Misdiagnosis of the Polymorphous Low-Grade Neuroepithelial Tumor of the Young as Ganglioglioma，A Case Report

Misdiagnosis of the Polymorphous Low-Grade Neuroepithelial Tumor of the Young as Ganglioglioma，A Case Report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Misdiagnosis of the Polymorphous Low-Grade Neuroepithelial Tumor of the Young as Ganglioglioma，A Case Report Lijuan Lai, wei Li, Guojun Song, Xuefeng Yang, Shizhao Ou, Chao Chen, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5447824/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background PLNTY traditionally manifests as an epileptogenic subtype, posing challenges in differential diagnosis. The low incidence of PLNTY and the similarity of its clinical presentation and imaging features with other types of neuroepithelial tumors have made it important to diagnose PLNTY accurately. Case presentation We report here an unusual case of a PLNTY in a 14-year-old girl who was admitted to the hospital with acute headache, nausea, and vomiting, initially misdiagnosed as ganglioglioma on imaging, and then diagnosed as pleomorphic low-grade neuroepithelial tumor (PLNTY) on surgical and pathological analyses. The patient did not present with typical epileptic symptoms, PLNTY is a rare adolescent low-grade brain tumor with imaging manifestations similar to those of other neuroepithelial tumors, which is easy to lead to misdiagnosis. Conclusion PLNTY is a class of adolescent low-grade neuroepithelial tumors with good prognosis. Definitive diagnosis of PLNTY is of great significance to avoid over-treatment and improve the quality of survival in pediatric and adolescent patients. PLNTY molecular features imaging characteristics differential diagnosis brain tumor Figures Figure 1 Figure 2 Figure 3 Background Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a rare low-grade brain tumor that is classified as a childhood-type diffuse low-grade glioma by the 2021 WHO Classification of Tumors of the Central Nervous System [ 1 ]. The analysis and application of imaging features play a crucial role in the diagnosis and treatment of neurological tumors. Polymorphic low-grade neuroepithelial tumor of the young (PLNTY) represents a set of solid tumors that predominantly affect adolescents. It was first described by Huse et al. in 2017[ 2 ]. The low incidence of PLNTY and the similarity of its clinical presentation and imaging features with other types of neuroepithelial tumors have made it important to diagnose PLNTY accurately. Despite its morphological variability, PLNTY exhibits unifying hallmarks such as oligodendroglioma-like cellular components, infiltrative growth patterns, and intense CD34 immunopositivity. These unique clinicopathologic features and molecular genetic alterations provide an important basis for the diagnosis of PLNTY [ 1 – 3 ]. Case presentation A 14-year-old girl presented with acute headache, nausea, and vomiting, but lacked classic epileptic signs. The patient was admitted to a local hospital for a complete cranial computed tomography (CT), which suggested space-occupying lesions in the right temporal lobe. Additionally, the patient reported having poor mental health, appetite, and sleep since the onset of the disease. She was also lethargic and poorly refractive, with a Glasgow Coma Scale (GCS) score of E3V5M6 = 14.The results of laboratory examination are presented below(Table 1): routine blood test showed that the white blood cell count was 15.32×10 9 /L; neutrophil ratio was 92.3%; neutrophil count was 14.15×10 9 /L; hemoglobin level was 106 g/l; FDP was 17.21 ug/mL; and the plasma D-dimer value was 6.55 mg/L. The nucleic acid test for pneumococcal pneumonia was negative. Table.1. Abnormal Laboratory Findings Patient’s results Normal range white blood cell(×10 9 /L) 15.32 4.0–10.0 neutrophil ratio(%) 92.3 50–70 neutrophil(×10 9 /L) 14.15 2.0–7.0 hemoglobin (g/l) 106 110–165 FDP(ug/mL) 17.21 0–5.0 D-dimer(mg/L) 6.55 0-0.55 The patient was admitted to the hospital and underwent a CT examination, which revealed a cystic occupation in right temporo-parietal lobe, with a visible wall nodule measuring 6.3 cm × 4.7 cm, and foci of calcification were visible at the edges of the lesion(Figure 1 A); in order to clarify the nature of the lesion, the patient underwent a cranial MRI and enhancement scanning examination(Fig. 1 B-H༉. then the patient underwent surgical intervention on the second day. during the surgery, the brain tissue was slightly swollen, and the cortex appeared grayish. The head of the tumor was exposed, so a cerebral puncture needle was inserted into the cystic cavity at the head of the tumor, and about 15 mL of cystic fluid was aspirated. The brain tissue collapsed, and the cortical fistula was taken to further resect the tumor. Postoperative immunohistochemistry revealed an intracranial space as a low-grade glioma. Immunohistochemical expression showed CD34 (diffuse + in some areas), glial fibrillary acidic protein (GFAP) (+), P53 (partially+, wild-type), S-100 (+), actin (-), Ki-67 (about 1%+), neuron-specific enolase (NSE) (partially+), Syn (+), IDH-1 (-), and epithelial membrane antigen (EMA) (±). Combined with the hematoxylin-eosin (HE) staining pattern, the diagnosis was suggestive of PLNTY (WHO grade 1) (Fig. 2 ). Postoperatively, the patient reviewed the cranial CT and MRI, and the mass was reduced in size compared with the previous one, and the size of the mass was 4.5cm×2.6cm×4.3cm in the postoperative review, the original wall nodule was not shown, and a small amount of new epidural hematoma was added on the right parietal side, and soft tissue edema of the scalp was found on the right temporal parietal side((Fig. 3).After treatment, the patient's postoperative headache, dizziness, nausea, and vomiting symptoms were relieved. Up until now, the girl has been followed up for 20 months. She recovered well postoperatively and was seizure-free. Discussion 3.1 Clinical characteristics and prognosis of PLNTY The strong epileptogenic propensity of PLNTY can cause patients to experience early-onset epilepsy or refractory epilepsy [ 4 , 5 ], Nevertheless, the presence of PLNTYs may also be accompanied by headaches, as with oligodendrogliomas [ 6 ]. Our patient’s clinical presentation differs from previous literature reports in that this patient was admitted with a sudden onset of dizziness and headache with nausea and vomiting and did not present with typical seizure symptoms. PLNTY is more likely to develop in female patients, which is a neuroepithelial tumor that mostly appears in children and adolescents [ 7 ]. Ganglioglioma is low incidence and is most common in children and young adults. Ganglioglioma occur predominantly in the temporal lobe. On imaging, GGs can be divided into cystic tumors (more than a 90% cystic component), cystic solid tumors (cystic component 10–90%), and solid tumors (cystic component less than 10%), of which cystic tumors account for about 44–80%[ 8 ]. This reported case is a 14-year-old adolescent and the lesion occurred in the temporal lobe with a predominantly cystic component, which led us to start the diagnostic consideration of a ganglioglioma. PLNTY is typically considered a benign tumor in most studies [ 2 , 9 – 12 ]. In a survey conducted by Huse et al., seven out of eight patients who underwent gross total resection of PLNTY achieved disease-free status over a mean follow-up period of 50.6 months. Interestingly, only one patient exhibited potential tumor recurrence, evident from the imaging results, 36 months after surgery [ 2 ]. This result highlights the importance of surgical resection in the management of PLNTY, especially in achieving disease-free status. However, PLNTYs located in surgically unresectable areas pose a particular challenge. Here, targeted molecular therapy targeting the MAPK pathway emerged as a promising treatment option. Specifically, BRAF/MEK inhibitors have the potential to exert both anti-epileptic and anti-tumor effects in such cases [ 13 ]. This strategy addresses the seizures that often accompany PLNTYs and aims to halt or reverse the malignant transformation of these tumors. In conclusion, while PLNTYs are typically benign, the risk of malignant transformation cannot be overlooked. The discovery of specific molecular markers such as the BRAF V600E mutation, provides valuable insights into the biology of these tumors and potential therapeutic strategies. With further research, targeted molecular therapy may become a vital tool in the armamentarium against PLNTYs, especially in those located in challenging-to-resect areas. 3.2 Molecular and histological features of PLNTY The 2021 WHO classification of CNS tumors has introduced new categories based on molecular characteristics [ 14 ]. Within the WHO CNS5 classification, diffuse gliomas are grouped into adult and pediatric types [ 15 ]. The pediatric type has similar histological morphology and a significantly different prognosis [ 1 ]. The WHO CNS5 categorizes pDLGG into four specific tumor types: I) diffuse astrocytoma with MYB- or MYBL1 alterations; II) angiocentric glioma (AG); III) PLNTY; and IV) diffuse low-grade glioma with MAPK pathway alterations [ 16 ]. This classification system aims to streamline the diagnosis and treatment of these distinct tumor types. PLNTY exhibits several distinct characteristics such as the presence of oligodendroglial-like cells in the temporal lobes and cortical/subcortical regions, dense calcification, infiltrative growth patterns, and diffuse expression of CD34 [ 17 , 18 ]. It is also common to see strong positivity for GFAP and OLIG2, while PLNTY generally remains negative for IDH1, R132H, EMA, NeuN, and other neuroendocrine markers. The MIB-1 proliferation index remains low, further supporting its benign nature. PLNTY, has been found to play a role in oncogenesis through the MAPK and PI3K/mTOR signaling pathways given the FGFR2-CTNNA3 fusion, as reported by Surrey et al. [ 9 ]. PLNTY is histologically characterized by calcification and oligodendrocyte-like features, infiltrative growth pattern, and intense CD34 immunopositivity. With regard to molecular genetic alterations, most PLNTY cases are caused by a mutation in the BRAF V600E gene, which accounts for approximately 60% of all mutations. For the diagnosis of PLNTY, these unique clinicopathologic characteristics and molecular genetic alterations are important. Younger patients are more likely to develop non-BRAF V600E tumors [ 19 ]. PLNTY shows low rates of mitosis, necrosis, and microvascular proliferation, distinguishing it from similar tumors such as pleomorphic xanthoastrocytoma (PXA), Ganglioglioma, pilocytic astrocytoma, and dysembryoplastic neuroepithelial tumor (DNET). Unlike other brain tumors, PLNTY lacks specific characteristic structures such as neurocytic, eosinophilic granular bodies, myxoid microcysts, and dysmorphic neuronal/ganglion cell forms [ 4 ]. Instead, PLNTY commonly exhibits alterations in BRAF or FGFR2/FGFR3, resulting in a low Ki-67 labeling index, consistent with its classification as a low-grade tumor[ 20 ]. 3.3 Imaging characteristics and differential diagnosis of PLNTY PLNTY tends to predominantly appear in the cerebral hemispheres, with the temporal lobe being the most common location followed by the occipital, frontal, and parietal lobes. These tumors usually have clearly defined boundaries and are most often seen in the right temporal lobe, with only a small percentage found in the ventricles [ 10 , 16 ]. A distinctive feature of PLNTY is the presence of a delineated mass in the temporal lobe that shows central calcifications, peripheral cysts, and a mix of signals on imaging tests. There are specific neuroimaging patterns that strongly suggest the presence of PLNTY [ 6 ], including a poorly defined cortical lesion with a “salt and pepper” appearance on T2-weighted imaging and calcification on CT scans [ 21 , 22 ]. Although coarse calcifications are less common in children, they can develop over time. Additionally, the trans-mantle-like sign and adjacent cortical dysplasia are key characteristics of these tumors [ 23 ]. It is crucial to distinguish PLNTY from other common brain tumors found in the temporal lobe such as ganglioglioma, oligodendroglioma, and DNET. By identifying the distinct features of PLNTY on imaging tests, healthcare providers can make a more accurate diagnosis and determine the best course of treatment for patients with these types of tumors. PLNTY showed characteristic calcification with subcortical localization and frequent temporal lobe localization [ 24 ]. The most important imaging feature in our patient was a large capsule containing small nodules and visible calcifications in the temporoparietal lobe. This specific feature known as cysts with a mural nodules tumor is commonly seen in various intracranial neoplastic lesions such as ganglioglioma, pilocytic astrocytoma, hemangioblastoma, and pleomorphic xanthoastrocytoma [ 25 ]. Considering the patient's age, tumor location, and imaging findings, our initial MRI report was suggestive of ganglioglioma. However, upon further examination, the final pathologic diagnosis revealed it to be PLNTY. Therefore, PLNTY needs to be mainly distinguished from ganglioglioma, oligodendroglioma, pilocytic astrocytoma, DNET, and pleomorphic xanthoastrocytoma (PXA). Ganglioglioma: Ganglioglioma is a unique type of brain tumor composed of glial cells and differentiated neurons. Although rare, it accounts for a small percentage of all primary brain tumors, with the majority affecting individuals under 30 years old [ 26 ]. Around 50% gangliogliomas show a cyst with a mural nodule on radiological examination. Other presentation comprises a solid tumor with low-to-intermediate T1-weighted (T1W) and high T2W signal or masses with predominant T1 hyperintensity [ 27 ]. Oligodendroglioma: These are most commonly found in the frontal lobe and are the third-most frequent type of glial neoplasm. They occur more often in male patients [ 28 ]. There is a high incidence of calcification in oligodendrogliomas that is visible on non-enhanced CT in up to 90% cases [ 29 ]. Factors such as tumor grade, size, MGMT methylation levels, extent of resection, midline crossing rates, Ki-67 index, presence of cysts, enhancement, and 1q/19p co-polysomy are linked to the occurrence of calcification in oligodendrogliomas. Understanding these associations can aid in diagnosing and managing patients with this type of brain tumor [ 30 ]. Pilocytic astrocytoma: This is the most common type of glioma in children and often presents with cysts or forms a tumor nodule within a cyst [ 31 ]. This variant is more prevalent in cerebellar and hemispheric tumors. Pilocytic astrocytomas affect the optic pathways, optic nerve, and chiasm including fusiform masses. Understanding these distinctive features can aid in the diagnosis and treatment of this type of brain tumor in pediatric patients [ 32 ]. DNET: Fine calcifications are often present in DNETs, and although they do not show the characteristic "salt and pepper sign," which commonly associated with PLNTY, DNETs usually display a distinct demarcation and a multinodular appearance resembling a "soap bubble" [ 33 ]. Approximately 44% patients may present with deformity of the overlying skull [ 34 ]. The T2-FLAIR mismatch sign is seen in 72.7% of all DNET cases [ 35 ]. Pleomorphic xanthoastrocytoma (PXA): This is an exceptionally rare yet typically benign form of astrocytic tumor, accounting for an infinitesimal fraction of all astrocytomas. Its prognosis is generally favorable, and it tends to occur primarily among those in the third decade of life, though it has been detected in patients as young as 6 years old to as old as 69 years old [ 36 ]. Manifesting predominantly as supratentorial, cortically-based hemispheric masses, PXAs have a tendency to extend along leptomeninges [ 37 ]. On MRI, a characteristic finding in PXA tumors is the presence of a cystic component, which exhibits hypointensity on T1W images and hyperintensity on T2W images. The solid portion, which may comprise mural nodules or thickened cyst walls, typically appears hypointense or isointense on T1WI and slightly hyperintense on T2WI. Moreover, notable enhancement of the solid component and a peripheral rim enhancement of the cyst are visible on gadolinium-enhanced T1WI, providing valuable diagnostic clues for this rare astrocytic neoplasm [ 38 ]. Conclusion PLNTY is a class of adolescent low-grade neuroepithelial tumors with good prognosis. Definitive diagnosis of PLNTY is of great significance to avoid over-treatment and improve the quality of survival in pediatric and adolescent patients. Given the polymorphic nature of the histological morphology of PLNTY and the lack of specificity in the clinical presentation and imaging features, further diagnosis,More cases need to be included in the later stage for analysis and screening to provide more reliable and adequate scientific basis for clinical diagnosis and treatment and improve the prognosis. Abbreviations PLNTY Polymorphous Low-Grade Neuroepithelial Tumor of the Young GG Ganglioglioma pDLGG Pediatric-type diffuse low-grade gliomas DNET Dysembryoplastic neuroepithelial tumor PXA Pleomorphic xanthoastrocytoma Declarations Ethics approval and consent to participate The present study was approved by the Ethics Committee of the Affiliated Nanhua Hospital, Hengyang Medical College. The participant in this study provided written informed consent for her participation, as well as for the publication of any potentially identifiable images or data included in this article. Consent for publication All authors and the guardians of the patient agreed for the publication of this study . Competing interests The authors have declared that they have no competing interests Availability of data and materials Suppotring data are available upon request. Funding The research and publication of this article were supported by funding from the National Natural Science Foundation of China (No. 81373465). Financial support played a crucial role in ensuring the successful completion of this work, highlighting the importance of funding in academic research. Authors' contributions LJL and WL wrote the manuscript,GJS and XFY supervised the process and participated in conducting a thorough literature review.SZO, CC, XC, HQ collected and analyzed clinical data, All authors read and approved the final manuscript. Acknowledgements We express our gratitude to Professor Xuefeng Yang for his invaluable advice and support in the initial stages of the case. Additionally, we extend our thanks to Dr. Guojun Song, Director of Radiology, for his crucial assistance throughout the submission process. Their expertise greatly contributed to the successful management of this case Authors' information 1 Department of Radiology, the Affiliated Nanhua Hospital, Hengyang Medical College, University of South China, Hengyang Hunan 421002, China; 2 Department of Gastroenterology, the Affiliated Nanhua Hospital, Hengyang Medical College, University of South China, Hengyang Hunan 421002, China. 3 Department of Pathology, the Affiliated Nanhua Hospital, Hengyang Medical College, University of South China, Hengyang Hunan 421002, China. * Correspondence: Yanfen Tang [email protected] ; Lijuan Lai, Wei Li：These authors contributed equally to this work References Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021;23(8):1231–51. Huse JT, Snuderl M, Jones DTW, Brathwaite CD, Altman N, Lavi E, et al. 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[cited 2024 Mar 25]. https://thejns.org/pediatrics/view/journals/j-neurosurg-pediatr/11/5/article-p518.xml Stanescu Cosson R, Varlet P, Beuvon F, Daumas Duport C, Devaux B, Chassoux F, et al. Dysembryoplastic neuroepithelial tumors: CT, MR findings and imaging follow-up: a study of 53 cases. J Neuroradiol. 2001;28(4):230–40. Onishi S, Amatya VJ, Kolakshyapati M, Takano M, Yonezawa U, Taguchi A, et al. T2-FLAIR mismatch sign in dysembryoplasticneuroepithelial tumor. Eur J Radiol. 2020;126. 10.1016/j.ejrad.2020.108924 . Detti B, Scoccianti S, Maragna V, Lucidi S, Ganovelli M, Teriaca MA, et al. Pleomorphic Xanthoastrocytoma: a single institution retrospective analysis and a review of the literature. Radiol med. 2022;127(10):1134–41. Crespo-Rodríguez AM, Smirniotopoulos JG, Rushing EJ. MR and CT imaging of 24 pleomorphic xanthoastrocytomas (PXA) and a review of the literature. Neuroradiology. 2007;49(4):307–15. Mahajan S, Dandapath I, Garg A, Sharma MC, Suri V, Sarkar C. The evolution of pleomorphic xanthoastrocytoma: from genesis to molecular alterations and mimics. Lab Invest. 2022;102(7):670–81. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-5447824","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":382130923,"identity":"ce12cb0d-5a9d-4a16-a786-4600869b7f2d","order_by":0,"name":"Lijuan Lai","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Lijuan","middleName":"","lastName":"Lai","suffix":""},{"id":382130924,"identity":"784cb8fd-4584-4d0a-8d7d-4a1f90f08068","order_by":1,"name":"wei Li","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"wei","middleName":"","lastName":"Li","suffix":""},{"id":382130925,"identity":"36ccd0b8-7ed1-43cb-b407-333c449ea40f","order_by":2,"name":"Guojun Song","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Guojun","middleName":"","lastName":"Song","suffix":""},{"id":382130926,"identity":"4b32fdee-b77e-4dc8-85d2-ff9055ec26d0","order_by":3,"name":"Xuefeng Yang","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Xuefeng","middleName":"","lastName":"Yang","suffix":""},{"id":382130927,"identity":"49525de1-b2f9-4077-ba9e-0d29beba2d4b","order_by":4,"name":"Shizhao Ou","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Shizhao","middleName":"","lastName":"Ou","suffix":""},{"id":382130928,"identity":"6212b6c1-76ed-40b7-b776-cfe6ba9bde61","order_by":5,"name":"Chao Chen","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Chao","middleName":"","lastName":"Chen","suffix":""},{"id":382130929,"identity":"a56b0a9d-0954-4a49-8fde-2048f73bf72f","order_by":6,"name":"Xia Chen","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Xia","middleName":"","lastName":"Chen","suffix":""},{"id":382130930,"identity":"909878c1-0a9b-45dd-b491-e24c584a365a","order_by":7,"name":"Hong Qian","email":"","orcid":"","institution":"University of South China","correspondingAuthor":false,"prefix":"","firstName":"Hong","middleName":"","lastName":"Qian","suffix":""},{"id":382130931,"identity":"75f3cf73-33ca-4921-97bb-b1ba9a1a697e","order_by":8,"name":"Yanfen Tang","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAyklEQVRIiWNgGAWjYNACAwk5fvbGxocfiFHMwwYiCyyMJXsONxtLEK/lQ0XihhvpbQI8xGixl29+9vCLgQRjw82HbQwSDHZyug0EbWEzN5YxkGBmnJ3Y9qCAIdnY7ABhh5lJSxhIsDFLJ7YbSDAcSNxGWAv7N5AWHjbJg20SPMRp4TGT/GAgIcEjwUislmM5ZdLAeAHakwgMZAMi/MLefHyb5I8/dfX7jx9/+PBDhZ0cQS0gwIyIDgMilIMA4w8iFY6CUTAKRsEIBQCDsTndkGLPEQAAAABJRU5ErkJggg==","orcid":"","institution":"University of South China","correspondingAuthor":true,"prefix":"","firstName":"Yanfen","middleName":"","lastName":"Tang","suffix":""}],"badges":[],"createdAt":"2024-11-13 14:38:14","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-5447824/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-5447824/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":71805382,"identity":"f2458e17-58fa-47a4-813d-79262f06853d","added_by":"auto","created_at":"2024-12-18 17:25:27","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":322914,"visible":true,"origin":"","legend":"\u003cp\u003eImaging examinations of the patient’s. (A) CT showed a round cystic solid mass in the right temporo-parietal lobe. Two slightly hyperdense nodules were seen in the wall of the capsule, punctate and striated calcifications were seen in the bicuspid nodules. (B) A cystic solid mass was seen in the right temporoparietal lobe, with mostly low signal and a slightly low signal wall nodule on T1WI. (C、E) The cystic component of the mass in the T2-weighted sequence is high signal, and the wall nodules are slightly high signal. (D) The cystic component of the T2-FLAIR sequence is slightly high signal, the wall nodule is high signal, and the edge of the lesion is well defined. (F) MRI enhancement of the wall nodule shows inhomogeneous enhancement,(G-H) No signs of diffusion restriction in wall nodules.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-5447824/v1/56fb330cd26264286717d5a9.png"},{"id":71805384,"identity":"e162933c-6cad-4ee2-b1a6-c3e82ce6cb1d","added_by":"auto","created_at":"2024-12-18 17:25:27","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1033187,"visible":true,"origin":"","legend":"\u003cp\u003ePathological features of PLNTY. (A) Tumor cells infiltrating and growing, visible as slender blood vessels, focal with oligodendroglioma-like components (round nuclei, perinuclear halos, chicken-claw-like vessels). (B) Extensive foci of calcification (scattered or fused calcifications). (C) CD34 was diffusely strongly positive, with scattered cells in branched protrusions. (D) GFAP (+). (E) Ki-67 (+) about 1%. (F) IDH-1 (-).\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-5447824/v1/69dd329b56d0492abe264c16.png"},{"id":71805383,"identity":"8918b9e3-f6f1-4287-8d5d-93a113b56347","added_by":"auto","created_at":"2024-12-18 17:25:27","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":187200,"visible":true,"origin":"","legend":"\u003cp\u003ePost-operative CT and MRI images \u0026nbsp;(A)Right temporoparietal cystic mass reduced in size, wall nodules disappeared, and a small epidural hematoma in the right temporoparietal region. (B) The mass was hyposignalized on T1-weighted images, and there was a new lesser pneumoperitoneum in the lesion. \u0026nbsp;(C) The lesion was high signal on T2-weighted images and no wall nodules were seen. (D) Enhanced scan with a little mild enhancement at the edge of the lesion.\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-5447824/v1/41f4e9b633f5d1c233d19e52.png"},{"id":85363526,"identity":"6a5a17bd-5b4f-4f04-af23-2419a2062d35","added_by":"auto","created_at":"2025-06-25 06:24:20","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2196362,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-5447824/v1/e675fb04-f5d1-4f71-8877-c4f9e4a2c7ae.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Misdiagnosis of the Polymorphous Low-Grade Neuroepithelial Tumor of the Young as Ganglioglioma，A Case Report","fulltext":[{"header":"Background","content":"\u003cp\u003ePolymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a rare low-grade brain tumor that is classified as a childhood-type diffuse low-grade glioma by the 2021 WHO Classification of Tumors of the Central Nervous System [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. The analysis and application of imaging features play a crucial role in the diagnosis and treatment of neurological tumors. Polymorphic low-grade neuroepithelial tumor of the young (PLNTY) represents a set of solid tumors that predominantly affect adolescents. It was first described by Huse et al. in 2017[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. The low incidence of PLNTY and the similarity of its clinical presentation and imaging features with other types of neuroepithelial tumors have made it important to diagnose PLNTY accurately. Despite its morphological variability, PLNTY exhibits unifying hallmarks such as oligodendroglioma-like cellular components, infiltrative growth patterns, and intense CD34 immunopositivity. These unique clinicopathologic features and molecular genetic alterations provide an important basis for the diagnosis of PLNTY [\u003cspan additionalcitationids=\"CR2\" citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 14-year-old girl presented with acute headache, nausea, and vomiting, but lacked classic epileptic signs. The patient was admitted to a local hospital for a complete cranial computed tomography (CT), which suggested space-occupying lesions in the right temporal lobe. Additionally, the patient reported having poor mental health, appetite, and sleep since the onset of the disease. She was also lethargic and poorly refractive, with a Glasgow Coma Scale (GCS) score of E3V5M6\u0026thinsp;=\u0026thinsp;14.The results of laboratory examination are presented below(Table\u0026nbsp;1): routine blood test showed that the white blood cell count was 15.32\u0026times;10\u003csup\u003e9\u003c/sup\u003e/L; neutrophil ratio was 92.3%; neutrophil count was 14.15\u0026times;10\u003csup\u003e9\u003c/sup\u003e/L; hemoglobin level was 106 g/l; FDP was 17.21 ug/mL; and the plasma D-dimer value was 6.55 mg/L. The nucleic acid test for pneumococcal pneumonia was negative.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\n \u003cdiv class=\"colspec\"\u003eTable.1. Abnormal Laboratory Findings\u003c/div\u003e\n \u003ctable id=\"Taba\" border=\"1\"\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003ePatient\u0026rsquo;s results\u003c/p\u003e\n \u003c/th\u003e\n \u003cth align=\"left\"\u003e\n \u003cp\u003eNormal range\u003c/p\u003e\n \u003c/th\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003ewhite blood cell(\u0026times;10\u003csup\u003e9\u003c/sup\u003e/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e15.32\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e4.0\u0026ndash;10.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eneutrophil ratio(%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e92.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e50\u0026ndash;70\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eneutrophil(\u0026times;10\u003csup\u003e9\u003c/sup\u003e/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e14.15\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e2.0\u0026ndash;7.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003ehemoglobin (g/l)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e106\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e110\u0026ndash;165\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eFDP(ug/mL)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e17.21\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e0\u0026ndash;5.0\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003eD-dimer(mg/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e6.55\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd align=\"left\"\u003e\n \u003cp\u003e0-0.55\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e\n\u003cp\u003eThe patient was admitted to the hospital and underwent a CT examination, which revealed a cystic occupation in right temporo-parietal lobe, with a visible wall nodule measuring 6.3 cm \u0026times; 4.7 cm, and foci of calcification were visible at the edges of the lesion(Figure\u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003eA); in order to clarify the nature of the lesion, the patient underwent a cranial MRI and enhancement scanning examination(Fig. \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003eB-H༉. then the patient underwent surgical intervention on the second day. during the surgery, the brain tissue was slightly swollen, and the cortex appeared grayish. The head of the tumor was exposed, so a cerebral puncture needle was inserted into the cystic cavity at the head of the tumor, and about 15 mL of cystic fluid was aspirated. The brain tissue collapsed, and the cortical fistula was taken to further resect the tumor. Postoperative immunohistochemistry revealed an intracranial space as a low-grade glioma.\u003c/p\u003e\n\u003cp\u003eImmunohistochemical expression showed CD34 (diffuse\u0026thinsp;+\u0026thinsp;in some areas), glial fibrillary acidic protein (GFAP) (+), P53 (partially+, wild-type), S-100 (+), actin (-), Ki-67 (about 1%+), neuron-specific enolase (NSE) (partially+), Syn (+), IDH-1 (-), and epithelial membrane antigen (EMA) (\u0026plusmn;). Combined with the hematoxylin-eosin (HE) staining pattern, the diagnosis was suggestive of PLNTY (WHO grade 1) (Fig. \u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e). Postoperatively, the patient reviewed the cranial CT and MRI, and the mass was reduced in size compared with the previous one, and the size of the mass was 4.5cm\u0026times;2.6cm\u0026times;4.3cm in the postoperative review, the original wall nodule was not shown, and a small amount of new epidural hematoma was added on the right parietal side, and soft tissue edema of the scalp was found on the right temporal parietal side((Fig. 3).After treatment, the patient\u0026apos;s postoperative headache, dizziness, nausea, and vomiting symptoms were relieved. Up until now, the girl has been followed up for 20 months. She recovered well postoperatively and was seizure-free.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003e \u003cb\u003e3.1\u003c/b\u003e \u003cb\u003eClinical characteristics and prognosis of PLNTY\u003c/b\u003e\u003c/p\u003e \u003cp\u003eThe strong epileptogenic propensity of PLNTY can cause patients to experience early-onset epilepsy or refractory epilepsy [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e], Nevertheless, the presence of PLNTYs may also be accompanied by headaches, as with oligodendrogliomas [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Our patient\u0026rsquo;s clinical presentation differs from previous literature reports in that this patient was admitted with a sudden onset of dizziness and headache with nausea and vomiting and did not present with typical seizure symptoms. PLNTY is more likely to develop in female patients, which is a neuroepithelial tumor that mostly appears in children and adolescents [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eGanglioglioma is low incidence and is most common in children and young adults. Ganglioglioma occur predominantly in the temporal lobe. On imaging, GGs can be divided into cystic tumors (more than a 90% cystic component), cystic solid tumors (cystic component 10\u0026ndash;90%), and solid tumors (cystic component less than 10%), of which cystic tumors account for about 44\u0026ndash;80%[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. This reported case is a 14-year-old adolescent and the lesion occurred in the temporal lobe with a predominantly cystic component, which led us to start the diagnostic consideration of a ganglioglioma. PLNTY is typically considered a benign tumor in most studies [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan additionalcitationids=\"CR10 CR11\" citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]. In a survey conducted by Huse et al., seven out of eight patients who underwent gross total resection of PLNTY achieved disease-free status over a mean follow-up period of 50.6 months. Interestingly, only one patient exhibited potential tumor recurrence, evident from the imaging results, 36 months after surgery [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. This result highlights the importance of surgical resection in the management of PLNTY, especially in achieving disease-free status. However, PLNTYs located in surgically unresectable areas pose a particular challenge. Here, targeted molecular therapy targeting the MAPK pathway emerged as a promising treatment option. Specifically, BRAF/MEK inhibitors have the potential to exert both anti-epileptic and anti-tumor effects in such cases [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. This strategy addresses the seizures that often accompany PLNTYs and aims to halt or reverse the malignant transformation of these tumors. In conclusion, while PLNTYs are typically benign, the risk of malignant transformation cannot be overlooked. The discovery of specific molecular markers such as the BRAF V600E mutation, provides valuable insights into the biology of these tumors and potential therapeutic strategies. With further research, targeted molecular therapy may become a vital tool in the armamentarium against PLNTYs, especially in those located in challenging-to-resect areas.\u003c/p\u003e\n\u003ch3\u003e3.2 Molecular and histological features of PLNTY\u003c/h3\u003e\n\u003cp\u003eThe 2021 WHO classification of CNS tumors has introduced new categories based on molecular characteristics [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. Within the WHO CNS5 classification, diffuse gliomas are grouped into adult and pediatric types [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]. The pediatric type has similar histological morphology and a significantly different prognosis [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. The WHO CNS5 categorizes pDLGG into four specific tumor types: I) diffuse astrocytoma with MYB- or MYBL1 alterations; II) angiocentric glioma (AG); III) PLNTY; and IV) diffuse low-grade glioma with MAPK pathway alterations [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. This classification system aims to streamline the diagnosis and treatment of these distinct tumor types. PLNTY exhibits several distinct characteristics such as the presence of oligodendroglial-like cells in the temporal lobes and cortical/subcortical regions, dense calcification, infiltrative growth patterns, and diffuse expression of CD34 [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e, \u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]. It is also common to see strong positivity for GFAP and OLIG2, while PLNTY generally remains negative for IDH1, R132H, EMA, NeuN, and other neuroendocrine markers. The MIB-1 proliferation index remains low, further supporting its benign nature.\u003c/p\u003e \u003cp\u003ePLNTY, has been found to play a role in oncogenesis through the MAPK and PI3K/mTOR signaling pathways given the FGFR2-CTNNA3 fusion, as reported by Surrey et al. [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. PLNTY is histologically characterized by calcification and oligodendrocyte-like features, infiltrative growth pattern, and intense CD34 immunopositivity. With regard to molecular genetic alterations, most PLNTY cases are caused by a mutation in the BRAF V600E gene, which accounts for approximately 60% of all mutations. For the diagnosis of PLNTY, these unique clinicopathologic characteristics and molecular genetic alterations are important. Younger patients are more likely to develop non-BRAF V600E tumors [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. PLNTY shows low rates of mitosis, necrosis, and microvascular proliferation, distinguishing it from similar tumors such as pleomorphic xanthoastrocytoma (PXA), Ganglioglioma, pilocytic astrocytoma, and dysembryoplastic neuroepithelial tumor (DNET). Unlike other brain tumors, PLNTY lacks specific characteristic structures such as neurocytic, eosinophilic granular bodies, myxoid microcysts, and dysmorphic neuronal/ganglion cell forms [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. Instead, PLNTY commonly exhibits alterations in BRAF or FGFR2/FGFR3, resulting in a low Ki-67 labeling index, consistent with its classification as a low-grade tumor[\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e].\u003c/p\u003e\n\u003ch3\u003e3.3 Imaging characteristics and differential diagnosis of PLNTY\u003c/h3\u003e\n\u003cp\u003ePLNTY tends to predominantly appear in the cerebral hemispheres, with the temporal lobe being the most common location followed by the occipital, frontal, and parietal lobes. These tumors usually have clearly defined boundaries and are most often seen in the right temporal lobe, with only a small percentage found in the ventricles [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. A distinctive feature of PLNTY is the presence of a delineated mass in the temporal lobe that shows central calcifications, peripheral cysts, and a mix of signals on imaging tests. There are specific neuroimaging patterns that strongly suggest the presence of PLNTY [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e], including a poorly defined cortical lesion with a \u0026ldquo;salt and pepper\u0026rdquo; appearance on T2-weighted imaging and calcification on CT scans [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e, \u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e]. Although coarse calcifications are less common in children, they can develop over time. Additionally, the trans-mantle-like sign and adjacent cortical dysplasia are key characteristics of these tumors [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]. It is crucial to distinguish PLNTY from other common brain tumors found in the temporal lobe such as ganglioglioma, oligodendroglioma, and DNET. By identifying the distinct features of PLNTY on imaging tests, healthcare providers can make a more accurate diagnosis and determine the best course of treatment for patients with these types of tumors.\u003c/p\u003e \u003cp\u003ePLNTY showed characteristic calcification with subcortical localization and frequent temporal lobe localization [\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]. The most important imaging feature in our patient was a large capsule containing small nodules and visible calcifications in the temporoparietal lobe. This specific feature known as cysts with a mural nodules tumor is commonly seen in various intracranial neoplastic lesions such as ganglioglioma, pilocytic astrocytoma, hemangioblastoma, and pleomorphic xanthoastrocytoma [\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]. Considering the patient's age, tumor location, and imaging findings, our initial MRI report was suggestive of ganglioglioma. However, upon further examination, the final pathologic diagnosis revealed it to be PLNTY. Therefore, PLNTY needs to be mainly distinguished from ganglioglioma, oligodendroglioma, pilocytic astrocytoma, DNET, and pleomorphic xanthoastrocytoma (PXA).\u003c/p\u003e \u003cp\u003eGanglioglioma: Ganglioglioma is a unique type of brain tumor composed of glial cells and differentiated neurons. Although rare, it accounts for a small percentage of all primary brain tumors, with the majority affecting individuals under 30 years old [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]. Around 50% gangliogliomas show a cyst with a mural nodule on radiological examination. Other presentation comprises a solid tumor with low-to-intermediate T1-weighted (T1W) and high T2W signal or masses with predominant T1 hyperintensity [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eOligodendroglioma: These are most commonly found in the frontal lobe and are the third-most frequent type of glial neoplasm. They occur more often in male patients [\u003cspan citationid=\"CR28\" class=\"CitationRef\"\u003e28\u003c/span\u003e]. There is a high incidence of calcification in oligodendrogliomas that is visible on non-enhanced CT in up to 90% cases [\u003cspan citationid=\"CR29\" class=\"CitationRef\"\u003e29\u003c/span\u003e]. Factors such as tumor grade, size, MGMT methylation levels, extent of resection, midline crossing rates, Ki-67 index, presence of cysts, enhancement, and 1q/19p co-polysomy are linked to the occurrence of calcification in oligodendrogliomas. Understanding these associations can aid in diagnosing and managing patients with this type of brain tumor [\u003cspan citationid=\"CR30\" class=\"CitationRef\"\u003e30\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePilocytic astrocytoma: This is the most common type of glioma in children and often presents with cysts or forms a tumor nodule within a cyst [\u003cspan citationid=\"CR31\" class=\"CitationRef\"\u003e31\u003c/span\u003e]. This variant is more prevalent in cerebellar and hemispheric tumors. Pilocytic astrocytomas affect the optic pathways, optic nerve, and chiasm including fusiform masses. Understanding these distinctive features can aid in the diagnosis and treatment of this type of brain tumor in pediatric patients [\u003cspan citationid=\"CR32\" class=\"CitationRef\"\u003e32\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eDNET: Fine calcifications are often present in DNETs, and although they do not show the characteristic \"salt and pepper sign,\" which commonly associated with PLNTY, DNETs usually display a distinct demarcation and a multinodular appearance resembling a \"soap bubble\" [\u003cspan citationid=\"CR33\" class=\"CitationRef\"\u003e33\u003c/span\u003e]. Approximately 44% patients may present with deformity of the overlying skull [\u003cspan citationid=\"CR34\" class=\"CitationRef\"\u003e34\u003c/span\u003e]. The T2-FLAIR mismatch sign is seen in 72.7% of all DNET cases [\u003cspan citationid=\"CR35\" class=\"CitationRef\"\u003e35\u003c/span\u003e].\u003c/p\u003e \u003cp\u003ePleomorphic xanthoastrocytoma (PXA): This is an exceptionally rare yet typically benign form of astrocytic tumor, accounting for an infinitesimal fraction of all astrocytomas. Its prognosis is generally favorable, and it tends to occur primarily among those in the third decade of life, though it has been detected in patients as young as 6 years old to as old as 69 years old [\u003cspan citationid=\"CR36\" class=\"CitationRef\"\u003e36\u003c/span\u003e]. Manifesting predominantly as supratentorial, cortically-based hemispheric masses, PXAs have a tendency to extend along leptomeninges [\u003cspan citationid=\"CR37\" class=\"CitationRef\"\u003e37\u003c/span\u003e]. On MRI, a characteristic finding in PXA tumors is the presence of a cystic component, which exhibits hypointensity on T1W images and hyperintensity on T2W images. The solid portion, which may comprise mural nodules or thickened cyst walls, typically appears hypointense or isointense on T1WI and slightly hyperintense on T2WI. Moreover, notable enhancement of the solid component and a peripheral rim enhancement of the cyst are visible on gadolinium-enhanced T1WI, providing valuable diagnostic clues for this rare astrocytic neoplasm [\u003cspan citationid=\"CR38\" class=\"CitationRef\"\u003e38\u003c/span\u003e].\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003ePLNTY is a class of adolescent low-grade neuroepithelial tumors with good prognosis. Definitive diagnosis of PLNTY is of great significance to avoid over-treatment and improve the quality of survival in pediatric and adolescent patients. Given the polymorphic nature of the histological morphology of PLNTY and the lack of specificity in the clinical presentation and imaging features, further diagnosis,More cases need to be included in the later stage for analysis and screening to provide more reliable and adequate scientific basis for clinical diagnosis and treatment and improve the prognosis.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003ePLNTY \u0026nbsp; Polymorphous Low-Grade Neuroepithelial Tumor of the Young\u003c/p\u003e\n\u003cp\u003eGG \u0026nbsp;Ganglioglioma\u003c/p\u003e\n\u003cp\u003epDLGG \u0026nbsp;Pediatric-type diffuse low-grade gliomas\u003c/p\u003e\n\u003cp\u003eDNET \u0026nbsp;Dysembryoplastic neuroepithelial tumor\u003c/p\u003e\n\u003cp\u003ePXA \u0026nbsp;Pleomorphic xanthoastrocytoma\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003e\u003cem\u003eEthics approval and consent to participate\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe present study was approved by the Ethics Committee of the Affiliated Nanhua Hospital, Hengyang Medical College. The participant in this study provided written informed consent for her participation, as well as for the publication of any potentially identifiable images or data included in this article.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eConsent for publication\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll authors and the guardians of the\u0026nbsp;patient agreed for the publication of this study .\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eCompeting interests\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors have declared that they have no competing interests\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAvailability of data and materials\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eSuppotring data are available upon request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eFunding\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe research and publication of this article were supported by funding from the National Natural Science Foundation of China (No. 81373465). Financial support played a crucial role in ensuring the successful completion of this work, highlighting the importance of funding in academic research.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAuthors\u0026apos; contributions\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eLJL and WL wrote the manuscript,GJS and XFY supervised the process and participated in conducting a thorough literature review.SZO, CC, XC, HQ collected and analyzed clinical data, All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAcknowledgements\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe express our gratitude to Professor Xuefeng Yang for his invaluable advice and support in the initial stages of the case. Additionally, we extend our thanks to Dr. Guojun Song, Director of Radiology, for his crucial assistance throughout the submission process. Their expertise greatly contributed to the successful management of this case\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAuthors\u0026apos; information\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e1 Department of Radiology, the Affiliated Nanhua Hospital, Hengyang Medical College, University of South China, Hengyang Hunan 421002, China;\u003c/p\u003e\n\u003cp\u003e2 Department of Gastroenterology, the Affiliated Nanhua Hospital, Hengyang Medical College, University of South China, Hengyang Hunan 421002, China.\u003c/p\u003e\n\u003cp\u003e3 Department of Pathology, the Affiliated Nanhua Hospital, Hengyang Medical College, University of South China, Hengyang Hunan 421002, China.\u003c/p\u003e\n\u003cp\u003e* Correspondence: Yanfen Tang [email protected];\u003c/p\u003e\n\u003cp\u003eLijuan Lai, Wei Li：These authors contributed equally to this work\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eLouis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, et al. 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MR and CT imaging of 24 pleomorphic xanthoastrocytomas (PXA) and a review of the literature. Neuroradiology. 2007;49(4):307\u0026ndash;15.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMahajan S, Dandapath I, Garg A, Sharma MC, Suri V, Sarkar C. The evolution of pleomorphic xanthoastrocytoma: from genesis to molecular alterations and mimics. Lab Invest. 2022;102(7):670\u0026ndash;81.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"PLNTY, molecular features, imaging characteristics, differential diagnosis, brain tumor","lastPublishedDoi":"10.21203/rs.3.rs-5447824/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-5447824/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003ePLNTY traditionally manifests as an epileptogenic subtype, posing challenges in differential diagnosis. The low incidence of PLNTY and the similarity of its clinical presentation and imaging features with other types of neuroepithelial tumors have made it important to diagnose PLNTY accurately.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase presentation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe report here an unusual case of a PLNTY in a 14-year-old girl who was admitted to the hospital with acute headache, nausea, and vomiting, initially misdiagnosed as ganglioglioma on imaging, and then diagnosed as pleomorphic low-grade neuroepithelial tumor (PLNTY) on surgical and pathological analyses. The patient did not present with typical epileptic symptoms, PLNTY is a rare adolescent low-grade brain tumor with imaging manifestations similar to those of other neuroepithelial tumors, which is easy to lead to misdiagnosis.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003ePLNTY is a class of adolescent low-grade neuroepithelial tumors with good prognosis. Definitive diagnosis of PLNTY is of great significance to avoid over-treatment and improve the quality of survival in pediatric and adolescent patients.\u003c/p\u003e","manuscriptTitle":"Misdiagnosis of the Polymorphous Low-Grade Neuroepithelial Tumor of the Young as Ganglioglioma，A Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-12-18 17:25:21","doi":"10.21203/rs.3.rs-5447824/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"7917bbf4-3b10-4363-8cb4-8bc3f326744f","owner":[],"postedDate":"December 18th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-06-25T06:23:57+00:00","versionOfRecord":[],"versionCreatedAt":"2024-12-18 17:25:21","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5447824","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5447824","identity":"rs-5447824","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}