Primary Central Nervous System Lymphoma in an Immunocompetent Young Woman

Document Type : Case Report


1 Assistant Professor of Neurology, Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran

2 Student of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran

3 Assistant Professor of Clinical and Surgical Pathology, Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran


Primary central nervous system lymphoma is a rare brain tumor and the most common risk factor is immune system deficiency. This tumor can be seen in normal people however it is mainly seen in an age range of 50-70 years and predominantly in the male gender. The incidence of this disease in different ages is also influenced by race. As reported, at the age below 50-years blacks have had higher overall incidence rates in comparison to whites. Although analysis of CSF and hematologic tests can help diagnosis the only definite diagnosis tool is biopsy. The tumor cell’s high susceptibility to corticosteroid-dependent apoptosis. Corticosteroids have not been used before biopsy. The main treatment strategy is high dose methotrexate (HD-MTX) - based chemotherapy and whole brain radiation and surgery is restricted to biopsy for diagnostic purposes only. Here we present a case of a young female without immune deficiency who was diagnosed with primary central nervous system lymphoma.


  1. Dolecek TA, Propp JM, Stroup NE, Kruchko C. CBTRUS statistical report: Primary brain and central nervous system tumors diagnosed in the United States in 2005–2009. NeuroOncol 2012; 14 Suppl 5(Suppl 5):v1-49.
  2. DeAngelis LM, Wong E, Rosenblum M, Furneaux H. Epstein-Barr virus in acquired immune deficiency syndrome (AIDS) and non-AIDS primary central nervous system lymphoma. Cancer 1992; 70(6):1607-11.
  3. 3. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. USA: IARC Publication; 2008.
  4. Pels H, Schlegel U. Primary central nervous system lymphoma. Curr Treat Options Neurol 2006; 8(4):346-57.
  5. Basmaci M, Hasturk AE, Ayata T. Management of primary diffuse large B-Cell lymphoma of the central nervous system: clinical study. Journal of Neurological Sciences (Turkish) 2013; 30(2):469-76.
  6. Doucet S, Kumthekar P, Raizer J. Primary central nervous system lymphoma. Current Treatment Options in Oncology 2013; 14(2):185-97.
  7. Baraniskin A, Deckert M, Schulte-Altedorneburg G, Schlegel U, Schroers R. Current strategies in the diagnosis of diffuse large B-cell lymphoma of the central nervous system. B J Haematol 2012; 156(4):421-32.
  8. Ghosal N, Hegde AS, Murthy G, Furtado SV. Smear preparation of intracranial lesions: a retrospective study of 306 cases. Diagn Cytopathol 2011; 39(8):582-92.
  9. Phillips EH, Fox CP, Cwynarski K. Primary CNS lymphoma. Curr Hematol Malig Rep 2014; 9(3):243-53.
  10.  Fine HA, Mayer RJ Primary central nervous system lymphoma. Ann Intern Med 1993; 119(11):1093-104.
  11. Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ. Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer 2011; 105(9):1414-8.
  12. Thurnher MM, Rieger A, Kleibl-Popov C, Settinek U, Henk C, Haberler C, et al. Primary central nervous system lymphoma in AIDS: a wider spectrum of CT and MRI findings. Neuroradiology 2001; 43(1):29-35.
  13. Rudresha AH, Chaudhuri T, Lakshmaiah KC, Babu G, Lokesh KN, Rajeev LK. Primary central nervous system lymphoma in immunocompetent patients: a regional cancer center experience. South Asian J Cancer 2017; 6(4):165-8.
  14. Ferreri AJ, Blay JY, Reni M, Pasini F, Spina M, Ambrosetti A, et al. Prognostic scoring system for primary CNS lymphomas: the international extranodal lymphoma study group experience. J Clin Oncol 2003; 21(2):266-72.
  15. Bruck W, Brunn A, Klapper W, Kulhmann T, Metz I, Paulus W, et al. Differential diagnosis of lymphoid infiltrates in the central nervous system: the experience of the network lymphomas and lymphomatoid lesions in the nervous system. Pathologe 2013; 34(3):186-97. [In German].
  16. Porter AB, Giannini C, Kaufmann T, Luchinetti CF, Wu W, Decker PA, et al. Primary central nervous system lymphoma can be histologically diagnosed after previous corticosteroid use: a pilot study to determine whether corticosteroids prevent the diagnosis of primary central nervous system lymphoma. Ann Neurol 2008; 63(5):662-7.