 years old, however, it has been reported in all age groups (range 2-75 years old) [7,8,12].…”

Section: Discussionmentioning

confidence: 99%

“…This means that the macroscopic mixture of solid and cystic components, microscopically, is formed by both glial (astrocytic) and neuronal elements [13]. The glial component consists of small uniform glial cells pseudopapillae lined with hyalinized vascular cores without atypia or mitosis [11,13]. Additionally, the neuronal component is characterized by an interpapillary zone occupied by neurocytes with uniform nuclei and occasional perinuclear halos and mixed with ganglion and ganglioid cells [13,14].…”

Section: Discussionmentioning

confidence: 99%

“…Glial appearance has an immunophenotype of GFAP+, S100+ and nestin+, while neuronal components are explained by synaptophysin+, neuron-specific enolase+, and class III beta-tu-bulin+ [5]. Molecular analysis has demonstrated the association of PGNT with the presence of a fusion gene SCL44A1/PRKCA, which seems to be a common characteristic of most PGNTs with a high diagnostic value and detectable by FISH [2,11,15]. In the new WHO classification in 2021, PRKCA is regarded as a key-diagnostic gene for papillary glioneuronal tumors [1].…”

Section: Discussionmentioning

confidence: 99%

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Papillary Glioneuronal Tumor of Third Ventricle Endoscopically Treated: Case Report and Review of Literature

Solou¹,

EK²,

Ydreos³

et al. 2022

COS

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Introduction: Papillary Glioneuronal Tumor (PGNT) is a grade I tumor based on the new World Health Organization CNS tumor classification. Its special feature concerns its biphasic histologic pattern of both glial and neuronal elements. Because of the rarity of this entity, literature is mostly based on case reports. Research Question: The objective of this paper is to display our experience treating endoscopically a papillary glioneuronal tumor located into the third ventricle in an adult patient and to highlight the main points of the literature. Material & Methods: A 75-year-old patient with known Parkinson disease presented with episodes of loss of consciousness and gait disorders in our institution. Brain MRI demonstrated enlargement of ventricular system due to an obstruction caused by an intraventricular tumor in the third ventricle. Results: Patient underwent an endoscopic subtotal removal of the tumor. Postoperative brain CT scan revealed minor residual of the tumor and patient was discharged 2 days after surgery in good clinical condition. Histopathological analysis of the tumor samples matched the diagnosis of PGNT. Postoperative follow-up in 1 month demonstrated great neurological improvement. Discussion & Conclusion: Literature reports that total surgical resection is the treatment of choice in almost all cases of PGNT giving the most favorable clinical outcome. Only 4 cases of PGNT located in 3rd ventricle have been recorded and data for endoscopic management found only for one of the cases concerning a pediatric patient. Therefore, we report for first time removal of third ventricle papillary glioneuronal tumor in adult patients via the endoscopic technique.

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“…A higher diagnostic accuracy can be obtained when squash smears and frozen section preparations are applied simultaneously [7,8]. Even though clinical, pathologic, radiologic, and molecular features of LEATs have been discussed intensely [9,10], the knowledge on cytopathologic features of LEATs is based on individual case reports and limited case series [11][12][13][14].…”

Section: Introductionmentioning

confidence: 99%

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

Kurtulan¹,

Bilginer²,

Söylemezoğlu³

2022

Acta Cytologica

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Introduction: 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). Methods: 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. Results: 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. Conclusion: 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.

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Papillary glioneuronal tumors: Distinctive cytological characteristics and cyto-histologic correlation

Cited by 5 publications

References 8 publications

Imaging manifestations of papillary glioneuronal tumors

Imaging manifestations of papillary glioneuronal tumors

Papillary Glioneuronal Tumor of Third Ventricle Endoscopically Treated: Case Report and Review of Literature

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

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