CC BY-NC-ND 4.0 · Asian J Neurosurg 2023; 18(02): 231-245
DOI: 10.1055/s-0043-1768574
Review Article

B-Cell Lymphoma Intramedullary Tumor: Case Report and Systematic Review

1   Graduation Medicine at Faculty of Medicine of Catanduva, Catanduva, São Paulo, Brazil
Paulo Eduardo Albuquerque Zito Raffa
1   Graduation Medicine at Faculty of Medicine of Catanduva, Catanduva, São Paulo, Brazil
Gabriela Gerenutti de Sousa
2   Graduation Medicine at Pontifical Catholic University of São Paulo, Sorocaba, Brazil
Melissa Esposito Gomes Rigueiral
3   Graduation Medicine at Faculty of Medicine of ABC, Santo André, São Paulo, Brazil
Iracema Araújo Estevão
4   Department of Neurosurgery, Santa Paula Hospital, São Paulo, Brazil
Cesar Cozar Pacheco
4   Department of Neurosurgery, Santa Paula Hospital, São Paulo, Brazil
Roger Thomaz Rotta Medeiros
4   Department of Neurosurgery, Santa Paula Hospital, São Paulo, Brazil
5   Department of Neurosurgery, University of Caxias do Sul, Rio Grande do Sul, Brazil
Paulo Henrique Pires de Aguiar
4   Department of Neurosurgery, Santa Paula Hospital, São Paulo, Brazil
6   Department of Research and Innovation, Laboratory of Cellular and Molecular Biology, Faculty of Medicine of ABC, Santo André, São Paulo, Brazil
7   Department of Neurology, School of Medicine of Pontifical Catholic University of São Paulo, Sorocaba, São Paulo, Brazil
› Author Affiliations
Funding None.


Intramedullary tumors represent the major cause of spinal cord injuries, and its symptoms include pain and weakness. Progressive weakness may concomitantly occur in the upper and lower limbs, along with lack of balance, spine tenderness, sensory loss, trophic changes of extremity, hyperreflexia, and clonus. The study protocol was in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. A systematic search of the MEDLINE electronic database was performed to identify the studies reporting the clinical features of children and adults who presented with an intramedullary lymphoma. Twenty-one studies were included, reporting 25 cases. Manuscripts were excluded if the full-text article was not available, original data were not reported (e.g., review articles), or if the main disease was not intramedullary lymphoma. A structured data extraction form was employed to standardize the identification and retrieval of data from manuscripts. To enlighten the discussion, a case is also presented. An 82-year-old woman with Fitzpatrick skin type II, diagnosed and treated for non-Hodgkin's lymphoma 7 years ago, was admitted with mental confusion and memory loss for the past 2 months—evolving with recurring falls from her own height. One day before admission, she displayed Brown-Séquard syndrome. An expansive lesion from C2 to C4 in the cervical spinal cord was found and a hypersignal spinal cord adjacent was described at the bulb medullary transition to the C6–C7 level. A primary spinal cord tumor was considered, as well as a melanoma metastasis, due to the lesion's flame pattern. The patient presented a partial recovery of symptoms and a reduction of the spinal cord edema after being empirically treated with corticosteroids, but the lesion maintained its extent. Subsequently, a large diffuse B-cell lymphoma with nongerminal center was found in open body biopsy, infiltrating neural tissue. The main objective of the present study is to report a surgical case treated for a large diffuse B-cell lymphoma, in addition to presenting the results of a systematic review of primary intramedullary spinal cord lymphoma.

Publication History

Article published online:
06 June 2023

© 2023. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

  • References

  • 1 Das JM, Hoang S, Mesfin FB. Intramedullary spinal cord tumors. StatPearls; 2022. Accessed August 3, 2022 at:
  • 2 Yang W, Garzon-Muvdi T, Braileanu M. et al. Primary intramedullary spinal cord lymphoma: a population-based study. Neuro-oncol 2017; 19 (03) 414-421
  • 3 Lv C, Wang J, Zhou M, Xu JY, Chen B, Wan Y. Primary central nervous system lymphoma in the United States, 1975-2017. Ther Adv Hematol 2022; 13: 2040620 7211066166
  • 4 Löw S, Han CH, Batchelor TT. Primary central nervous system lymphoma. Ther Adv Neurol Disord 2018; 11: 1756286 418793562
  • 5 Shams S, Arain A. Brown Sequard Syndrome. StatPearls; 2022. Accessed August 3, 2022 at:
  • 6 Aycan A, Celik S, Kuyumcu F. et al. Spinal metastasis of unknown primary accompanied by neurologic deficit or vertebral instability. World Neurosurg 2018; 109: e33-e42
  • 7 Chen W, Hika B, Smith CJ, Parrett TJ, Mesfin FB. A conservative approach to the treatment of a rare case of cervical spine double expressor diffuse large B-cell lymphoma: a case report. Cureus 2022; 14 (01) e21208
  • 8 Iriyama C, Murate K, Iba S. et al. Detection of circulating tumor DNA in cerebrospinal fluid prior to diagnosis of spinal cord lymphoma by flow cytometric and cytologic analyses. Ann Hematol 2022; 101 (05) 1157-1159
  • 9 Erdem MB, Kale A, Yaman ME, Emmez H. A rare entity in the lumbar epidural region: T-cell lymphoblastic lymphoma. Int J Spine Surg 2021; 14 (s4): S52-S56
  • 10 De Vries J, Oterdoom MD, Den Dunnen WF. et al. Primary cauda equina T-cell lymphoblastic lymphoma. World Neurosurg 2020; 142: 227-232
  • 11 Natteru PA, Shekhar S, Nair LR, Uschmann H. Primary central nervous system lymphoma mimicking longitudinally extensive transverse myelitis. Neurohospitalist 2021; 11 (02) 170-174
  • 12 Wang D, Su M, Xiao J. A rare case of primary ventricular lymphoma presented on FDG PET/CT. Clin Nucl Med 2020; 45 (02) 156-158
  • 13 Singh SS, Mittal BR, Kumar R, Singh H, Balaini N, Goyal M. Primary central nervous system lymphoma with diffuse neurolymphomatosis involving multiple cranial and spinal nerve roots. Clin Nucl Med 2020; 45 (06) e285-e287
  • 14 Fakhouri F, Shoumal N, Obeid B, Alkhoder A. Primary diffuse large B-cell non-Hodgkin's lymphoma of the thoracic spine presented initially as an epigastric pain. Asian J Neurosurg 2020; 15 (01) 162-164
  • 15 Pandey S, Gokden M, Kazemi NJ, Post GR. Hematolymphoid malignancies presenting with spinal epidural mass and spinal cord compression: a case series with rare entities. Ann Clin Lab Sci 2019; 49 (06) 818-828
  • 16 Beume LA, Wolf K, Urbach H. et al. Primary intraspinal non-Hodgkin's lymphoma: case report and review of literature. J Clin Neurosci 2019; 61: 262-264
  • 17 Yim J, Song SG, Kim S. et al. Primary peripheral gamma delta T-cell lymphoma of the central nervous system: Report of a case involving the intramedullary spinal cord and presenting with myelopathy. J Pathol Transl Med 2019; 53 (01) 57-61
  • 18 Feng L, Chen D, Zhou H. et al. Spinal primary central nervous system lymphoma: case report and literature review. J Clin Neurosci 2018; 50: 16-19
  • 19 Arslan H, Yavuz A, Aycan A. Primary spinal lymphoma masquerading as meningioma: preoperative and postoperative magnetic resonance imaging findings. World Neurosurg 2018; 118: 86-87
  • 20 Li X, Qi S, Jiao Y, Gao J, Du H. A case report of primary central nervous system lymphoma with intestinal obstruction as the initial symptom. Medicine (Baltimore) 2018; 97 (10) e0080
  • 21 Suzuki K, Yasuda T, Hiraiwa T, Kanamori M, Kimura T, Kawaguchi Y. Primary cauda equina lymphoma diagnosed by nerve biopsy: a case report and literature review. Oncol Lett 2018; 16 (01) 623-631
  • 22 Patel M, Wu OC, Kasliwal MK. Wrap-around appearance: underrecognized radiologic feature of spinal lymphoma. World Neurosurg 2018; 115: 157-158
  • 23 Fastré S, London F, Lelotte J, Camboni A, Jeanjean A. Primary central nervous system lymphoma of T-cell origin: an unusual cause of spinal cord disease. Acta Neurol Belg 2017; 117 (03) 765-767
  • 24 Geevarghese R, Marcus R, Aizpurua M, Al-Sarraj S, Ashkan K. Non-Hodgkin lymphoma of the cauda equina: a rare entity. Br J Neurosurg 2017; 31 (06) 734-735
  • 25 Alaya Z, Achour B. Primary spinal marginal zone lymphoma: an unusual cause of spinal cord compression. Pan Afr Med J 2017; 27: 171
  • 26 Chida K, Sugawara A, Koji T. et al. Primary intramedullary malignant lymphoma in the cervical cord with a presyrinx state. Cureus 2017; 9 (12) e2006
  • 27 Córdoba-Mosqueda ME, Guerra-Mora JR, Sánchez-Silva MC, Vicuña-González RM, Torre AI. Primary spinal epidural lymphoma as a cause of spontaneous spinal anterior syndrome: a case report and literature review. J Neurol Surg Rep 2017; 78 (01) e1-e4
  • 28 Samartzis D, Gillis CC, Shih P, O'Toole JE, Fessler RG. Intramedullary spinal cord tumors: part i-epidemiology, pathophysiology, and diagnosis. Global Spine J 2015; 5 (05) 425-435
  • 29 Ogden AT, Francavilla TL. Intramedullary Spinal Cord Tumors. Medscape; 2020. Accessed August 3, 2022 at:
  • 30 Tuz Zahra F, Ajmal Z, Qian J, Wrzesinski S. Primary intramedullary spinal melanoma: a rare disease of the spinal cord. Cureus 2021; 13 (07) e1619
  • 31 Raffa PEAZ, Vencio RCC, Ponce ACC. et al. Spinal intramedullary abscess due to Candida albicans in an immunocompetent patient: a rare case report. Surg Neurol Int 2021; 12: 275
  • 32 Bowzyk Al-Naeeb A, Ajithkumar T, Behan S, Hodson DJ. Non-Hodgkin lymphoma. BMJ 2018; 362: k3204
  • 33 Urasaki E, Yamada H, Tokimura T, Yokota A. T-cell type primary spinal intramedullary lymphoma associated with human T-cell lymphotropic virus type I after a renal transplant: case report. Neurosurgery 1996; 38 (05) 1036-1039
  • 34 Mullangi S, Lekkala MR. CNS Lymphoma. StatPearls; 2021. Accessed August 3, 2022 at:
  • 35 Dähnert W. Radiology Review Manual. Nucl Med Commun 2011; 32 (10) 195-196
  • 36 Flanagan EP, O'Neill BP, Porter AB, Lanzino G, Haberman TM, Keegan BM. Primary intramedullary spinal cord lymphoma. Neurology 2011; 77 (08) 784-791
  • 37 Hochberg FH, Miller DC. Primary central nervous system lymphoma. J Neurosurg 1988; 68 (06) 835-853
  • 38 Kitley JL, Leite MI, George JS, Palace JA. The differential diagnosis of longitudinally extensive transverse myelitis. Mult Scler 2012; 18 (03) 271-285
  • 39 Cobo-Calvo Á, Alentorn A, Mañé Martínez MA. et al. Etiologic spectrum and prognosis of longitudinally extensive transverse myelopathies. Eur Neurol 2014; 72 (1-2): 86-94
  • 40 Elavarasi A, Dash D, Warrier AR. et al. Spinal cord involvement in primary CNS lymphoma. J Clin Neurosci 2018; 47: 145-148
  • 41 Fitzsimmons A, Upchurch K, Batchelor T. Clinical features and diagnosis of primary central nervous system lymphoma. Hematol Oncol Clin North Am 2005; 19 (04) 689-703 , vii
  • 42 Haque S, Law M, Abrey LE, Young RJ. Imaging of lymphoma of the central nervous system, spine, and orbit. Radiol Clin North Am 2008; 46 (02) 339-361 , ix
  • 43 Bekar A, Cordan T, Evrensel T, Tolunay S. A case of primary spinal intramedullary lymphoma. Surg Neurol 2001; 55 (05) 261-264
  • 44 Schwarz S, Zoubaa S, Knauth M, Sommer C, Storch-Hagenlocher B. Intravascular lymphomatosis presenting with a conus medullaris syndrome mimicking disseminated encephalomyelitis. Neuro-oncol 2002; 4 (03) 187-191
  • 45 Iqbal S, Wein S. Lymphoma of the spinal cord. Radiopaedia.Org.; 2012. Accessed March 17, 2023 at: