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Primary T-cell CNS lymphoma presenting with leptomeningeal spread and neurolymphomatosis

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Abstract

Primary CNS lymphoma (PCNSL), a rare form of non-Hodgkin lymphoma that is confined to the brain, is usually of B-cell origin. Primary leptomeningeal lymphoma, regardless of T or B-cell origin, is an unusual site of presentation. Out of 100 consecutive PCNSL patients that we have followed up in our center during the last 10 years, five had T-cell lymphoma (5%). All presented with leptomeningeal involvement as the sole manifestation and four of them presented with neuronal lymphomatosis. Presenting symptoms included signs of elevated intracranial pressure with 6th nerve palsy; headache and bilateral 3rd nerve palsy; mononeuritis multiplex and unilateral hearing loss; bilateral 7th nerve paralysis and bilateral uveitis. Because neither the CSF nor the MRI were indicative, meningeal or nerve biopsies were required for conclusive diagnosis. Four patients died 10–19 months from disease onset and one patient is alive 36 months following the diagnosis. We conclude that T-cell PCNSL can present as an isolated leptomeningeal involvement which may be associated with neurolymphomatosis affecting cranial and peripheral nerves. These manifestations mimic other neurological conditions such as pseudotumor cerebri or vasculitis. Diagnosis is difficult and, as a result, frequently delayed. This calls for early consideration of meningeal or nerve biopsy whenever CSF findings are inconclusive.

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References

  1. Miller DC, Hochberg FH, Harris NL, Gruber ML, Louis DN, Cohen H (1994) Pathology with clinical correlations of primary central nervous system non-Hodgkin’s lymphoma. The Massachusetts General Hospital experience 1958–1989. Cancer 74:1383–1397

    Article  CAS  PubMed  Google Scholar 

  2. Camilleri-Broet S, Martin A, Moreau A, Angonin R, Henin D, Gontier MF, Rousselet MC, Caulet-Maugendre S, Cuilliere P, Lefrancq T, Mokhtari K, Morcos M, Broet P, Kujas M, Hauw JJ, Desablens B, Raphael M (1998) Primary central nervous system lymphomas in 72 immunocompetent patients: pathologic findings and clinical correlations. Groupe Ouest Est d’etude des Leucenies et Autres Maladies du Sang (GOELAMS). Am J Clin Pathol 110:607–612

    CAS  PubMed  Google Scholar 

  3. Gijtenbeek JM, Rosenblum MK, DeAngelis LM (2001) Primary central nervous system T-cell lymphoma. Neurology 57:716–718

    CAS  PubMed  Google Scholar 

  4. Hayabuchi N, Shibamoto Y, Onizuka Y (1999) Primary central nervous system lymphoma in Japan: a nationwide survey. Int J Radiat Oncol Biol Phys 44:265–272

    Article  CAS  PubMed  Google Scholar 

  5. Choi JS, Nam DH, Ko YH, Seo JW, Choi YL, Suh YL, Ree HJ (2003) Primary central nervous system lymphoma in Korea: comparison of B- and T-cell lymphomas. Am J Surg Pathol 27:919–928

    Article  PubMed  Google Scholar 

  6. Shenkier TN, Blay JY, O’Neill BP, Poortmans P, Thiel E, Jahnke K, Abrey LE, Neuwelt E, Tsang R, Batchelor T, Harris N, Ferreri AJ, Ponzoni M, O’Brien P, Rubenstein J, Connors JM (2005) Primary CNS lymphoma of T-cell origin: a descriptive analysis from the international primary CNS lymphoma collaborative group. J Clin Oncol 23:2233–2239

    Article  PubMed  Google Scholar 

  7. Grove A, Vyberg M (1993) Primary leptomeningeal T-cell lymphoma a case and a review of primary T-cell lymphoma of the central nervous system. Clin Neuropathol 12:7–12

    CAS  PubMed  Google Scholar 

  8. Kadoch C, Treseler P, Rubenstein JL (2006) Molecular pathogenesis of primary central nervous system lymphoma. Neurosurg Focus 21(5):E1

    Google Scholar 

  9. Grimm SA, Pulido JS, Jahnke K, Schiff D, Hall AJ, Shenkier TN, Siegal T, Doolittle ND, Batchelor T, Herrlinger U, Neuwelt EA, Laperriere N, Chamberlain MC, Blay JY, Ferreri AJ, Omuro AM, Thiel E, Abrey LE (2007) Primary intraocular lymphoma: an International Primary Central Nervous System Lymphoma Collaborative Group Report. Ann Oncol 18:1851–1855

    Article  CAS  PubMed  Google Scholar 

  10. Lachance DH, O’Neill BP, Macdonald DR, Jaeckle KA, Witzig TE, Li CY, Posner JB (1991) Primary leptomeningeal lymphoma: report of 9 cases, diagnosis with immunocytochemical analysis, and review of the literature. Neurology 41:95–100

    CAS  PubMed  Google Scholar 

  11. DeAngelis LM (1999) Primary central nervous system lymphoma. Curr Opin Neurol 12:687–691

    Article  CAS  PubMed  Google Scholar 

  12. Baehring JM, Damek D, Martin EC, Betensky RA, Hochberg FH (2003) Neurolymphomatosis. Neuro-oncology 5:104–115

    PubMed  Google Scholar 

  13. Villegas E, Villa S, López-Guillermo A, Petit J, Ribalta T, Graus F (1997) Primary central nervous system lymphoma of T-cell origin: description of two cases and review of the literature. J Neurooncol 34(2):157–161

    Article  CAS  PubMed  Google Scholar 

  14. Carod J, Eiras J, Alberdi J, Gimenez-Mas JA, Fayed N, Mayayo P, Martinez D (1995) Primary T-cell lymphoma in the centralnervous system of immunocompetent patients. Clinical and pathological study of two cases. Neurologia 10:346–349

    CAS  PubMed  Google Scholar 

  15. Dias-Silva JJ, Maestre A, Diaz-Valino J, Lopez-Facal S (1997) Primary cerebral lymphoma of T phenotype in an immunocompetent patient. Rev Neurol 25:550–552

    CAS  PubMed  Google Scholar 

  16. Feldges A, Gerhard L, Reinhardt V, Budach V (1992) Primary cerebral anaplastic T-cell-lymphoma (type Ki-1): review and case report. Clin Neuropathol 11:55–59

    CAS  PubMed  Google Scholar 

  17. Ferracini R, Bergmann M, Pileri S, Rigobello L, Azzolini U, Manetto V, Poggi S, Sabattini E, Frank G, Spagnolli F et al (1995) Primary T-cell lymphoma of the central nervous system. Clin Neuropathol 14:125–129

    CAS  PubMed  Google Scholar 

  18. Kanavaros P, Mikol J, Nemeth J, Galian A, Vaunaize J, Morinet F, Thurel C (1993) Primary T-cell malignant lymphoma of the central nervous system. Histological, immunohistochemical and ultrastructural study of a case. Pathol Res Pract 189:93–98; discussion 98–101

    Google Scholar 

  19. Kim EY, Kim SS (2005) Magnetic resonance findings of primary central nervous system T-cell lymphoma in immunocompetent patients. Acta Radiol 46:187–192

    Article  CAS  PubMed  Google Scholar 

  20. Kuwata T, Funahashi K, Itakura T, Miyamoto K, Nishiguchi T, Hayashi S, Komai N (1987) Primary T-cell type lymphoma of the central nervous system: a case report. No Shinkei Geka 15:657–661

    CAS  PubMed  Google Scholar 

  21. Lee DK, Chung CK, Kim HJ, Kim K, Choe G, Moon CW, Kim DY (2002) Multifocal primary CNS T cell lymphoma of the spinal cord. Clin Neuropathol 21:149–155

    CAS  PubMed  Google Scholar 

  22. Stark AM, Tiemann M, Dorner L, Melnikowa E, Mehdorn HM, Blomer U (2004) Primary peripheral T-cell lymphoma of the central nervous system. Zentralbl Neurochir 65:191–194

    Article  CAS  PubMed  Google Scholar 

  23. Takeshita M, Kubo O, Tajika Y, Kawamoto T, Hori T, Takakura K (1999) Primary central nervous system T-cell lymphoma case report. Neurol Med Chir (Tokyo) 39:452–458

    Article  CAS  Google Scholar 

  24. Yeh KH, Cheng AL, Tien HF (1995) Primary T cell leptomeningeal lymphoma—successful treatment with systemic chemotherapy. Oncology 52:501–504

    Article  CAS  PubMed  Google Scholar 

  25. Dulai MS, Park CY, Howell WD, Smyth LT, Desai M, Carter DM, Vogel H (2008) CNS T-cell lymphoma: an under-recognized entity? Acta Neuropathol 115:345–356

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Leslie and Michael Gaffin Center for Neuro-Oncology, Hadassah Hebrew University Hospital, Jerusalem, Israel

    Netta Levin, Natasha Aizikovich & Tali Siegal

  2. Department of Neurology, Hadassah University Hospital, Jerusalem, 91120, Israel

    Netta Levin

  3. Department of Pathology, Hadassah Hebrew University Hospital, Jerusalem, Israel

    Dov Soffer

  4. Department of Hematology, Hadassah Hebrew University Hospital, Jerusalem, Israel

    Sigal Grissaru

  5. Neuro-Radiology Unit, Hadassah Hebrew University Hospital, Jerusalem, Israel

    J. Moshe Gomori

Authors
  1. Netta Levin
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  2. Dov Soffer
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  3. Sigal Grissaru
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  4. Natasha Aizikovich
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  5. J. Moshe Gomori
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  6. Tali Siegal
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Correspondence to Netta Levin.

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Levin, N., Soffer, D., Grissaru, S. et al. Primary T-cell CNS lymphoma presenting with leptomeningeal spread and neurolymphomatosis. J Neurooncol 90, 77–83 (2008). https://doi.org/10.1007/s11060-008-9633-2

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  • DOI: https://doi.org/10.1007/s11060-008-9633-2

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