Expanding Horizons: A Novel Presentation of Posterior Fossa Pilocytic Astrocytoma With Intracranial and Spinal Leptomeningeal Spread

Expanding Horizons: A Novel Presentation of Posterior Fossa Pilocytic Astrocytoma With Intracranial and Spinal Leptomeningeal Spread | 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 Expanding Horizons: A Novel Presentation of Posterior Fossa Pilocytic Astrocytoma With Intracranial and Spinal Leptomeningeal Spread Muhammad Kaleem, Nadia Hanif, Ehsan Bari, Muhammad Tahir Khan This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8025242/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 Pilocytic astrocytoma is a low-grade tumor with excellent prognosis after total resection. However, leptomeningeal spread leads to unfavorable prognosis. A novel case of pilocytic astrocytoma (PCA) with intracranial as well as spinal leptomeningeal dissemination (LD) of a 15-year-old boy who presented with headache, vomiting and fainting spells. A magnetic resonance imaging (MRI) brain showed a mass lesion with cystic component and enhancing mural nodule in posterior fossa near cisterna magna making communication with ventricular chain with unique diffuse cranial basal leptomeningeal enhancement. Biopsy was done and case was diagnosed by department of pathology to be a PCA. Patient then underwent MRI whole neuro-axis for LD, which showed leptomeningeal spread with enhancing mural nodule within a cystic lesion in sacral portion of spinal canal. Our case report highlights the importance of close surveillance of intra-cranial as well as leptomeningeal spread of pilocytic astrocytoma. Pilocytic astrocytoma leptomeningeal spread Magnetic resonance Imaging Figures Figure 1 Figure 2 INTRODUCTION Of all primary paediatric brain tumors, PCA is the most common CNS astrocytoma in children, making for about 30% of cases [ 9 , 10 ]. Usually benign, these low-grade gliomas hardly ever show signs of spread along the neuro-axis. Though they can arise from any area that contains astrocytes, i.e. cerebrum, hypothalamus, brainstem, optic nerve as well as spinal cord [ 2 , 4 ]. In Post contrast MRI images, this tumor shows enhancement. In rare instances, both brain and spinal cord PCAs have shown aggressive characteristics, such as metastatic dissemination to the leptomeninges [ 1 ]. In 1976, the first instance of PCA with LD was documented. There were sixty cases by 2013 [ 11 ]. This case report study describes a case of leptomeningeal and spinal spread from a pilocytic astrocytoma. CASE PRESENTATION In February 2022, a 13 years old boy presented with history of headache, vomiting, generalized fits and fainting spells. Imaging was performed with the concern of raised intra-cranial pressure and evaluation of ventriculo-peritoneal (VP) shunt. A VP shunt was placed 5 years ago when patient presented with similar presentation. MRI was performed. T2 weighted images showed a large CSF signals intensity lesion with eccentrically placed nodule noted in posterior cranial fossa making communication with 4th ventricle (Fig. 1 ). On DWI/ADC images, there was restriction in nodule. Post contrast images showed enhancement of cystic wall and mural nodule as well as diffuse leptomeningeal enhancement around supra seller, cavernous, CP angle, suprasellar, pre pontine, ambient cisterns and craniocervical junction. Spread of disease was also seen in bilateral foramen Luschka and Magendie. There was no flow void area/ hemorrhage. Occipital craniotomy was done for biopsy. The histopathology report showed a biphasic appearance with compact fibrillary portions composed of elongated nuclei, bipolar piloid processes and Rosenthal fibers. The loose microcystic areas consist of cobweb like process and eosinophil granular bodies. No increased mitotic bodies seen. Diagnosis was Pilocytic Astrocytoma CNS WHO grade I. Patient developed bilateral lower limb weakness 3 months after biopsy. Then, whole neuro-axis MRI was performed with suspicion of caudal LD. MRI showed intra-dural, extra-medullary lesion with enhancing nodule and enhancing septa associated with fluid-fluid level indicative of hemorrhage. This mass is causing leftward displacement of thecal sac as well as posterior scalloping of S1 to S3 vertebral bodies. Enhancing nodular meningeal thickening was also seen on surface of the spinal cord mainly in lower dorsal (D9-D10) and lumbar region, however cord itself shows normal signals as shown in Fig. 2 . DISCUSSION Medulloblastoma, pilocytic astrocytoma, brainstem gliomas, and ependymoma are the most common posterior fossa tumors in children [ 9 ]. The age of the patient plays a crucial role, especially since atypical teratoid rhabdoid tumor is more common in children under 3 years and might look similar to medulloblastoma on scans [ 8 , 9 ]. Medulloblastoma typically affects the cerebellar vermis in children, exhibits restricted diffusion, shows higher perfusion rates, and can be found in those over 3 years as well. Ependymoma is often found in the fourth ventricle, presenting as a varied tumor that may include hemorrhage or calcifications and can extend through the fourth ventricle's foramina. While pilocytic astrocytoma frequently presents in the cerebellar hemispheres with both cystic and solid parts, it can also be found in the midline without any cystic areas and usually does not show restricted diffusion [ 9 ]. Rarely does PCA, a benign form of glioma with a good prognosis, spread along the neuro-axis. There are 60 documented cases of LD in PCA, five of which had LD at the time of diagnosis like in our case, making it incredibly rare, especially in children [ 7 , 11 ]. The behavior of Pilocytic Astrocytoma (PCA) can be highly variable, encompassing possibilities like local re-emergence, malignant evolution, as well as spreading to multiple centers or distant parts of the body [ 6 , 7 ]. When distinguishing between different kinds of pathological changes, LD and multicentric disease are identified as separate conditions [ 7 ]. In the context of primary CNS tumors in the pediatric population, leptomeningeal spread is more typically associated with ependymomas, germ-cell tumors (GCT), primitive neuroectodermal tumors, and advanced gliomas. Conversely, the occurrence of LD from low-grade gliomas is comparatively rare, with estimates suggesting it happens in 4 to 17% of cases [2,5. According to another analysis, the leptomeningeal dissemination of pilocytic astrocytoma presents in 2 to 12% of instances [ 2 , 3 ]. Our case showed pilocytic astrocytoma arising from posterior fossa near giant cisterna communicating with 4th ventricle with leptomeningeal spread in brain as well as in spine. A case report was done by Abel showed intramedullary spinal cord astrocytoma in 2-year-old boy after three surgeries, Through the leptomeninges, it was spread to the brain. This case contributes to the few number of cases of a primary spinal PCA that spread intracranially in the literature. In our case, there were both intracranial as well as spinal leptomeningeal spread which is unique and no such case is found in literature. In one case report. pilocytic astrocytomas with spinal dissemination occurred in adult patients almost two decades after the primary surgical treatment emphasizing a long-term follow-up in patients with diagnosed pilocytic astrocytoma [ 4 , 6 ]. The spread of Pilocytic Astrocytoma (PCA) does not always indicate a grim outlook, unlike with most other types of glial tumors. In many cases, patients without symptoms can live for an extended period despite the dissemination. While there's growing recognition of leptomeningeal spread in PCA patients, documentation primarily exists in the form of individual case studies or small group analyses. CONCLUSION This case report revealed atypical presentation of posterior fossa pilocytic astrocytomas (PCAs) with whole neuro-axis LD. It emphasizes the importance of long term follow up MRI examinations of the neuro-axis to promptly identify any signs of the tumor spread beyond its original location. Detecting such spread at an early stage is crucial as it has a positive impact on the effectiveness of the treatment strategies employed, potentially improving patient outcomes. Declarations Ethics approval and consent to participate Written informed consent was obtained. This research follows the most recent version of the Helsinki Declaration. Consent for publication The patient and his parents gave written informed consent for the publication of this case and the necessary radiological pictures. Competing interests There are no competing interests among the writers. Funding This research had no funding. Author Contribution MK and EB conducted literature review, drafted initial document, created images, and amended the final draft. NH oversaw the research and helped with revision. MTK revised the manuscript and edited images. The final version of the manuscript was approved by all authors. Acknowledgements None Availability of data and materials All relevant data and material has been provided with the manuscript. References Chourmouzi D, Papadopoulou E, Konstantinidis M, Syrris V, Kouskouras K, Haritanti A, Karkavelas G, Drevelegas A (2014) Manifestations of pilocytic astrocytoma: a pictorial review. Insights Imaging 5(3):387–402. https://doi.org/10.1007/s13244-014-0328-2 Abel TJ, Chowdhary A, Thapa M, Rutledge JC, Geyer JR, Ojemann J, Avellino AM (2006) Spinal cord pilocytic astrocytoma with leptomeningeal dissemination to the brain. Case report and review of the literature. J Neurosurg 105(6 Suppl):508–514. https://doi.org/10.3171/ped.2006.105.6.508 Raheja A, Singh PK, Nambirajan A, Sharma MC, Sharma BS (2015) Diffuse leptomeningeal spread of supratentorial recurrent pilocytic astrocytoma in a child. J Pediatr Neurosci 10(4):408–411. https://doi.org/10.4103/1817-1745.174452 Aryan HE, Meltzer HS, Lu DC, Ozgur BM, Levy ML, Bruce DA (2005) Management of pilocytic astrocytoma with diffuse leptomeningeal spread: two cases and review of the literature. Childs Nerv Syst 21(6):477–481. https://doi.org/10.1007/s00381-004-1002-7 Bayart CB, Ishak GE, Finn LS, Lee A, Baran F, Sun A, Gupta D, Vitanza NA (2018) Pilocytic astrocytoma with leptomeningeal spread in a patient with incontinentia pigmenti presenting with unilateral nystagmus. Pediatr Blood Cancer 65(3). https://doi.org/10.1002/pbc.26886 Alyeldien A, Teuber-Hanselmann S, Cheko A, Höll T, Scholz M, Petridis AK (2016) Diffuse Spinal Leptomeningeal Spread of a Pilocytic Astrocytoma in a 3-year-old Child. Clin Pract 31(1):813. https://doi.org/10.4081/cp.2016.813 Su YF, Chang CH, Chiou SS, Chai CY, Hwang SL, Loh JK (2017) A Case of Leptomeningeal Dissemination of Pilocytic Astrocytoma in a Child. Can J Neurol Sci 44(5):607–610. https://doi.org/10.1017/cjn.2017.22 Figueiredo EG, Matushita H, Machado AG, Plese JP, Rosemberg S, Marino R Jr. (2003) Leptomeningeal dissemination of pilocytic astrocytoma at diagnosis in childhood: two cases report. Arq Neuropsiquiatr 61(3B):842–847. https://doi.org/10.1590/s0004-282x2003000500025 de Morais RQ, da Silva RM, Corrêa DG (2021) Post-operative meningeal thickening seen on magnetic resonance imaging: tumor spread or transient reaction. JBNC-JORNAL BRASILEIRO DE NEUROCIRURGIA 14(2):207–211 CASE REPORT Post-operative meningeal thickening seen.indd Donofrio CA, Gagliardi F, Callea M, da Passano CF, Terreni MR, Cavalli A, Spina A, Acerno S, Bailo M, Elbabaa SK, Mortini P (2020) Pediatric cerebellar pilocytic astrocytoma presenting with spontaneous intratumoral hemorrhage. Neurosurg Rev 43(1):9–16. https://doi.org/10.1007/s10143-018-0969-6 Soliman RK, Budai C, Mundada P, Aljohani B, Rushing EJ, Kollias SS (2016) Suprasellar pilocytic astrocytoma in an adult with hemorrhage and leptomeningeal dissemination: case report and review of literature. Radiol Case Rep 29(4):411–418. https://doi.org/10.1016/j.radcr.2016.08.011 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-8025242","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":564979022,"identity":"bd30ad1d-9c7f-428d-a158-593775f917bc","order_by":0,"name":"Muhammad Kaleem","email":"","orcid":"","institution":"Woodlands Health Campus","correspondingAuthor":false,"prefix":"","firstName":"Muhammad","middleName":"","lastName":"Kaleem","suffix":""},{"id":564979024,"identity":"aa3a0355-b065-43ba-8708-3993a2cfde9e","order_by":1,"name":"Nadia Hanif","email":"","orcid":"","institution":"Lahore General Hospital","correspondingAuthor":false,"prefix":"","firstName":"Nadia","middleName":"","lastName":"Hanif","suffix":""},{"id":564979026,"identity":"73a5b259-4e31-43bb-9787-6c5d2b17ace8","order_by":2,"name":"Ehsan Bari","email":"","orcid":"","institution":"Lahore General Hospital","correspondingAuthor":false,"prefix":"","firstName":"Ehsan","middleName":"","lastName":"Bari","suffix":""},{"id":564979027,"identity":"5d4acfad-6962-42f2-890c-3c4a50f21efa","order_by":3,"name":"Muhammad Tahir Khan","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA3ElEQVRIiWNgGAWjYFACHoYDIIqNgYHxAYjLR1gDTAsbA7MBSICNGC0QwMbAJgG1Dj+wZz978HDljm3yfPLNxyq/5tjJAG17+OgGPlt48hIOnj1z27CNjS3ttuy2ZKDD2IyNc/A6LMfgYGPb7QQ2Nh6z25LbmIFaeNik8WrhfwPTwv+tWHJbPRFaJBC2sDF+3HaYCC03QLaA/ZJmLM247TgPGzMBv7D35xh/bNxxW16++fDDjz+3Vdvzszc/fIxPCxgwNkBoZnAcMRNSjqyF8QcxqkfBKBgFo2DEAQCUnEL2kRcuJAAAAABJRU5ErkJggg==","orcid":"","institution":"Hameed Latif Teaching Hospital","correspondingAuthor":true,"prefix":"","firstName":"Muhammad","middleName":"Tahir","lastName":"Khan","suffix":""}],"badges":[],"createdAt":"2025-11-04 06:38:27","currentVersionCode":1,"declarations":{"humanSubjects":false,"vertebrateSubjects":false,"conflictsOfInterestStatement":false,"humanSubjectEthicalGuidelines":false,"humanSubjectConsent":false,"humanSubjectClinicalTrial":false,"humanSubjectCaseReport":false,"vertebrateSubjectEthicalGuidelines":false},"doi":"10.21203/rs.3.rs-8025242/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-8025242/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":99318417,"identity":"27333725-58ea-4477-83e9-fb843573a631","added_by":"auto","created_at":"2025-12-31 16:33:08","extension":"docx","order_by":0,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":332443,"visible":true,"origin":"","legend":"","description":"","filename":"Figures.docx","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/8971ac70484b52b59f5240fc.docx"},{"id":99218379,"identity":"aecaead5-6c4d-4de9-a13e-ae52c02cadfa","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"docx","order_by":1,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":345260,"visible":true,"origin":"","legend":"","description":"","filename":"Menuscript.docx","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/13f36a2a6373d941a36f8f53.docx"},{"id":99218377,"identity":"b036cc22-c75b-4ea9-a905-6faaef553b1d","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"json","order_by":2,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":5239,"visible":true,"origin":"","legend":"","description":"","filename":"fab90ebab9ed4b909d431106fa3adcdc.json","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/7fe93aed84c6a4965b6f09a0.json"},{"id":99218382,"identity":"deb99ada-de04-4897-a186-a5481aad8187","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"xml","order_by":3,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":36578,"visible":true,"origin":"","legend":"","description":"","filename":"fab90ebab9ed4b909d431106fa3adcdc1enriched.xml","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/67b8de4471b45bb1019062c0.xml"},{"id":99317994,"identity":"20351d39-0117-46fe-8374-d5348be22f6f","added_by":"auto","created_at":"2025-12-31 16:31:07","extension":"jpeg","order_by":4,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":175088,"visible":true,"origin":"","legend":"","description":"","filename":"floatimage1.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/362a6c8d60a1b08b669edd26.jpeg"},{"id":99317181,"identity":"8ccd632c-1651-4ec4-85f3-7e931bc74ed4","added_by":"auto","created_at":"2025-12-31 16:29:44","extension":"jpeg","order_by":5,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":815042,"visible":true,"origin":"","legend":"","description":"","filename":"floatimage2.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/1a2607e37133a7f8bca23a79.jpeg"},{"id":99317261,"identity":"98400cf3-a199-493e-ad2e-9410b56aa295","added_by":"auto","created_at":"2025-12-31 16:29:51","extension":"jpeg","order_by":6,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":971089,"visible":true,"origin":"","legend":"","description":"","filename":"floatimage3.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/4faccc755bf8a97944b81b63.jpeg"},{"id":99218385,"identity":"18c0a1ca-a3e1-45cc-beb8-e5eb8710c54a","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"jpeg","order_by":7,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":815006,"visible":true,"origin":"","legend":"","description":"","filename":"floatimage4.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/a699824734f474094f8ca944.jpeg"},{"id":99218384,"identity":"be15ffea-be97-4bbf-b2dd-18b3d64d8bbd","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"png","order_by":8,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":144993,"visible":true,"origin":"","legend":"","description":"","filename":"Onlinefloatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/92d459be4ff65bd1e9a1106a.png"},{"id":99218391,"identity":"b2561857-9915-4676-bfcd-51b4a229a7e0","added_by":"auto","created_at":"2025-12-30 09:18:50","extension":"png","order_by":9,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":1499384,"visible":true,"origin":"","legend":"","description":"","filename":"Onlinefloatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/e4756c0cf03cdac09c9ef430.png"},{"id":99218387,"identity":"2f6e95ea-86b2-4097-a379-8e581dbddae8","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"png","order_by":10,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":327722,"visible":true,"origin":"","legend":"","description":"","filename":"Onlinefloatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/38979f17df6c22682b7a24dc.png"},{"id":99318585,"identity":"ccf26dec-2b8e-4bbb-88d6-2ef38c8e88df","added_by":"auto","created_at":"2025-12-31 16:33:40","extension":"png","order_by":11,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":1499361,"visible":true,"origin":"","legend":"","description":"","filename":"Onlinefloatimage4.png","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/b4d42a37bce1b9b2ea69677f.png"},{"id":99218389,"identity":"10168167-c54a-46a9-a3ea-02b6bb442a78","added_by":"auto","created_at":"2025-12-30 09:18:50","extension":"xml","order_by":12,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":36141,"visible":true,"origin":"","legend":"","description":"","filename":"fab90ebab9ed4b909d431106fa3adcdc1structuring.xml","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/609ac549cf60ab93ec35c795.xml"},{"id":99218388,"identity":"5da336d1-4fea-465e-ac5c-bcba95b9b258","added_by":"auto","created_at":"2025-12-30 09:18:50","extension":"html","order_by":13,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":40518,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/1d779e083d6e9b1fe6d5bc9a.html"},{"id":99218376,"identity":"c12652c4-e535-4df8-8adf-4d421032a6f7","added_by":"auto","created_at":"2025-12-30 09:18:49","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":172924,"visible":true,"origin":"","legend":"\u003cp\u003ePost contrast MRI brain images. \u003cstrong\u003e(A \u0026amp; B)\u003c/strong\u003e axial and sagittal T1-weighted images showing posterior fossa midline cystic lesion with enhancing mural nodule communicating with 4\u003csup\u003eth\u003c/sup\u003e ventricle as indicated with white arrows. \u003cstrong\u003e(C \u0026amp; D)\u003c/strong\u003e Sagittal and coronal T1-weighted images depicted leptomeningeal enhancement in ambient and suprasellar cisterns. as indicated by white arrows.\u003c/p\u003e","description":"","filename":"1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/ea5622b0a8e36b92a9466608.jpg"},{"id":99318509,"identity":"80915404-38ba-4d5b-a4b5-9f99b2613f2c","added_by":"auto","created_at":"2025-12-31 16:33:30","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":145125,"visible":true,"origin":"","legend":"\u003cp\u003eMRI Lumbosacral spine. \u003cstrong\u003e(A \u0026amp; B)\u003c/strong\u003e Sagittal and axial T2 Images showing cystic lesion in sacral portion of spinal canal along with the mural nodule. \u003cstrong\u003e(C \u0026amp; D)\u003c/strong\u003e axial and sagittal post contrast images showing the abnormal leptomeningeal enhancement as well as enhancement of nodule in above mentioned cystic component.\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/d70da60c59ed1c8815fdcc66.jpg"},{"id":100363958,"identity":"f4ec6fe4-90bd-4f06-a33e-fdb9009fd215","added_by":"auto","created_at":"2026-01-16 07:52:14","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":657325,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8025242/v1/28021d91-68ee-4681-b9ba-fb7fd8f9d6e0.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003eExpanding Horizons: A Novel Presentation of Posterior Fossa Pilocytic Astrocytoma With Intracranial and Spinal Leptomeningeal Spread\u003c/p\u003e","fulltext":[{"header":"INTRODUCTION","content":"\u003cp\u003eOf all primary paediatric brain tumors, PCA is the most common CNS astrocytoma in children, making for about 30% of cases [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e]. Usually benign, these low-grade gliomas hardly ever show signs of spread along the neuro-axis. Though they can arise from any area that contains astrocytes, i.e. cerebrum, hypothalamus, brainstem, optic nerve as well as spinal cord [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. In Post contrast MRI images, this tumor shows enhancement. In rare instances, both brain and spinal cord PCAs have shown aggressive characteristics, such as metastatic dissemination to the leptomeninges [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. In 1976, the first instance of PCA with LD was documented. There were sixty cases by 2013 [\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. This case report study describes a case of leptomeningeal and spinal spread from a pilocytic astrocytoma.\u003c/p\u003e"},{"header":"CASE PRESENTATION","content":"\u003cp\u003eIn February 2022, a 13 years old boy presented with history of headache, vomiting, generalized fits and fainting spells. Imaging was performed with the concern of raised intra-cranial pressure and evaluation of ventriculo-peritoneal (VP) shunt. A VP shunt was placed 5 years ago when patient presented with similar presentation. MRI was performed. T2 weighted images showed a large CSF signals intensity lesion with eccentrically placed nodule noted in posterior cranial fossa making communication with 4th ventricle (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). On DWI/ADC images, there was restriction in nodule. Post contrast images showed enhancement of cystic wall and mural nodule as well as diffuse leptomeningeal enhancement around supra seller, cavernous, CP angle, suprasellar, pre pontine, ambient cisterns and craniocervical junction. Spread of disease was also seen in bilateral foramen Luschka and Magendie. There was no flow void area/ hemorrhage. Occipital craniotomy was done for biopsy. The histopathology report showed a biphasic appearance with compact fibrillary portions composed of elongated nuclei, bipolar piloid processes and Rosenthal fibers. The loose microcystic areas consist of cobweb like process and eosinophil granular bodies. No increased mitotic bodies seen. Diagnosis was Pilocytic Astrocytoma CNS WHO grade I. Patient developed bilateral lower limb weakness 3 months after biopsy. Then, whole neuro-axis MRI was performed with suspicion of caudal LD. MRI showed intra-dural, extra-medullary lesion with enhancing nodule and enhancing septa associated with fluid-fluid level indicative of hemorrhage. This mass is causing leftward displacement of thecal sac as well as posterior scalloping of S1 to S3 vertebral bodies. Enhancing nodular meningeal thickening was also seen on surface of the spinal cord mainly in lower dorsal (D9-D10) and lumbar region, however cord itself shows normal signals as shown in Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"DISCUSSION","content":"\u003cp\u003eMedulloblastoma, pilocytic astrocytoma, brainstem gliomas, and ependymoma are the most common posterior fossa tumors in children [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. The age of the patient plays a crucial role, especially since atypical teratoid rhabdoid tumor is more common in children under 3 years and might look similar to medulloblastoma on scans [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Medulloblastoma typically affects the cerebellar vermis in children, exhibits restricted diffusion, shows higher perfusion rates, and can be found in those over 3 years as well. Ependymoma is often found in the fourth ventricle, presenting as a varied tumor that may include hemorrhage or calcifications and can extend through the fourth ventricle's foramina. While pilocytic astrocytoma frequently presents in the cerebellar hemispheres with both cystic and solid parts, it can also be found in the midline without any cystic areas and usually does not show restricted diffusion [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eRarely does PCA, a benign form of glioma with a good prognosis, spread along the neuro-axis. There are 60 documented cases of LD in PCA, five of which had LD at the time of diagnosis like in our case, making it incredibly rare, especially in children [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe behavior of Pilocytic Astrocytoma (PCA) can be highly variable, encompassing possibilities like local re-emergence, malignant evolution, as well as spreading to multiple centers or distant parts of the body [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. When distinguishing between different kinds of pathological changes, LD and multicentric disease are identified as separate conditions [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. In the context of primary CNS tumors in the pediatric population, leptomeningeal spread is more typically associated with ependymomas, germ-cell tumors (GCT), primitive neuroectodermal tumors, and advanced gliomas. Conversely, the occurrence of LD from low-grade gliomas is comparatively rare, with estimates suggesting it happens in 4 to 17% of cases [2,5. According to another analysis, the leptomeningeal dissemination of pilocytic astrocytoma presents in 2 to 12% of instances [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eOur case showed pilocytic astrocytoma arising from posterior fossa near giant cisterna communicating with 4th ventricle with leptomeningeal spread in brain as well as in spine. A case report was done by Abel showed intramedullary spinal cord astrocytoma in 2-year-old boy after three surgeries, Through the leptomeninges, it was spread to the brain. This case contributes to the few number of cases of a primary spinal PCA that spread intracranially in the literature.\u003c/p\u003e \u003cp\u003eIn our case, there were both intracranial as well as spinal leptomeningeal spread which is unique and no such case is found in literature. In one case report. pilocytic astrocytomas with spinal dissemination occurred in adult patients almost two decades after the primary surgical treatment emphasizing a long-term follow-up in patients with diagnosed pilocytic astrocytoma [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe spread of Pilocytic Astrocytoma (PCA) does not always indicate a grim outlook, unlike with most other types of glial tumors. In many cases, patients without symptoms can live for an extended period despite the dissemination. While there's growing recognition of leptomeningeal spread in PCA patients, documentation primarily exists in the form of individual case studies or small group analyses.\u003c/p\u003e"},{"header":"CONCLUSION","content":"\u003cp\u003eThis case report revealed atypical presentation of posterior fossa pilocytic astrocytomas (PCAs) with whole neuro-axis LD. It emphasizes the importance of long term follow up MRI examinations of the neuro-axis to promptly identify any signs of the tumor spread beyond its original location. Detecting such spread at an early stage is crucial as it has a positive impact on the effectiveness of the treatment strategies employed, potentially improving patient outcomes.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e \u003ch2\u003eEthics approval and consent to participate\u003c/h2\u003e \u003cp\u003eWritten informed consent was obtained. This research follows the most recent version of the Helsinki Declaration.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent for publication\u003c/strong\u003e \u003cp\u003eThe patient and his parents gave written informed consent for the publication of this case and the necessary radiological pictures.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eCompeting interests\u003c/strong\u003e \u003cp\u003eThere are no competing interests among the writers.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eThis research had no funding.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eMK and EB conducted literature review, drafted initial document, created images, and amended the final draft. NH oversaw the research and helped with revision. MTK revised the manuscript and edited images. The final version of the manuscript was approved by all authors.\u003c/p\u003e\u003ch2\u003eAcknowledgements\u003c/h2\u003e \u003cp\u003eNone\u003c/p\u003e\u003ch2\u003eAvailability of data and materials\u003c/h2\u003e \u003cp\u003eAll relevant data and material has been provided with the manuscript.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eChourmouzi D, Papadopoulou E, Konstantinidis M, Syrris V, Kouskouras K, Haritanti A, Karkavelas G, Drevelegas A (2014) Manifestations of pilocytic astrocytoma: a pictorial review. Insights Imaging 5(3):387\u0026ndash;402. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s13244-014-0328-2\u003c/span\u003e\u003cspan address=\"10.1007/s13244-014-0328-2\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAbel TJ, Chowdhary A, Thapa M, Rutledge JC, Geyer JR, Ojemann J, Avellino AM (2006) Spinal cord pilocytic astrocytoma with leptomeningeal dissemination to the brain. Case report and review of the literature. J Neurosurg 105(6 Suppl):508\u0026ndash;514. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3171/ped.2006.105.6.508\u003c/span\u003e\u003cspan address=\"10.3171/ped.2006.105.6.508\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eRaheja A, Singh PK, Nambirajan A, Sharma MC, Sharma BS (2015) Diffuse leptomeningeal spread of supratentorial recurrent pilocytic astrocytoma in a child. J Pediatr Neurosci 10(4):408\u0026ndash;411. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.4103/1817-1745.174452\u003c/span\u003e\u003cspan address=\"10.4103/1817-1745.174452\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAryan HE, Meltzer HS, Lu DC, Ozgur BM, Levy ML, Bruce DA (2005) Management of pilocytic astrocytoma with diffuse leptomeningeal spread: two cases and review of the literature. Childs Nerv Syst 21(6):477\u0026ndash;481. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s00381-004-1002-7\u003c/span\u003e\u003cspan address=\"10.1007/s00381-004-1002-7\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBayart CB, Ishak GE, Finn LS, Lee A, Baran F, Sun A, Gupta D, Vitanza NA (2018) Pilocytic astrocytoma with leptomeningeal spread in a patient with incontinentia pigmenti presenting with unilateral nystagmus. Pediatr Blood Cancer 65(3). \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1002/pbc.26886\u003c/span\u003e\u003cspan address=\"10.1002/pbc.26886\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAlyeldien A, Teuber-Hanselmann S, Cheko A, H\u0026ouml;ll T, Scholz M, Petridis AK (2016) Diffuse Spinal Leptomeningeal Spread of a Pilocytic Astrocytoma in a 3-year-old Child. Clin Pract 31(1):813. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.4081/cp.2016.813\u003c/span\u003e\u003cspan address=\"10.4081/cp.2016.813\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSu YF, Chang CH, Chiou SS, Chai CY, Hwang SL, Loh JK (2017) A Case of Leptomeningeal Dissemination of Pilocytic Astrocytoma in a Child. Can J Neurol Sci 44(5):607\u0026ndash;610. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1017/cjn.2017.22\u003c/span\u003e\u003cspan address=\"10.1017/cjn.2017.22\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFigueiredo EG, Matushita H, Machado AG, Plese JP, Rosemberg S, Marino R Jr. (2003) Leptomeningeal dissemination of pilocytic astrocytoma at diagnosis in childhood: two cases report. Arq Neuropsiquiatr 61(3B):842\u0026ndash;847. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1590/s0004-282x2003000500025\u003c/span\u003e\u003cspan address=\"10.1590/s0004-282x2003000500025\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003ede Morais RQ, da Silva RM, Corr\u0026ecirc;a DG (2021) Post-operative meningeal thickening seen on magnetic resonance imaging: tumor spread or transient reaction. JBNC-JORNAL BRASILEIRO DE NEUROCIRURGIA 14(2):207\u0026ndash;211 CASE REPORT Post-operative meningeal thickening seen.indd\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDonofrio CA, Gagliardi F, Callea M, da Passano CF, Terreni MR, Cavalli A, Spina A, Acerno S, Bailo M, Elbabaa SK, Mortini P (2020) Pediatric cerebellar pilocytic astrocytoma presenting with spontaneous intratumoral hemorrhage. Neurosurg Rev 43(1):9\u0026ndash;16. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s10143-018-0969-6\u003c/span\u003e\u003cspan address=\"10.1007/s10143-018-0969-6\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSoliman RK, Budai C, Mundada P, Aljohani B, Rushing EJ, Kollias SS (2016) Suprasellar pilocytic astrocytoma in an adult with hemorrhage and leptomeningeal dissemination: case report and review of literature. Radiol Case Rep 29(4):411\u0026ndash;418. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1016/j.radcr.2016.08.011\u003c/span\u003e\u003cspan address=\"10.1016/j.radcr.2016.08.011\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\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":true,"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":"Pilocytic astrocytoma, leptomeningeal spread, Magnetic resonance Imaging","lastPublishedDoi":"10.21203/rs.3.rs-8025242/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8025242/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003ePilocytic astrocytoma is a low-grade tumor with excellent prognosis after total resection. However, leptomeningeal spread leads to unfavorable prognosis. A novel case of pilocytic astrocytoma (PCA) with intracranial as well as spinal leptomeningeal dissemination (LD) of a 15-year-old boy who presented with headache, vomiting and fainting spells. A magnetic resonance imaging (MRI) brain showed a mass lesion with cystic component and enhancing mural nodule in posterior fossa near cisterna magna making communication with ventricular chain with unique diffuse cranial basal leptomeningeal enhancement. Biopsy was done and case was diagnosed by department of pathology to be a PCA. Patient then underwent MRI whole neuro-axis for LD, which showed leptomeningeal spread with enhancing mural nodule within a cystic lesion in sacral portion of spinal canal. Our case report highlights the importance of close surveillance of intra-cranial as well as leptomeningeal spread of pilocytic astrocytoma.\u003c/p\u003e","manuscriptTitle":"Expanding Horizons: A Novel Presentation of Posterior Fossa Pilocytic Astrocytoma With Intracranial and Spinal Leptomeningeal Spread","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-12-30 09:18:44","doi":"10.21203/rs.3.rs-8025242/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":"c2864649-29b0-441f-beb1-d2b5e478add9","owner":[],"postedDate":"December 30th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-01-12T17:39:05+00:00","versionOfRecord":[],"versionCreatedAt":"2025-12-30 09:18:44","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8025242","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8025242","identity":"rs-8025242","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}