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Primary Central Nervous System Lymphoma Resulting in Stroke and Leukoencephalopathy

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Tumors of the Central Nervous System, Volume 9

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 9))

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Abstract

In rare instances primary CNS lymphoma (PCNSL) manifests as an intravascular tumour or shows a diffuse infiltrating phenotype known as “lymphomatosis cerebri”. Intravascular PCNSL presents with multiple cerebral infarcts caused by the occlusion of small- to medium-sized vessels while diffuse infiltrating PCNSL is characterized by progressive white matter lesions translating into behavioural changes and cognitive decline. We describe the clinical and histological peculiarities, key imaging findings, diagnostic challenges and differential diagnoses of these special types of PCNSL, and provide a review of literature cases. Prognosis is generally worse than that of classic PCNSL and critically depends on an early diagnosis.

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References

  • Anda T, Haraguchi W, Miyazato H, Tanaka S, Ishihara T, Aozasa K, Nakamichi I (2008) Ruptured distal middle cerebral artery aneurysm filled with tumor cells in a patient with intravascular large B-cell lymphoma. J Neurosurg 109:492–496

    Article  PubMed  Google Scholar 

  • Anghel G, Petrinato G, Severino A, Remotti D, Insabato L, De Renzo A, Rotoli B, Majolino I (2003) Intravascular B-cell lymphoma: report of two cases with different clinical presentation but rapid central nervous system involvement. Leuk Lymphoma 44:1353–1359

    Article  PubMed  CAS  Google Scholar 

  • Ayuso-Peralta L, Orti-Pareja M, Zurdo-Hernandez M, Jimenez-Jimenez FJ, Tejeiro-Martinez J, Ricoy JR, de la Lama A, Bernardo AI (2001) Cerebral lymphoma presenting as a leukoencephalopathy. J Neurol Neurosurg Psychiatry 71:243–246

    Article  PubMed  CAS  Google Scholar 

  • 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 

  • Batchelor T, Loeffler JS (2006) Primary CNS lymphoma. J Clin Oncol 24:1281–1288

    Article  PubMed  CAS  Google Scholar 

  • Bergmann M, Terzija-Wessel U, Blasius S, Kuchelmeister K, Kryne-Kubat B, Gerhard L, Beneicke U, Berlit P (1994) Intravascular lymphomatosis of the CNS: clinicopathologic study and search for expression of oncoproteins and Epstein-Barr virus. Clin Neurol Neurosurg 96:236–243

    Article  PubMed  CAS  Google Scholar 

  • Beristain X, Azzarelli B (2002) The neurological masquerade of intravascular lymphomatosis. Arch Neurol 59:439–443

    Article  PubMed  Google Scholar 

  • Brecher K, Hochberg FH, Louis DN, De la Monte S, Riskind P (1998) Case report of unusual leukoencephalopathy preceding primary CNS lymphoma. J Neurol Neurosurg Psychiatry 65:917–920

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  • De Toledo M, Lopez-Valdes E, Ferreiro M, Cervera JL, Ramos A, Cabello A, Hernandez-Lain A, Montes-Montes S, Lagares A, Varez-Linera Prado J (2008) Lymphomatosis cerebri as the cause of leukoen­cephalopathy. Rev Neurol 46:667–670

    PubMed  Google Scholar 

  • Ferreri AJ, Campo E, Ambrosetti A, Ilariucci F, Seymour JF, Willemze R, Arrigoni G, Rossi G, Lopez-Guillermo A, Berti E, Eriksson M, Federico M, Cortelazzo S, Govi S, Frungillo N, Dell’Oro S, Lestani M, Asioli S, Pedrinis E, Ungari M, Motta T, Rossi R, Artusi T, Iuzzolino P, Zucca E, Cavalli F, Ponzoni M (2004a) Anthracycline-based chemotherapy as primary treatment for intravascular lymphoma. Ann Oncol 15:1215–1221

    Article  PubMed  CAS  Google Scholar 

  • Ferreri AJ, Campo E, Seymour JF, Willemze R, Ilariucci F, Ambrosetti A, Zucca E, Rossi G, Lopez-Guillermo A, Pavlovsky MA, Geerts ML, Candoni A, Lestani M, Asioli S, Milani M, Piris MA, Pileri S, Facchetti F, Cavalli F, Ponzoni M (2004b) Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the ‘cutaneous variant’. Br J Haematol 127:173–183

    Article  PubMed  Google Scholar 

  • 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 

  • Gerstner EE, Batchelor TT (2007) Primary CNS lymphoma. Expert Rev Anticancer Ther 7:689–700

    Article  PubMed  Google Scholar 

  • Gerstner ER, Batchelor TT (2010) Primary central nervous system lymphoma. Arch Neurol 67:291–297

    Article  PubMed  Google Scholar 

  • Glass J, Hochberg FH, Miller DC (1993) Intravascular lymphomatosis. A systemic disease with neurologic manifestations. Cancer 71:3156–3164

    Article  PubMed  CAS  Google Scholar 

  • Greenberg SM, Rapalino O, Frosch MP (2010) Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 26–1997. A 64-year-old man with progressive dementia, seizures, and unstable gait. N Engl J Med 337:549–556

    Google Scholar 

  • 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 86:83–88

    Article  PubMed  Google Scholar 

  • Kinoshita M, Izumoto S, Hashimoto N, Kishima H, Kagawa N, Hashiba T, Chiba Y, Yoshimine T (2008) Immunohistochemical analysis of adhesion molecules and matrix metalloproteinases in malignant CNS lymphomas: a study comparing primary CNS malignant and CNS intravascular lymphomas. Brain Tumor Pathol 25:73–78

    Article  PubMed  Google Scholar 

  • Kuker W, Nagele T, Korfel A, Heckl S, Thiel E, Bamberg M, Weller M, Herrlinger U (2005) Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients. J Neurooncol 72:169–177

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • Matosevic B, Knoflach M, Furtner M, Gotwald T, Maier H, Kiechl S, Willeit J (2010) Progressive leukoencephalopathy caused by primary CNS lymphoma. J Clin Pathol 63:359–361

    Article  PubMed  Google Scholar 

  • 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) 35:360–363

    Article  CAS  Google Scholar 

  • Mihaljevic B, Sternic N, Skender GM, Sretenovic A, Antic D, Terzic T, Kostic V (2010) Intravascular large B-cell lymphoma of central nervous system – a report of two cases and literature review. Clin Neuropathol 29:233–238

    PubMed  CAS  Google Scholar 

  • Nakamichi N, Fukuhara S, Aozasa K, Morii E (2008) NK-cell intravascular lymphomatosis–a mini-review. Eur J Haematol 81:1–7

    Article  PubMed  Google Scholar 

  • Rahman F, Pittaluga S (2009) Intravascular lymphoma. Blood 114:1140

    Article  Google Scholar 

  • 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:e183–e186

    Article  Google Scholar 

  • 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 

  • Shimada K, Murase T, Matsue K, Okamoto M, Ichikawa N, Tsukamoto N, Niitsu N, Miwa H, Asaoku H, Kosugi H, Kikuchi A, Matsumoto M, Saburi Y, Masaki Y, Yamamoto K, Yamaguchi M, Nakamura S, Naoe T, Kinoshita T (2010) Central nervous system involvement in intravascular large B-cell lymphoma: a retrospective analysis of 109 patients. Cancer Sci 101:1480–1486

    Article  PubMed  CAS  Google Scholar 

  • Sierra del Rio M, Rousseau A, Soussain C, Ricard D, Hoang-Xuan K (2009) Primary CNS lymphoma in immunocompetent patients. Oncologist 14:526–539

    Article  PubMed  CAS  Google Scholar 

  • Soussain C, Hoang-Xuan K (2009) Primary central nervous system lymphoma: an update. Curr Opin Oncol 21:550–558

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  • Thurnher MM, Rieger A, Kleibl-Popov C, Settinek U, Henk C, Haberler C, Schindler E (2001) Primary central nervous system lymphoma in AIDS: a wider spectrum of CT and MRI findings. Neuroradiology 43:29–35

    Article  PubMed  CAS  Google Scholar 

  • Vital A, Sibon I (2007) A 64-year-old woman with progressive dementia and leukoencephalopathy. Brain Pathol 17(117–118):121

    Google Scholar 

  • Weaver JD, Vinters HV, Koretz B, Xiong Z, Mischel P, Kado D (2007) Lymphomatosis cerebri presenting as rapidly progressive dementia. Neurologist 13:150–153

    Article  PubMed  Google Scholar 

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Kiechl, S., Matosevic, B., Willeit, J. (2012). Primary Central Nervous System Lymphoma Resulting in Stroke and Leukoencephalopathy. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 9. Tumors of the Central Nervous System, vol 9. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5488-1_4

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  • DOI: https://doi.org/10.1007/978-94-007-5488-1_4

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