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Isolated Primary Lymphomatoid Granulomatosis of Central Nervous System

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Abstract

Lymphomatoid granulomatosis (LG) is an infrequent extranodal Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder characterized by angiocentric and angiodestructive polymorphic lymphoid infiltration. CNS is involved in one of every 4 patients, but isolated CNS involvement at presentation is rare. A 67-year-old man was admitted to our hospital because of visual impairment. Magnetic resonance imaging (MRI) revealed a suprasellar mass lesion isointense to gray matter on T1 and T2-weighted images. The hypotalamic/chiazmatic mass was resected through a transsphenoidal approach. Pathological examination of the biopsy specimen revealed large atypical, CD20-positive B-lymphocytes within a background containing numerous CD3-positive small T-lymphocytes and scattered admixed plasma cells and histiocytes. Necrotic areas and vascular infiltration by a mixed mononuclear cell infiltrate with scattered large atypical lymphoid cells was present. In situ hybridization for EBV showed few large cells both around blood vessels and adjacent to the necrotic zone. This morphologic and immunophenotypic features was diagnostic for lymphomatoid granulomatosis. The patient was successfully treated with steroids, high-dose methotrexate and radiotherapy.

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References

  1. Pittaluga S, Wilson WH, Jaffe ES (2008) Lymphomatoid granulomatosis. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (eds) WHO classification of tumours of haematopoietic and lymphoid tissues, 4th edn. IARC Press, Lyon, pp 247–249

    Google Scholar 

  2. Jaffe ES, Wilson WH (2001) Lymphomatoid granulomatosis. In: Jaffe ES, Harris NL, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC (eds) World Health Organization classification of tumors. Pathology and genetics of tumors of haematopoietic and lymphoid tissues. IARC Press, Lyon, pp 185–187

    Google Scholar 

  3. Katzenstein AL, Carrington CB, Liebow AA (1979) Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases. Cancer 43:360–373

    Article  PubMed  CAS  Google Scholar 

  4. Katzenstein AL, Doxtader E, Narendra S (2010) Lymphomatoid granulomatosis: insights gained over 4 decades. Am J Surg Pathol 34:e35–e48

    Article  PubMed  Google Scholar 

  5. Gitelson E, Al-Saleem T, Smith MR (2009) Lymphomatoid granulomatosis: challenges in diagnosis and treatment. Clin Adv Hematol Oncol 7:68–70

    PubMed  Google Scholar 

  6. Kokmen E, Billman JK Jr, Abell MR (1977) Lymphomatoid granulomatosis clinically confined to the CNS. A case report. Arch Neurol 34:782–784

    Article  PubMed  CAS  Google Scholar 

  7. Schmidt BJ, Meagher-Villemure K, Del Carpio J (1984) Lymphomatoid granulomatosis with isolated involvement of the brain. Ann Neurol 15:478–481

    Article  PubMed  CAS  Google Scholar 

  8. Kerr RS, Hughes JT, Blamires T, Teddy PJ (1987) Lymphomatoid granulomatosis apparently confined to one temporal lobe. Case report. J Neurosurg 67:612–615

    Article  PubMed  CAS  Google Scholar 

  9. Demetrick DJ, Hamilton MG, Curry BC, Tranmer BI (1992) Epstein-Barr virus associated primary B-cell lymphoproliferative disorder of the cerebellum in an immune competent man. Cancer 70:519–528

    Article  PubMed  CAS  Google Scholar 

  10. Hamilton MG, Demetrick DJ, Tranmer BI, Curry B (1994) Isolated cerebellar lymphomatoid granulomatosis progressing to malignant lymphoma. Case report. J Neurosurg 80:314–320

    Article  PubMed  CAS  Google Scholar 

  11. Tateishi U, Terae S, Ogata A, Sawamura Y, Suzuki Y, Abe S, Miyasaka K (2001) MR imaging of the brain in lymphomatoid granulomatosis. Am J Neuroradiol 22:1283–1290

    PubMed  CAS  Google Scholar 

  12. Okuda T, Fumiharu A, Kataoka K, Taneda M (2008) A case of lymphomatoid granulomatosis followed for 14 months on the basis of clinical and histological findings. Brain Tumor Pathol 25:33–38

    Article  PubMed  Google Scholar 

  13. Lucantoni C, De Bonis P, Doglietto F, Esposito G, Larocca LM, Mangiola A, Martini M, Papacci F, Teofili L, Pompucci A (2009) Primary cerebral lymphomatoid granulomatosis: report of four cases and literature review. J Neurooncol 94:235–242

    Article  PubMed  Google Scholar 

  14. Katzenstein A-LA, Peiper SC (1990) Detection of Epstein-Barr virus genomes in lymphomatoid granulomatosis: analysis of 29 cases by the polymerase chain reaction technique. Mod Pathol 3:435–441

    PubMed  CAS  Google Scholar 

  15. Wilson WH, Kingma DW, Raffeld M, Wittes RE, Jaffe ES (1996) Association of lymphomatoid granulomatosis with Epstein-Barr viral infection of B lymphocytes and response to interferon-alpha 2b. Blood 87:4531–4537

    PubMed  CAS  Google Scholar 

  16. George JC, Caldemeyer KS, Smith RR, Czaja JT (1993) CNS lymphomatoid granulomatosis in AIDS: CT and MR appearances. AJR Am J Roentgenol 161:381–383

    PubMed  CAS  Google Scholar 

  17. Wyen C, Stenzel W, Hoffmann C, Lehmann C, Deckert M, Fatkenheuer G (2007) Fatal cerebral lymphomatoid granulomatosis in an HIV-1-infected patient. J Infect 54:e175–e178

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Medical Oncology, Department of Internal Medicine, Gazi University Faculty of Medicine, Besevler, Ankara, 06500, Turkey

    Bulent Cetin, Mustafa Benekli, Ugur Coskun & Suleyman Buyukberber

  2. Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey

    Nalan Akyurek & Esra Karakus

  3. Department of Radiology, Goztepe Education and Research Hospital, Istanbul, Turkey

    Senem Senturk

  4. Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey

    Hayrettin Koklu

Authors
  1. Bulent Cetin
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  2. Mustafa Benekli
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  3. Nalan Akyurek
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  4. Senem Senturk
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  5. Hayrettin Koklu
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  6. Esra Karakus
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  7. Ugur Coskun
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  8. Suleyman Buyukberber
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Correspondence to Bulent Cetin.

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Cetin, B., Benekli, M., Akyurek, N. et al. Isolated Primary Lymphomatoid Granulomatosis of Central Nervous System. Indian J Hematol Blood Transfus 29, 39–42 (2013). https://doi.org/10.1007/s12288-011-0123-x

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  • DOI: https://doi.org/10.1007/s12288-011-0123-x

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