Primary Intracranial Plasmablastic Lymphoma with Intradural Extramedullary Metastasis: a Case Report

Primary Intracranial Plasmablastic Lymphoma with Intradural Extramedullary Metastasis: 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 Primary Intracranial Plasmablastic Lymphoma with Intradural Extramedullary Metastasis: a Case Report Zhuoru Jiang, Zhengyang Zhu, Zhennan Tao, Fengnan Niu, Xin Zhang, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6195925/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 29 Sep, 2025 Read the published version in BMC Neurology → Version 1 posted 11 You are reading this latest preprint version Abstract Background Primary central nervous system plasmablastic lymphoma (PCNSPBL) represents an exceptionally rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL), characterized by its distinct immunophenotypic profile and predilection for immunocompromised individuals. Accurate preoperative diagnosis remains challenging due to the nonspecific radiological features observed on conventional magnetic resonance imaging (MRI), necessitating comprehensive histopathological evaluation for definitive diagnosis. While intracranial involvement of PBL has been documented in the literature, spinal cord metastasis of this malignancy has not been previously reported. This study presents a novel case of PCNSPBL with concurrent spinal cord metastasis in an elderly male patient, with a detailed analysis of advanced neuroimaging characteristics that may aid in the diagnostic evaluation of this rare entity. Case Presentation A 52-year-old male patient presented to our institution with left-sided limb weakness. Laboratory investigations revealed positive Epstein-Barr virus-encoded small RNA (EBER). Conventional MRI demonstrated a nodular lesion with a clear anatomical relationship to the inferior horn of the right lateral ventricle. Due to the mass effect, the patient underwent surgical resection of the right temporal lobe lesion, with histopathological examination confirming the diagnosis of plasmablastic lymphoma. Conclusions This report represents the first documented case of PCNSPBL with intradural extramedullary metastasis, underscoring the diagnostic challenges associated with this rare malignancy. Advanced neuroimaging techniques, including functional MRI, serve as valuable complements to conventional MRI in the diagnostic workup. Early and accurate diagnosis of PBL is critical for timely intervention, and further studies are warranted to establish standardized imaging protocols for this rare entity. plasmablastic lymphoma magnetic resonance imaging case report Figures Figure 1 Figure 2 Figure 3 Introduction Plasmablastic lymphoma (PBL), a rare and clinically aggressive variant of diffuse large B-cell lymphoma (DLBCL), poses significant diagnostic challenges due to its heterogeneous clinical presentations and nonspecific pathological features 1 . Although PBL predominantly arises in the oral cavity, emerging evidence has documented its extraoral manifestations across multiple anatomical systems, including the sinonasal tract (maxillary sinus, nasopharynx), gastrointestinal system (stomach, small bowel, anus), respiratory system (lung), integumentary system (skin), soft tissues, and even rare sites such as the heart and spermatic cord 2 – 5 . Of particular clinical significance is primary central nervous system PBL (PCNSPBL), an exceptionally rare entity that accounts for less than 1% of all CNS lymphomas 6 , 7 . The diagnostic paradigm for PCNSPBL requires a multidisciplinary approach, integrating clinical suspicion with histopathological confirmation, while recognizing the limitations of conventional neuroimaging findings that often demonstrate nonspecific characteristics, potentially leading to diagnostic ambiguity or oversight 8 , 9 . Recent advances in neuroimaging modalities, including advanced MRI techniques and metabolic imaging, have provided novel insights into the radiologic features of CNS lymphomas 10 – 12 , offering improved diagnostic accuracy in differentiating PBL from other intracranial pathologies. In this context, we present a comprehensive neuroimaging evaluation of a rare case of intracranial plasmablastic lymphoma with intradural extramedullary metastasis, aiming to contribute to the growing body of literature on the radiologic-pathologic correlation of this rare disease entity. Case presentation A 52-year-old man experienced left limbs weakness for over 1 month. Magnetic Resonance Imaging (MRI) performed at the local hospital suggested abnormal signals in the right side of the brainstem, the right basal ganglia and the temporal lobe. Neurological physical examination revealed that the muscle strength of the left limbs was graded as 4, while the right limbs exhibited grade 5 strength. Conventional MRI, perfusion-weighted MRI, dynamic contrast enhanced MRI (DCE-MRI) (Table 1 ) and MR spectroscopy were obtained. Brain MRI showed obvious enhancement with cystic necrosis and large area of peritumor edema. Diffusion weighted imaging (Fig. 1 ) showed limited diffusion, indicating high cell density of the tumor. MR spectroscopy (Fig. 1 ) revealed a slight increase choline (Cho) peak and a slight decrease in the peaks of creatine (Cr) and N-acetylaspartate (NAA), the peaks of lipid (Lip) and lactate (Lac) are relatively high. The Cho/Cr ratio was 8.27 while Cho/NAA ratio was 2.75. Considering the size and hypervascular space-occupying lesions in the right basal ganglia region, insular and temporal lobes, midbrain and brainstem, accompanied by extensive edema of the surrounding brain tissue, with dissemination in the right cerebellopontine angle region, after excluding contraindications, the patient received surgical treatment. The pathology of the resected tumor indicated plasmablastic lymphoma, but the patient refused to take chemotherapy. Five months after the first surgery (Fig. 2 , Table 1 ), the patient was admitted again for neck discomfort. MRI of the cervical spine demonstrated an intradural extramedullary mass at the C4 vertebral level, measuring approximately 18mm × 10mm × 22mm, with clear enhancement on contrast imaging and a dural tail sign, suggesting spinal cord compression (Fig. 3 ). His cerebrospinal fluid (CSF) analysis includes elevated white blood cell count (67.0 ×10^6/L), lymphocyte ratio (100.0%), and positive CSF globulin. Cytology and special staining for hematologic malignancies revealed lymphocytosis (96%). He underwent the second surgery. Immunophenotyping of the lesions within the spinal canal revealed a population of cells positive for PAX5, Bcl2 (80%++), MUM1 and CD79a; it was negative for Olig-2, GFAP, CK, CD3, CD20, CD10, Bcl6, Kappa and Lambda. Clonal immunoglobulin gene rearrangements (IGH-DH, IGκ-V/in) were detected. The in situ hybridization experiment suggested positive for EBER. The patient underwent four cycles of intravenous chemotherapy with Lenalidomide + CHOP after the second surgery. However, in preparing autologous stem cell transplantation, he underwent seizures, urinary and fecal incontinence, altered mental status, nausea, and vomiting. then he became bedridden. The patient passed away shortly thereafter. Table 1 Cerebral hemodynamic parameters of the ROI of the lesion and normal control. Before the First Surgery Parameter ROI of the legion ROI of normal control Ktrans (10 − 3 /min) 182.7 33.5 Kep (10 − 3 /min) 525.8 2639.7 Ve (10 − 3 ) 350.4 19.1 iAUC (10 − 3 /min · mmol/l) 493 63 After the First Surgery Parameter ROI of the legion ROI of normal control Ktrans (10 − 3 /min) 84.1 27 Kep (10 − 3 /min) 448.5 1634.9 Ve (10 − 3 ) 317 37 iAUC (10 − 3 /min · mmol/l) 23.5 63 Discussion Plasmablastic lymphoma (PBL), a rare and aggressive subtype of diffuse large B-cell lymphoma 13 , has been documented in various anatomical sites, including the brain and epidural space, with spinal cord compression reported in isolated cases 7 , 14 – 16 . While PBL predominantly affects immunocompromised individuals, particularly those with HIV infection, and frequently involves extranodal sites such as the oral cavity, its pathogenesis remains incompletely elucidated 17 – 19 . The disease is characterized by a distinct immunophenotypic profile, marked by the loss of conventional B-cell markers (e.g., CD20) and the expression of plasma cell-associated antigens (e.g., CD138, MUM1) 20 – 22 . This unique immunophenotype, coupled with its aggressive biological behavior, contributes to significant diagnostic and therapeutic challenges. Central nervous system (CNS) involvement by PBL, though rare, presents with a spectrum of nonspecific clinical manifestations, including cognitive impairment, focal neurological deficits, and symptoms of elevated intracranial pressure 15 . Radiologically, cerebral PBL typically manifests as solitary or multifocal enhancing lesions with perilesional edema, often involving periventricular regions and deep gray matter structures. Urrego et al. 23 reported a patient with PCNSPBL, an axial T1-weighted contrast-enhancing MRI showed a solid heterogeneous area of patterned enhancement with scalloped peripheral low signal enhancement, higher signal enhancement along the juxtaposed white matter. However, the absence of pathognomonic imaging features frequently leads to diagnostic ambiguity, necessitating histopathological confirmation. Notably, our case demonstrated cystic necrosis on MRI, a feature that deviates from the more common solid appearance of CNS lymphomas and may mimic high-grade gliomas, further complicating the diagnostic process. Intradural extramedullary metastasis by PBL is exceptionally rare and presents distinct diagnostic challenges. Patients often exhibit myelopathic symptoms, including sensory deficits, motor weakness, and sphincter dysfunction. Imaging findings typically include intramedullary lesions with variable enhancement patterns and associated spinal cord swelling. In the report of Gao et al., 16 a discus-like lesion was located posterior to the spinal cord and showed an isointense signal on T1- and T2-weighted images. After injection of an intravenous contrast agent, the lesion was homogeneously enhanced with a typical mouse tail sign. Ella et al. 24 reported that the PBL extended through the exit foramina with a small enhancing paraspinal component. Differential diagnoses encompass a broad spectrum of intramedullary pathologies, including astrocytoma, ependymoma, and metastatic lesions. The presence of multifocal involvement, rapid progression, and systemic symptoms may raise clinical suspicion for PBL, though definitive diagnosis requires histopathological confirmation. In our case, the intradural extramedullary mass was suspected to represent metastatic involvement, marking the first reported instance of CNS PBL with intradural extramedullary metastasis. Advanced neuroimaging techniques, including diffusion-weighted imaging (DWI), dynamic contrast-enhanced MRI (DCE-MRI), and magnetic resonance spectroscopy (MRS), have significantly enhanced the diagnostic evaluation of CNS PBL. DCE-MRI provides insights into microvascular physiology 25 , while MRS offers metabolic profiling, revealing alterations in key metabolites such as choline (Cho), creatine (Cr), and N-acetylaspartate (NAA) 26 . In our case, MRS demonstrated a mild increase in Cho and decreased Cr and NAA (Cho/Cr = 8.27; Cho/NAA = 2.75), consistent with cellular proliferation and neuronal injury. These findings, combined with whole-brain volumetric analysis and tractography, underscore the utility of advanced imaging in assessing disease extent and treatment response. Conclusion In summary, PCNSPBL represents a rare and diagnostically challenging entity characterized by nonspecific clinical and radiological features. The absence of definitive imaging biomarkers, coupled with limited clinical awareness, often results in diagnostic delays or misdiagnosis. Advanced neuroimaging modalities, including functional MRI and metabolic imaging, have emerged as critical tools in differentiating PCNSPBL from other intracranial and spinal pathologies. Histopathological examination remains the gold standard for diagnosis, highlighting the importance of a multidisciplinary diagnostic approach. This case, the first to report CNS PBL with intradural extramedullary metastasis, contributes to the growing understanding of this rare disease and emphasizes the need for heightened clinical suspicion in immunocompromised patients or those with atypical CNS lesions. Future research should focus on identifying specific imaging biomarkers and developing standardized diagnostic protocols to improve early detection and management of this aggressive malignancy. Declarations Ethics approval and consent to participate Not applicable. Consent for publication Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article. Availability of data and materials The original contributions presented in the study are included in the article/Supplementary material, further inquiries can be directed to the corresponding author. Competing interests The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Funding The author(s) declare no financial support was received for the research, authorship, and/or publication of the article. Authors' contributions ZJ: Writing−original draft. ZZ: Writing−review & editing. ZT: Writing−review and editing. FN: Writing−review and editing. XZ: Writing−review and editing, Funding acquisition, Data curation. BZ: Writing−review and editing, Project administration, Data curation. All authors read and approved the final manuscript. Acknowledgements Not applicable. References Ramirez-Gamero A, Martínez-Cordero H, Beltrán BE, Florindez J, Malpica L, Castillo JJ. Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management. American Journal of Hematology . 2024/08/01 2024;99(8):1586-1594. doi:https://doi.org/10.1002/ajh.27376 Morscio J, Dierickx D, Nijs J, et al. Clinicopathologic comparison of plasmablastic lymphoma in HIV-positive, immunocompetent, and posttransplant patients: single-center series of 25 cases and meta-analysis of 277 reported cases. Am J Surg Pathol . Jul 2014;38(7):875-86. doi:10.1097/pas.0000000000000234 Cao C, Liu T, Lou S, Liu W, Shen K, Xiang B. Unusual presentation of duodenal plasmablastic lymphoma in an immunocompetent patient: A case report and literature review. Oncol Lett . Dec 2014;8(6):2539-2542. doi:10.3892/ol.2014.2604 Eke U, Pratt G, Muc R, Heagerty A. Cutaneous plasmablastic lymphoma in a nonimmunocompromised patient. J Am Acad Dermatol . Dec 2013;69(6):e315-7. doi:10.1016/j.jaad.2013.07.003 Lin Y, Rodrigues GD, Turner JF, Vasef MA. Plasmablastic lymphoma of the lung: report of a unique case and review of the literature. Arch Pathol Lab Med . Feb 2001;125(2):282-5. doi:10.5858/2001-125-0282-plotl Khan UT, Racu-Amoasii I, Arumainathan A, Meswani U, Pettitt AR, Menon G. Central nervous system plasmablastic lymphoma evolving from polyclonal plasmacytosis. Br J Haematol . Nov 2018;183(3):345. doi:10.1111/bjh.15489 Ma H, Wei MH, Qin HM, Wang GY, Hu ZC. Long-Term Survival of Primary Intracranial Plasmablastic Lymphoma: Case Report and Review of the Literature. World Neurosurg . Jan 2017;97:750.e5-750.e10. doi:10.1016/j.wneu.2016.10.098 Lee RS, Lohano S, Safdar M. Plasmablastic myeloma with a history of diffuse large B-cell lymphoma. BMJ Case Rep . Dec 20 2024;17(12)doi:10.1136/bcr-2024-263511 Harandou M, Bensalah M, Seddik R. Plasmablastic Lymphoma Masquerading as Plasma Cell Myeloma: A Case Report. Cureus . Jan 2025;17(1):e77310. doi:10.7759/cureus.77310 Puhakka IK, Sunela KL, Rönkä AL, et al. Defining MRI-based follow-up protocol for primary central nervous system lymphoma. Ann Hematol . Jan 2025;104(1):627-634. doi:10.1007/s00277-024-06145-5 Li EH, Davila C, Zuraski C, Chang J, Goodwill V, Farid N. Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma. Front Radiol . 2024;4:1479282. doi:10.3389/fradi.2024.1479282 Majovsky M, Moravec T, Komarc M, et al. Surgical results in patients with CNS lymphoma. Comparison of predictive value of intraoperative MRI and intraoperative histological examination for diagnostic biopsy yield. Brain Spine . 2024;4:103926. doi:10.1016/j.bas.2024.103926 Bibas M, Castillo JJ. Current knowledge on HIV-associated Plasmablastic Lymphoma. Mediterr J Hematol Infect Dis . 2014;6(1):e2014064. doi:10.4084/mjhid.2014.064 Romero M, González-Fontal GR, Saavedra C, et al. Primary CNS plasmablastic lymphoma in an HIV/EBV negative patient: A case report. Diagn Cytopathol . Jan 2016;44(1):61-5. doi:10.1002/dc.23374 Zhang LY, Lin HY, Gao LX, et al. Primary central nervous system plasmablastic lymphoma presenting in human immunodeficiency virus-negative but Epstein-Barr virus-positive patient: a case report. Diagn Pathol . May 8 2012;7:51. doi:10.1186/1746-1596-7-51 Gao J, Kong X, Zhong D, et al. An uncommon case of epidural plasmablastic lymphoma presents as spinal cord compression. Clin Neurol Neurosurg . Oct 2013;115(10):2301-3. doi:10.1016/j.clineuro.2013.08.006 Corti M, Minué G, Campitelli A, Narbaitz M, Gilardi L. An Aggressive Plasmablastic Lymphoma of the Oral Cavity as Primary Manifestation of Acquired Immunodeficiency Syndrome: Case Report and Literature Review. Int Arch Otorhinolaryngol . Oct 2015;19(4):354-8. doi:10.1055/s-0034-1397335 Carbone A, Gaidano G, Gloghini A, Ferlito A, Rinaldo A, Stein H. AIDS-related plasmablastic lymphomas of the oral cavity and jaws: a diagnostic dilemma. Ann Otol Rhinol Laryngol . Jan 1999;108(1):95-9. doi:10.1177/000348949910800115 Podder S, Khetan P, Sivamurthy S, Mandal K. Rare presentation of orbital plasmablastic lymphoma with oral cavity involvement in an HIV-negative patient. BMJ Case Rep . Sep 16 2015;2015doi:10.1136/bcr-2015-211960 Kane S, Khurana A, Parulkar G, et al. Minimum diagnostic criteria for plasmablastic lymphoma of oral/sinonasal region encountered in a tertiary cancer hospital of a developing country. J Oral Pathol Med . Jan 2009;38(1):138-44. doi:10.1111/j.1600-0714.2008.00673.x Hsi ED, Lorsbach RB, Fend F, Dogan A. Plasmablastic lymphoma and related disorders. Am J Clin Pathol . Aug 2011;136(2):183-94. doi:10.1309/ajcpv1i2qwkzknjh Carbone A, Gaidano G, Gloghini A, et al. Differential expression of BCL-6, CD138/syndecan-1, and Epstein-Barr virus-encoded latent membrane protein-1 identifies distinct histogenetic subsets of acquired immunodeficiency syndrome-related non-Hodgkin's lymphomas. Blood . Feb 1 1998;91(3):747-55. Urrego PA, Smethurst M, Fowkes M, et al. Primary CNS plasmablastic lymphoma: report of a case with CSF cytology, flow cytometry, radiology, histological correlation, and review of the literature. Diagn Cytopathol . Aug 2011;39(8):616-20. doi:10.1002/dc.21520 Harris E, Butler JS, Cassidy N. Aggressive plasmablastic lymphoma of the thoracic spine presenting as acute spinal cord compression in a case of asymptomatic undiagnosed human immunodeficiency virus infection. Spine J . Jul 1 2014;14(7):e1-5. doi:10.1016/j.spinee.2013.12.018 Zimny A, Sasiadek M. Contribution of perfusion-weighted magnetic resonance imaging in the differentiation of meningiomas and other extra-axial tumors: case reports and literature review. J Neurooncol . Jul 2011;103(3):777-83. doi:10.1007/s11060-010-0445-9 Callot V, Galanaud D, Le Fur Y, Confort-Gouny S, Ranjeva JP, Cozzone PJ. (1)H MR spectroscopy of human brain tumours: a practical approach. Eur J Radiol . Aug 2008;67(2):268-274. doi:10.1016/j.ejrad.2008.02.036 Additional Declarations No competing interests reported. Supplementary Files Supplementarymaterial.docx Cite Share Download PDF Status: Published Journal Publication published 29 Sep, 2025 Read the published version in BMC Neurology → Version 1 posted Editorial decision: Revision requested 21 Jul, 2025 Reviews received at journal 20 Jul, 2025 Reviewers agreed at journal 20 Jul, 2025 Reviewers agreed at journal 10 Jun, 2025 Reviews received at journal 01 Apr, 2025 Reviewers agreed at journal 01 Apr, 2025 Reviewers invited by journal 01 Apr, 2025 Editor invited by journal 14 Mar, 2025 Editor assigned by journal 13 Mar, 2025 Submission checks completed at journal 13 Mar, 2025 First submitted to journal 10 Mar, 2025 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. 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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-6195925","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":429347423,"identity":"a72956ac-4ec8-46f2-8ee3-b34982e61269","order_by":0,"name":"Zhuoru Jiang","email":"","orcid":"","institution":"Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University","correspondingAuthor":false,"prefix":"","firstName":"Zhuoru","middleName":"","lastName":"Jiang","suffix":""},{"id":429347425,"identity":"4d86de01-6050-4514-a684-7dd793003d83","order_by":1,"name":"Zhengyang Zhu","email":"","orcid":"","institution":"Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University","correspondingAuthor":false,"prefix":"","firstName":"Zhengyang","middleName":"","lastName":"Zhu","suffix":""},{"id":429347427,"identity":"8559104b-69f5-41f4-9e4f-b6142d4da047","order_by":2,"name":"Zhennan Tao","email":"","orcid":"","institution":"Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University","correspondingAuthor":false,"prefix":"","firstName":"Zhennan","middleName":"","lastName":"Tao","suffix":""},{"id":429347429,"identity":"0474dc28-509e-4324-9417-e12e9133ef74","order_by":3,"name":"Fengnan Niu","email":"","orcid":"","institution":"Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University","correspondingAuthor":false,"prefix":"","firstName":"Fengnan","middleName":"","lastName":"Niu","suffix":""},{"id":429347430,"identity":"e26a917e-1283-4087-99e0-d48bd04079c2","order_by":4,"name":"Xin Zhang","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAsElEQVRIiWNgGAWjYDADfmbmww9I0yLZzpZmQJoWg/M8ChJEqTRn7zH78IGhLnHzYR4GA4Yam2iCWix7zhjPnMHAZmx2mPfAA4ZjabkNBN1zI8eYmYeBR87sMF+CAWPDYSK03H8D0iLBY9zMYyBBnJYbPCAtBnIGzERrOZNWzDiDIcFY4jAwkBOI8svxw5sZQCHW33/48IMPNTaEtYAB4z8oI4Eo5aNgFIyCUTAKCAIALHE07kWeZc4AAAAASUVORK5CYII=","orcid":"","institution":"Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University","correspondingAuthor":true,"prefix":"","firstName":"Xin","middleName":"","lastName":"Zhang","suffix":""},{"id":429347432,"identity":"b486b106-4d71-4a9a-ae5a-6540022e1a85","order_by":5,"name":"Bing Zhang","email":"","orcid":"","institution":"Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University","correspondingAuthor":false,"prefix":"","firstName":"Bing","middleName":"","lastName":"Zhang","suffix":""}],"badges":[],"createdAt":"2025-03-10 13:23:22","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6195925/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6195925/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12883-025-04409-9","type":"published","date":"2025-09-29T15:57:42+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":78733595,"identity":"bd88eeb0-e33e-4c47-b067-7c65989993ca","added_by":"auto","created_at":"2025-03-18 07:54:06","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":221627,"visible":true,"origin":"","legend":"\u003cp\u003eThe initial MRI brain images before the first surgery. (A) Axial T1W sequence, pre-contrast; (B) axial T2W sequence, pre-contrast; (C) axial T2-FLAIR sequence, pre-contrast; (D) gadolinium-enhanced MR T1-weighted image; (E) axial diffusion weighted imaging sequence; (F) apparent diffusion coefficient image; (G) transfer constant image; (H) magnetic resonance spectroscopy image. Cho = 28.53, Lip = 50.36, Cr = 3.45.\u003c/p\u003e","description":"","filename":"floatimage1.png","url":"https://assets-eu.researchsquare.com/files/rs-6195925/v1/b7f68ce83b096525072cb34c.png"},{"id":78733592,"identity":"5f1537f5-462c-4fbc-999f-8791ca2b5f33","added_by":"auto","created_at":"2025-03-18 07:54:05","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":234255,"visible":true,"origin":"","legend":"\u003cp\u003eThe MRI brain images after the first surgery. (A) Axial T1W sequence, pre-contrast; (B) axial T2W sequence, pre-contrast; (C) axial T2-FLAIR sequence, pre-contrast; (D) gadolinium-enhanced MR T1-weighted image; (E) axial diffusion weighted imaging sequence; (F) apparent diffusion coefficient image; (G) transfer constant image; (H) magnetic resonance spectroscopy image. Cho = 9.53, Lip = 6.98, Cr = 4.33.\u003c/p\u003e","description":"","filename":"floatimage2.png","url":"https://assets-eu.researchsquare.com/files/rs-6195925/v1/e18ddd0cedfb3c943d40c2f2.png"},{"id":78733596,"identity":"3bea668a-6649-4dbf-b5be-eef6399e3236","added_by":"auto","created_at":"2025-03-18 07:54:06","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":125031,"visible":true,"origin":"","legend":"\u003cp\u003eThe neck MRI images. (Q) Sagittal T1W sequence, pre-contrast; (R) sagittal T2W sequence, pre-contrast; (S) sagittal T2-FLAIR sequence, pre-contrast; (T) gadolinium-enhanced MR T1-weighted image. Arrows demonstrate the occupying lesions.\u003c/p\u003e","description":"","filename":"floatimage3.png","url":"https://assets-eu.researchsquare.com/files/rs-6195925/v1/800b7f857209457cfca0cffa.png"},{"id":92883935,"identity":"e663ea1f-7376-4043-893f-7bb001840e24","added_by":"auto","created_at":"2025-10-06 16:11:16","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1095795,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6195925/v1/53e4e843-d2c8-433b-a019-559da459fb4f.pdf"},{"id":78734615,"identity":"5aded253-f944-4e0d-8eb0-9a4af39c487c","added_by":"auto","created_at":"2025-03-18 08:02:07","extension":"docx","order_by":2,"title":"","display":"","copyAsset":false,"role":"supplement","size":15756,"visible":true,"origin":"","legend":"","description":"","filename":"Supplementarymaterial.docx","url":"https://assets-eu.researchsquare.com/files/rs-6195925/v1/1d276001b3b29b374192fc06.docx"}],"financialInterests":"No competing interests reported.","formattedTitle":"Primary Intracranial Plasmablastic Lymphoma with Intradural Extramedullary Metastasis: a Case Report","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePlasmablastic lymphoma (PBL), a rare and clinically aggressive variant of diffuse large B-cell lymphoma (DLBCL), poses significant diagnostic challenges due to its heterogeneous clinical presentations and nonspecific pathological features\u003csup\u003e\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e\u003c/sup\u003e. Although PBL predominantly arises in the oral cavity, emerging evidence has documented its extraoral manifestations across multiple anatomical systems, including the sinonasal tract (maxillary sinus, nasopharynx), gastrointestinal system (stomach, small bowel, anus), respiratory system (lung), integumentary system (skin), soft tissues, and even rare sites such as the heart and spermatic cord\u003csup\u003e\u003cspan additionalcitationids=\"CR3 CR4\" citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e\u003c/sup\u003e. Of particular clinical significance is primary central nervous system PBL (PCNSPBL), an exceptionally rare entity that accounts for less than 1% of all CNS lymphomas\u003csup\u003e\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e,\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e\u003c/sup\u003e. The diagnostic paradigm for PCNSPBL requires a multidisciplinary approach, integrating clinical suspicion with histopathological confirmation, while recognizing the limitations of conventional neuroimaging findings that often demonstrate nonspecific characteristics, potentially leading to diagnostic ambiguity or oversight\u003csup\u003e\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e,\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e\u003c/sup\u003e. Recent advances in neuroimaging modalities, including advanced MRI techniques and metabolic imaging, have provided novel insights into the radiologic features of CNS lymphomas\u003csup\u003e\u003cspan additionalcitationids=\"CR11\" citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e\u003c/sup\u003e, offering improved diagnostic accuracy in differentiating PBL from other intracranial pathologies. In this context, we present a comprehensive neuroimaging evaluation of a rare case of intracranial plasmablastic lymphoma with intradural extramedullary metastasis, aiming to contribute to the growing body of literature on the radiologic-pathologic correlation of this rare disease entity.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 52-year-old man experienced left limbs weakness for over 1 month. Magnetic Resonance Imaging (MRI) performed at the local hospital suggested abnormal signals in the right side of the brainstem, the right basal ganglia and the temporal lobe. Neurological physical examination revealed that the muscle strength of the left limbs was graded as 4, while the right limbs exhibited grade 5 strength.\u003c/p\u003e \u003cp\u003eConventional MRI, perfusion-weighted MRI, dynamic contrast enhanced MRI (DCE-MRI) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) and MR spectroscopy were obtained. Brain MRI showed obvious enhancement with cystic necrosis and large area of peritumor edema. Diffusion weighted imaging (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) showed limited diffusion, indicating high cell density of the tumor. MR spectroscopy (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e) revealed a slight increase choline (Cho) peak and a slight decrease in the peaks of creatine (Cr) and N-acetylaspartate (NAA), the peaks of lipid (Lip) and lactate (Lac) are relatively high. The Cho/Cr ratio was 8.27 while Cho/NAA ratio was 2.75.\u003c/p\u003e \u003cp\u003eConsidering the size and hypervascular space-occupying lesions in the right basal ganglia region, insular and temporal lobes, midbrain and brainstem, accompanied by extensive edema of the surrounding brain tissue, with dissemination in the right cerebellopontine angle region, after excluding contraindications, the patient received surgical treatment. The pathology of the resected tumor indicated plasmablastic lymphoma, but the patient refused to take chemotherapy.\u003c/p\u003e \u003cp\u003eFive months after the first surgery (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e, Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e), the patient was admitted again for neck discomfort. MRI of the cervical spine demonstrated an intradural extramedullary mass at the C4 vertebral level, measuring approximately 18mm \u0026times; 10mm \u0026times; 22mm, with clear enhancement on contrast imaging and a dural tail sign, suggesting spinal cord compression (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). His cerebrospinal fluid (CSF) analysis includes elevated white blood cell count (67.0 \u0026times;10^6/L), lymphocyte ratio (100.0%), and positive CSF globulin. Cytology and special staining for hematologic malignancies revealed lymphocytosis (96%). He underwent the second surgery. Immunophenotyping of the lesions within the spinal canal revealed a population of cells positive for PAX5, Bcl2 (80%++), MUM1 and CD79a; it was negative for Olig-2, GFAP, CK, CD3, CD20, CD10, Bcl6, Kappa and Lambda. Clonal immunoglobulin gene rearrangements (IGH-DH, IGκ-V/in) were detected. The in situ hybridization experiment suggested positive for EBER.\u003c/p\u003e \u003cp\u003eThe patient underwent four cycles of intravenous chemotherapy with Lenalidomide\u0026thinsp;+\u0026thinsp;CHOP after the second surgery. However, in preparing autologous stem cell transplantation, he underwent seizures, urinary and fecal incontinence, altered mental status, nausea, and vomiting. then he became bedridden. The patient passed away shortly thereafter.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eCerebral hemodynamic parameters of the ROI of the lesion and normal control.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"3\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colspan=\"3\" nameend=\"c3\" namest=\"c1\"\u003e \u003cp\u003eBefore the First Surgery\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eParameter\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eROI of the legion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eROI of normal control\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKtrans (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e/min)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e182.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e33.5\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKep (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e/min)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e525.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e2639.7\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eVe (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e350.4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e19.1\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eiAUC (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e/min \u0026middot; mmol/l)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e493\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e63\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colspan=\"3\" nameend=\"c3\" namest=\"c1\"\u003e \u003cp\u003eAfter the First Surgery\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eParameter\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003eROI of the legion\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eROI of normal control\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKtrans (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e/min)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e84.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eKep (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e/min)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e448.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e1634.9\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eVe (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e317\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e37\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eiAUC (10\u003csup\u003e\u0026minus;\u0026thinsp;3\u003c/sup\u003e/min \u0026middot; mmol/l)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e23.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e63\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003ePlasmablastic lymphoma (PBL), a rare and aggressive subtype of diffuse large B-cell lymphoma\u003csup\u003e\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e\u003c/sup\u003e, has been documented in various anatomical sites, including the brain and epidural space, with spinal cord compression reported in isolated cases\u003csup\u003e\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e,\u003cspan additionalcitationids=\"CR15\" citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e\u003c/sup\u003e. While PBL predominantly affects immunocompromised individuals, particularly those with HIV infection, and frequently involves extranodal sites such as the oral cavity, its pathogenesis remains incompletely elucidated\u003csup\u003e\u003cspan additionalcitationids=\"CR18\" citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e\u003c/sup\u003e. The disease is characterized by a distinct immunophenotypic profile, marked by the loss of conventional B-cell markers (e.g., CD20) and the expression of plasma cell-associated antigens (e.g., CD138, MUM1)\u003csup\u003e\u003cspan additionalcitationids=\"CR21\" citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e\u003c/sup\u003e. This unique immunophenotype, coupled with its aggressive biological behavior, contributes to significant diagnostic and therapeutic challenges.\u003c/p\u003e \u003cp\u003eCentral nervous system (CNS) involvement by PBL, though rare, presents with a spectrum of nonspecific clinical manifestations, including cognitive impairment, focal neurological deficits, and symptoms of elevated intracranial pressure\u003csup\u003e\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u003c/sup\u003e. Radiologically, cerebral PBL typically manifests as solitary or multifocal enhancing lesions with perilesional edema, often involving periventricular regions and deep gray matter structures. Urrego et al.\u003csup\u003e\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e\u003c/sup\u003e reported a patient with PCNSPBL, an axial T1-weighted contrast-enhancing MRI showed a solid heterogeneous area of patterned enhancement with scalloped peripheral low signal enhancement, higher signal enhancement along the juxtaposed white matter. However, the absence of pathognomonic imaging features frequently leads to diagnostic ambiguity, necessitating histopathological confirmation. Notably, our case demonstrated cystic necrosis on MRI, a feature that deviates from the more common solid appearance of CNS lymphomas and may mimic high-grade gliomas, further complicating the diagnostic process.\u003c/p\u003e \u003cp\u003eIntradural extramedullary metastasis by PBL is exceptionally rare and presents distinct diagnostic challenges. Patients often exhibit myelopathic symptoms, including sensory deficits, motor weakness, and sphincter dysfunction. Imaging findings typically include intramedullary lesions with variable enhancement patterns and associated spinal cord swelling. In the report of Gao et al.,\u003csup\u003e\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e\u003c/sup\u003e a discus-like lesion was located posterior to the spinal cord and showed an isointense signal on T1- and T2-weighted images. After injection of an intravenous contrast agent, the lesion was homogeneously enhanced with a typical mouse tail sign. Ella et al.\u003csup\u003e\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e\u003c/sup\u003e reported that the PBL extended through the exit foramina with a small enhancing paraspinal component. Differential diagnoses encompass a broad spectrum of intramedullary pathologies, including astrocytoma, ependymoma, and metastatic lesions. The presence of multifocal involvement, rapid progression, and systemic symptoms may raise clinical suspicion for PBL, though definitive diagnosis requires histopathological confirmation. In our case, the intradural extramedullary mass was suspected to represent metastatic involvement, marking the first reported instance of CNS PBL with intradural extramedullary metastasis.\u003c/p\u003e \u003cp\u003eAdvanced neuroimaging techniques, including diffusion-weighted imaging (DWI), dynamic contrast-enhanced MRI (DCE-MRI), and magnetic resonance spectroscopy (MRS), have significantly enhanced the diagnostic evaluation of CNS PBL. DCE-MRI provides insights into microvascular physiology\u003csup\u003e\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e\u003c/sup\u003e, while MRS offers metabolic profiling, revealing alterations in key metabolites such as choline (Cho), creatine (Cr), and N-acetylaspartate (NAA)\u003csup\u003e\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e\u003c/sup\u003e. In our case, MRS demonstrated a mild increase in Cho and decreased Cr and NAA (Cho/Cr\u0026thinsp;=\u0026thinsp;8.27; Cho/NAA\u0026thinsp;=\u0026thinsp;2.75), consistent with cellular proliferation and neuronal injury. These findings, combined with whole-brain volumetric analysis and tractography, underscore the utility of advanced imaging in assessing disease extent and treatment response.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eIn summary, PCNSPBL represents a rare and diagnostically challenging entity characterized by nonspecific clinical and radiological features. The absence of definitive imaging biomarkers, coupled with limited clinical awareness, often results in diagnostic delays or misdiagnosis. Advanced neuroimaging modalities, including functional MRI and metabolic imaging, have emerged as critical tools in differentiating PCNSPBL from other intracranial and spinal pathologies. Histopathological examination remains the gold standard for diagnosis, highlighting the importance of a multidisciplinary diagnostic approach. This case, the first to report CNS PBL with intradural extramedullary metastasis, contributes to the growing understanding of this rare disease and emphasizes the need for heightened clinical suspicion in immunocompromised patients or those with atypical CNS lesions. Future research should focus on identifying specific imaging biomarkers and developing standardized diagnostic protocols to improve early detection and management of this aggressive malignancy.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe original contributions presented in the study are included in the article/Supplementary material, further inquiries can be directed to the corresponding author.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe author(s) declare no financial support was received for the research, authorship, and/or publication of the article.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors' contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eZJ: Writing−original draft. ZZ: Writing−review \u0026amp; editing. ZT: Writing−review and editing. FN: Writing−review and editing. XZ: Writing−review and editing, Funding acquisition, Data curation. BZ: Writing−review and editing, Project administration, Data curation. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eRamirez-Gamero A, Mart\u0026iacute;nez-Cordero H, Beltr\u0026aacute;n BE, Florindez J, Malpica L, Castillo JJ. Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management. \u003cem\u003eAmerican Journal of Hematology\u003c/em\u003e. 2024/08/01 2024;99(8):1586-1594. doi:https://doi.org/10.1002/ajh.27376\u003c/li\u003e\n\u003cli\u003eMorscio J, Dierickx D, Nijs J, et al. 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Dec 20 2024;17(12)doi:10.1136/bcr-2024-263511\u003c/li\u003e\n\u003cli\u003eHarandou M, Bensalah M, Seddik R. Plasmablastic Lymphoma Masquerading as Plasma Cell Myeloma: A Case Report. \u003cem\u003eCureus\u003c/em\u003e. Jan 2025;17(1):e77310. doi:10.7759/cureus.77310\u003c/li\u003e\n\u003cli\u003ePuhakka IK, Sunela KL, R\u0026ouml;nk\u0026auml; AL, et al. Defining MRI-based follow-up protocol for primary central nervous system lymphoma. \u003cem\u003eAnn Hematol\u003c/em\u003e. Jan 2025;104(1):627-634. doi:10.1007/s00277-024-06145-5\u003c/li\u003e\n\u003cli\u003eLi EH, Davila C, Zuraski C, Chang J, Goodwill V, Farid N. Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma. \u003cem\u003eFront Radiol\u003c/em\u003e. 2024;4:1479282. doi:10.3389/fradi.2024.1479282\u003c/li\u003e\n\u003cli\u003eMajovsky M, Moravec T, Komarc M, et al. Surgical results in patients with CNS lymphoma. Comparison of predictive value of intraoperative MRI and intraoperative histological examination for diagnostic biopsy yield. \u003cem\u003eBrain Spine\u003c/em\u003e. 2024;4:103926. doi:10.1016/j.bas.2024.103926\u003c/li\u003e\n\u003cli\u003eBibas M, Castillo JJ. Current knowledge on HIV-associated Plasmablastic Lymphoma. \u003cem\u003eMediterr J Hematol Infect Dis\u003c/em\u003e. 2014;6(1):e2014064. doi:10.4084/mjhid.2014.064\u003c/li\u003e\n\u003cli\u003eRomero M, Gonz\u0026aacute;lez-Fontal GR, Saavedra C, et al. Primary CNS plasmablastic lymphoma in an HIV/EBV negative patient: A case report. \u003cem\u003eDiagn Cytopathol\u003c/em\u003e. Jan 2016;44(1):61-5. doi:10.1002/dc.23374\u003c/li\u003e\n\u003cli\u003eZhang LY, Lin HY, Gao LX, et al. Primary central nervous system plasmablastic lymphoma presenting in human immunodeficiency virus-negative but Epstein-Barr virus-positive patient: a case report. \u003cem\u003eDiagn Pathol\u003c/em\u003e. May 8 2012;7:51. doi:10.1186/1746-1596-7-51\u003c/li\u003e\n\u003cli\u003eGao J, Kong X, Zhong D, et al. An uncommon case of epidural plasmablastic lymphoma presents as spinal cord compression. \u003cem\u003eClin Neurol Neurosurg\u003c/em\u003e. Oct 2013;115(10):2301-3. doi:10.1016/j.clineuro.2013.08.006\u003c/li\u003e\n\u003cli\u003eCorti M, Minu\u0026eacute; G, Campitelli A, Narbaitz M, Gilardi L. An Aggressive Plasmablastic Lymphoma of the Oral Cavity as Primary Manifestation of Acquired Immunodeficiency Syndrome: Case Report and Literature Review. \u003cem\u003eInt Arch Otorhinolaryngol\u003c/em\u003e. Oct 2015;19(4):354-8. doi:10.1055/s-0034-1397335\u003c/li\u003e\n\u003cli\u003eCarbone A, Gaidano G, Gloghini A, Ferlito A, Rinaldo A, Stein H. AIDS-related plasmablastic lymphomas of the oral cavity and jaws: a diagnostic dilemma. \u003cem\u003eAnn Otol Rhinol Laryngol\u003c/em\u003e. Jan 1999;108(1):95-9. doi:10.1177/000348949910800115\u003c/li\u003e\n\u003cli\u003ePodder S, Khetan P, Sivamurthy S, Mandal K. Rare presentation of orbital plasmablastic lymphoma with oral cavity involvement in an HIV-negative patient. \u003cem\u003eBMJ Case Rep\u003c/em\u003e. Sep 16 2015;2015doi:10.1136/bcr-2015-211960\u003c/li\u003e\n\u003cli\u003eKane S, Khurana A, Parulkar G, et al. Minimum diagnostic criteria for plasmablastic lymphoma of oral/sinonasal region encountered in a tertiary cancer hospital of a developing country. \u003cem\u003eJ Oral Pathol Med\u003c/em\u003e. Jan 2009;38(1):138-44. doi:10.1111/j.1600-0714.2008.00673.x\u003c/li\u003e\n\u003cli\u003eHsi ED, Lorsbach RB, Fend F, Dogan A. Plasmablastic lymphoma and related disorders. \u003cem\u003eAm J Clin Pathol\u003c/em\u003e. Aug 2011;136(2):183-94. doi:10.1309/ajcpv1i2qwkzknjh\u003c/li\u003e\n\u003cli\u003eCarbone A, Gaidano G, Gloghini A, et al. Differential expression of BCL-6, CD138/syndecan-1, and Epstein-Barr virus-encoded latent membrane protein-1 identifies distinct histogenetic subsets of acquired immunodeficiency syndrome-related non-Hodgkin\u0026apos;s lymphomas. \u003cem\u003eBlood\u003c/em\u003e. Feb 1 1998;91(3):747-55.\u003c/li\u003e\n\u003cli\u003eUrrego PA, Smethurst M, Fowkes M, et al. Primary CNS plasmablastic lymphoma: report of a case with CSF cytology, flow cytometry, radiology, histological correlation, and review of the literature. \u003cem\u003eDiagn Cytopathol\u003c/em\u003e. Aug 2011;39(8):616-20. doi:10.1002/dc.21520\u003c/li\u003e\n\u003cli\u003eHarris E, Butler JS, Cassidy N. Aggressive plasmablastic lymphoma of the thoracic spine presenting as acute spinal cord compression in a case of asymptomatic undiagnosed human immunodeficiency virus infection. \u003cem\u003eSpine J\u003c/em\u003e. Jul 1 2014;14(7):e1-5. doi:10.1016/j.spinee.2013.12.018\u003c/li\u003e\n\u003cli\u003eZimny A, Sasiadek M. Contribution of perfusion-weighted magnetic resonance imaging in the differentiation of meningiomas and other extra-axial tumors: case reports and literature review. \u003cem\u003eJ Neurooncol\u003c/em\u003e. Jul 2011;103(3):777-83. doi:10.1007/s11060-010-0445-9\u003c/li\u003e\n\u003cli\u003eCallot V, Galanaud D, Le Fur Y, Confort-Gouny S, Ranjeva JP, Cozzone PJ. (1)H MR spectroscopy of human brain tumours: a practical approach. \u003cem\u003eEur J Radiol\u003c/em\u003e. Aug 2008;67(2):268-274. doi:10.1016/j.ejrad.2008.02.036\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"plasmablastic lymphoma, magnetic resonance imaging, case report","lastPublishedDoi":"10.21203/rs.3.rs-6195925/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6195925/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\u003ePrimary central nervous system plasmablastic lymphoma (PCNSPBL) represents an exceptionally rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL), characterized by its distinct immunophenotypic profile and predilection for immunocompromised individuals. Accurate preoperative diagnosis remains challenging due to the nonspecific radiological features observed on conventional magnetic resonance imaging (MRI), necessitating comprehensive histopathological evaluation for definitive diagnosis. While intracranial involvement of PBL has been documented in the literature, spinal cord metastasis of this malignancy has not been previously reported. This study presents a novel case of PCNSPBL with concurrent spinal cord metastasis in an elderly male patient, with a detailed analysis of advanced neuroimaging characteristics that may aid in the diagnostic evaluation of this rare entity.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase Presentation\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA 52-year-old male patient presented to our institution with left-sided limb weakness. Laboratory investigations revealed positive Epstein-Barr virus-encoded small RNA (EBER). Conventional MRI demonstrated a nodular lesion with a clear anatomical relationship to the inferior horn of the right lateral ventricle. Due to the mass effect, the patient underwent surgical resection of the right temporal lobe lesion, with histopathological examination confirming the diagnosis of plasmablastic lymphoma.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis report represents the first documented case of PCNSPBL with intradural extramedullary metastasis, underscoring the diagnostic challenges associated with this rare malignancy. Advanced neuroimaging techniques, including functional MRI, serve as valuable complements to conventional MRI in the diagnostic workup. Early and accurate diagnosis of PBL is critical for timely intervention, and further studies are warranted to establish standardized imaging protocols for this rare entity.\u003c/p\u003e","manuscriptTitle":"Primary Intracranial Plasmablastic Lymphoma with Intradural Extramedullary Metastasis: a Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-03-18 07:53:26","doi":"10.21203/rs.3.rs-6195925/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-07-21T21:34:33+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-07-20T11:31:11+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"87913790414561344140851357296312265745","date":"2025-07-20T10:58:05+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"213664191291668887708708909259659143138","date":"2025-06-10T20:02:49+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-04-02T02:52:25+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"238061118376869398106209918557640368681","date":"2025-04-01T15:42:43+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-04-01T09:09:49+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2025-03-14T05:47:20+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-03-13T06:45:23+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-03-13T06:41:10+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Neurology","date":"2025-03-10T13:13:15+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"a6d9785f-62cc-46cf-8a2e-05cb0612ac40","owner":[],"postedDate":"March 18th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-10-06T16:06:25+00:00","versionOfRecord":{"articleIdentity":"rs-6195925","link":"https://doi.org/10.1186/s12883-025-04409-9","journal":{"identity":"bmc-neurology","isVorOnly":false,"title":"BMC Neurology"},"publishedOn":"2025-09-29 15:57:42","publishedOnDateReadable":"September 29th, 2025"},"versionCreatedAt":"2025-03-18 07:53:26","video":"","vorDoi":"10.1186/s12883-025-04409-9","vorDoiUrl":"https://doi.org/10.1186/s12883-025-04409-9","workflowStages":[]},"version":"v1","identity":"rs-6195925","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6195925","identity":"rs-6195925","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}