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Primary central nervous system anaplastic large-cell lymphoma mimicking lymphomatosis cerebri

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Abstract

Primary central nervous system lymphoma (PCNSL) is usually diffuse large B-cell lymphoma. Anaplastic large-cell lymphoma (ALCL) rarely occurs in the central nervous system. PCNSL always presents as single or multiple nodular contrast-enhancing mass lesions within T2-hyperintense areas on magnetic resonance imaging (MRI). Infrequently, diffuse infiltrating change with little contrast enhancement called lymphomatosis cerebri can be seen in PCNSL. In this report, we describe a 75-year-old immunocompetent man who had progressive dementia. On MRI, diffuse white matter lesions with little contrast enhancement were observed to gradually progress, which was clinically consistent with his worsening condition. A biopsy specimen revealed non-destructive, diffusely infiltrating, anaplastic large CD30-positive lymphoma, indicating a diagnosis of ALCL. After the biopsy, he was treated by whole brain irradiation (total 46 Gy) and focal boost irradiation (total 14 Gy). However, his performance status worsened and there was no symptom improvement. The patient died 8 months after symptom onset. The clinical course, diagnostic workup, pathologic correlates, and treatment outcomes are described herein.

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References

  1. Bakshi R, Mazziotta JC, Mischel PS, Jahan R, Seligson DB, Vinters HV (1999) Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings. Dement Geriatr Cogn Disord 10:152–157

    Article  PubMed  CAS  Google Scholar 

  2. Basso U, Brandes AA (2002) Diagnostic advances and new trends for the treatment of primary central nervous system lymphoma. Eur J Cancer 38:1298–1312

    Article  PubMed  CAS  Google Scholar 

  3. Bogdahn U, Bogdahn S, Mertens HG, Dommasch D, Wodarz R, Wunsch PH, Kuhl P, Richter E (1986) Primary non-Hodgkin’s lymphomas of the CNS. Acta Neurol Scand 73:602–614

    Article  PubMed  CAS  Google Scholar 

  4. Carlson BA (1996) Rapidly progressive dementia caused by nonenhancing primary lymphoma of the central nervous system. AJNR Am J Neuroradiol 17:1695–1697

    PubMed  CAS  Google Scholar 

  5. Cooper PB, Auerbach A, Aguilera NS, Adair C, Moores L, Geyer D, Rushing EJ (2006) Rare primary CNS anaplastic large-cell lymphoma in an immunocompetent adult: a clinical-pathologic case report and review case of the literature. Clin Neuropathol 25(5):232–236

    PubMed  CAS  Google Scholar 

  6. DeAngelis LM (1993) Cerebral lymphoma presenting as a nonenhancing lesion on computed tomographic/magnetic resonance scan. Ann Neurol 33:308–311

    Article  PubMed  CAS  Google Scholar 

  7. Furusawa T, Okamoto K, Ito J, Kojima N, Oyanagi K, Tokiguchi S, Sakai K (1998) Primary central nervous system lymphoma presenting as diffuse cerebral infiltration. Radiat Med 16:137–140

    PubMed  CAS  Google Scholar 

  8. George DH, Scheithauer BW, Aker FV, Kurtin PJ, Burger PC, Cameselle-Teijeiro J, McLendon RE, Parisi JE, Paulus W, Roggendorf W, Sotelo C (2003) Primary anaplastic large-cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression. Am J Surg Pathol 27(4):487–493

    Article  PubMed  Google Scholar 

  9. Gliemroth J, Kehler U, Gaebel C, Arnold H, Missler U (2003) Neuroradiological findings in primary cerebral lymphomas of non-AIDS patients. Clin Neurol Neurosurg 105:78–86

    Article  PubMed  Google Scholar 

  10. Johnson BA, Fram EK, Johnson PC, Jacobowitz R (1997) The variable MR appearance of primary lymphoma of the central nervous system: comparison with histopathologic features. AJNR Am J Neuroradiol 18(3):563–572

    PubMed  CAS  Google Scholar 

  11. Kanai R, Shibuya M, Hata T, Hori M, Hirabayashi K, Terada T, Fujii K (2008) A case of ‘lymphomatosis cerebri’ diagnosed in an early phase and treated by whole brain radiation: case report and literature review. J Neurooncol 86(1):83–88

    Article  PubMed  Google Scholar 

  12. Kawai N, Kawanishi M, Tamiya T, Nagao S (2005) Usefulness of [18F]FDG-PET kinetic analysis in non-enhancing primary central nervous system lymphoma: case report. Ann Nucl Med 19:415–419

    Article  PubMed  Google Scholar 

  13. Kazahaya Y, Doi A, Hamaya K (1992) An autopsy case of primary central nervous system lymphoma with diffuse intracerebral lesion. No To Shinkei 44:65–70

    PubMed  CAS  Google Scholar 

  14. Kim EY, Kim SS (2005) Magnetic resonance findings of primary central nervous system T-cell lymphoma in immunocompetent patients. Acta Radiol 46(2):187–192

    Article  PubMed  CAS  Google Scholar 

  15. Kodama K, Hokama M, Kawaguchi K, Tanaka Y, Hongo K (2009) Primary ALK-1-negative anaplastic large-cell lymphoma of the brain: case report and review of the literature. Neuropathology 29(2):166–171

    Article  PubMed  Google Scholar 

  16. Lewerenz J, Ding X, Matschke J, Schnabel C, Emami P, von Borczyskowski D, Buchert R, Krieger T, de Wit M, Munchau A (2007) Dementia and leukoencephalopathy due to lymphomatosis cerebri. J Neurol Neurosurg Psychiatry 78(7):777–778

    Article  PubMed  Google Scholar 

  17. Matsumoto K, Kohmura E, Fujita T, Tsuruzono K, Tsujimura T, Kawano K (1995) Recurrent primary central nervous system lymphoma mimicking neurodegenerative disease—an autopsy case report. Neurol Med Chir (Tokyo) 35(6):360–363

    Article  CAS  Google Scholar 

  18. Omuro AM, Leite CC, Mokhtari K, Delattre JY (2006) Pitfalls in the diagnosis of brain tumours. Lancet Neurol 5(11):937–948

    Article  PubMed  Google Scholar 

  19. Ponzoni M, Terreni MR, Ciceri F, Ferreri AJ, Gerevini S, Anzalone N, Valle M, Pizzolito S, Arrigoni G (2002) Primary brain CD30+ ALK1+ anaplastic large-cell lymphoma (‘ALKoma’): the first case with a combination of ‘not common’ variants. Ann Oncol 13(11):1827–1832

    Article  PubMed  CAS  Google Scholar 

  20. Raz E, Tinelli E, Antonelli M, Canevelli M, Fiorelli M, Bozzao L, Di Piero V, Caramia F (2011) MRI findings in lymphomatosis cerebri: description of a case and revision of the literature. J Neuroimaging 21(2):e183–e186

    Article  PubMed  Google Scholar 

  21. Rollins KE, Kleinschmidt-DeMasters BK, Corboy JR, Damek DM, Filley CM (2005) Lymphomatosis cerebri as a cause of white matter dementia. Hum Pathol 36:282–290

    Article  PubMed  Google Scholar 

  22. Roman-Goldstein SM, Goldman DL, Howieson J, Belkin R, Neuwelt EA (1992) MR of primary CNS lymphoma in immunologically normal patients. AJNR Am J Neuroradiol 13:1207–1213

    PubMed  CAS  Google Scholar 

  23. Rowsell EHZN, Liwnicz BH, Cao JD, Huang Q, Wang J (2004) Primary anaplastic lymphoma kinase-negative anaplastic large-cell lymphoma of the brain in a patient with acquired immunodeficiency syndrome. Arch Pathol Lab Med 128(3):324–327

    PubMed  Google Scholar 

  24. Rupani AMC, Desai S, Rege J (2005) Primary anaplastic large-cell lymphoma of central nervous system–a case report. J Postgrad Med 51(4):326–327

    PubMed  CAS  Google Scholar 

  25. Sugie M, Ishihara K, Kato H, Nakano I, Kawamura M (2009) Primary central nervous system lymphoma initially mimicking lymphomatosis cerebri: an autopsy case report. Neuropathology 29(6):704–707

    Article  PubMed  Google Scholar 

  26. Tajima Y, Miyazaki Y, Higashi T, Kishimoto R, Sudoh K, Matsumoto A, Kikuchi S, Tashiro K (2003) Primary CD30/Ki-1 positive anaplastic large-cell lymphoma of the central nervous system occurring in a patient with a seventeen-year history of essential thrombocythemia. Leuk Lymphoma 44(7):1243–1245

    Article  PubMed  CAS  Google Scholar 

  27. Terae S, Ogata A (1996) Nonenhancing primary central nervous system lymphoma. Neuroradiology 38:34–37

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Takeshi Mikami.

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Sugino, T., Mikami, T., Akiyama, Y. et al. Primary central nervous system anaplastic large-cell lymphoma mimicking lymphomatosis cerebri. Brain Tumor Pathol 30, 61–65 (2013). https://doi.org/10.1007/s10014-012-0094-0

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  • DOI: https://doi.org/10.1007/s10014-012-0094-0

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