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Clinical and Diagnostic Considerations in Primary Central Nervous System Lymphoma

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Abstract

The clinical presentation and radiographic appearance of PCNSL differ in immunocompetent and immunocompromised patients and are non-specific for both. A cranial MRI revealing a homogenously enhancing, single lesion in a periventricular location is suggestive of PCNSL in the immunocompetent patient. After neuroimaging suggestive of PCNSL, definitive diagnosis should be established by analysis of CSF, vitreous fluid, or biopsy specimen. Brain biopsy remains the gold standard for PCNSL diagnosis in all patients. At the time of diagnosis, the patient should undergo an extent of disease evaluation as recommended by the International PCNSL Collaborative Group (IPCG). This evaluation includes a physical examination; ophthalmological evaluation with a slit-lamp examination; body CT scans; contrast-enhanced brain MRI; lumbar puncture, if safe; bone marrow biopsy; serum LDH; and HIV serology. Testicular ultrasound, for men, and body FDG-PET should also be considered. Baseline and serial evaluation of cognitive function is critical in this patient population, as both the tumor and anti-tumor therapy are capable of negatively impacting neuropsychological function. Prognostic scoring systems are emerging for the PCNSL patient population.

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References

  1. Schabet M (1999) Epidemiology of primary CNS lymphoma. J Neurooncol 43:199–201

    Article  PubMed  CAS  Google Scholar 

  2. Kadan-Lattick NS, Skluzacek MC, Gurney JG (2002) Decreasing incidence rates of primary central nervous system lymphoma. Cancer 95:193–202

    Article  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  4. Josephson SA, Papanastassiou AM, Berger MS et al (2007) The diagnostic utility of brain biopsy procedures in patients with rapidly deteriorating neurological conditions or dementia. J Neurosurg 106:72–75

    Article  PubMed  Google Scholar 

  5. Weller M (1999) Glucocorticoid treatment of primary CNS lymphoma. J Neurooncol 43:237–239

    Article  PubMed  CAS  Google Scholar 

  6. Mathew BS, Carson K, Grossman SA (2006) Initial response to glucocorticoids. Cancer 106:383–387

    Article  PubMed  Google Scholar 

  7. Bataille B, Delwail V, Menet E et al (2000) Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg 92:261–266

    Article  PubMed  CAS  Google Scholar 

  8. Kiewe P, Fischer L, Martus P et al (2010) Meningeal dissemination in primary CNS lymphoma: diagnosis, treatment, and survival in a large monocenter cohort. Neurooncology 12:409–417

    Google Scholar 

  9. Balmaceda C, Gaynor JJ, Sun M et al (1995) Leptomeningeal tumor in primary central nervous system lymphoma: recognition, significance, and implications. Ann Neurol 38(2):202–209

    Article  PubMed  CAS  Google Scholar 

  10. Grimm SA, Pulido JS, Jahnke K et al (2007) Primary intraocular lymphoma: an international primary central nervous system lymphoma collaborative group report. Ann Oncol 18:1851–1855

    Article  PubMed  CAS  Google Scholar 

  11. DeAngelis LM (1990) Primary central nervous system lymphoma imitates multiple sclerosis. J Neurooncol 9:177–181

    Article  PubMed  CAS  Google Scholar 

  12. Buhring U, Herrlinger U, Krings T et al (2001) MRI features of primary central nervous system lymphomas at presentation. Neurology 57:393–396

    PubMed  CAS  Google Scholar 

  13. 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 

  14. Holtas S, Nyman U, Cronqvist S (1984) Computed tomography of malignant lymphoma of the brain. Neuroradiology 26:33–38

    Article  PubMed  CAS  Google Scholar 

  15. Jack CR Jr., O’Neill BP, Banks PM, Reese DF (1988) Central nervous system lymphoma: histologic types and CT appearance. Radiology 167:211–215

    PubMed  Google Scholar 

  16. 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. Am J Neuroradiol 18:563–572

    PubMed  CAS  Google Scholar 

  17. Lee YY, Bruner JM, Van Tassel P, Libshitz HI (1986) Primary central nervous system lymphoma: CT and pathologic correlation. Am J Roentgenol 147:747–752

    CAS  Google Scholar 

  18. Roman-Goldstein SM, Goldman DL, Howieson J et al (1992) MR of primary CNS lymphoma in immunologically normal patients. Am J Neuroradiol 13:1207–1213

    PubMed  CAS  Google Scholar 

  19. Watanabe M, Tanaka R, Takeda N et al (1992) Correlation of computed tomography with the histopathology of primary malignant lymphoma of the brain. Neuroradiology 34:36–42

    Article  PubMed  CAS  Google Scholar 

  20. Kuker W, Nagele T, Korfel A et al (2005) Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients. J Neurooncol 72:169–177

    Article  PubMed  Google Scholar 

  21. Hochberg FH, Miller DC (1988) Primary central nervous system lymphoma. J Neurosurg 68:835–853

    Article  PubMed  CAS  Google Scholar 

  22. Barajas RF, Rubenstein JL, Chang JS et al (2010) Diffusion-weighted MR imaging derived apparent diffusion coefficient is predictive of clinical outcome in primary central nervous system lymphoma. Am J Neuroradiol 31:60–66

    Article  PubMed  Google Scholar 

  23. Hoffman JM, Waskin HA, Schifter T et al (1993) FDG-PET in differentiating lymphoma from nonmalignant central nervous system lesions in patients with AIDS. J Nucl Med 34:567–575

    PubMed  CAS  Google Scholar 

  24. Villringer K, Jager H, Dichgans M et al (1995) Differential diagnosis of CNS lesions in AIDS patients by FDG-PET. J Comput Assist Tomogr 19:532–536

    Article  PubMed  CAS  Google Scholar 

  25. Mohile NA, DeAngelis LM, Abrey LE (2008) Utility of brain FDG-PET in primary CNS lymphoma. Clin Adv Hematol Oncol 6:818–820

    PubMed  Google Scholar 

  26. Fischer L, Martus P, Weller M et al (2008) Meningeal dissemination in primary CNS lymphoma. Neurology 71:1102–1108

    Article  PubMed  CAS  Google Scholar 

  27. Galoin S, Daste G, Apoil P-A et al (1997) Polymerase chain reaction on cerebrospinal fluid cells in the detection of leptomeningeal involvement by B-cell lymphoma and leukaemia: a novel strategy and its implications. Br J Haematol 99:122–130

    Article  PubMed  CAS  Google Scholar 

  28. Rhodes CM, Glantz MJ, Glantz L et al (1996) A comparison of polymerase chain reaction examination of cerebrospinal fluid and conventional cytology in the diagnosis of lymphomatous meningitis. Cancer 77:543–548

    Article  PubMed  CAS  Google Scholar 

  29. Storch-Hagenlocher B, Haas J, Vogt-Schaden ME et al (2000) Molecular analysis of the CDR3 encoding region of the immunoglobulin heavy chain locus in cerebrospinal fluid cells as a diagnostic tool in lymphomatous meningitis. Ann Neurol 47(2):211–217

    Article  PubMed  CAS  Google Scholar 

  30. Chan CC, Wallace DJ (2004) Intraocular lymphoma: update on diagnosis and management. Cancer Control 11:285–295

    PubMed  Google Scholar 

  31. Grimm SA, McCannel CA, Omuro AMP et al (2008) Primary CNS lymphoma with intraocular involvement. International PCNSL collaborative group report. Neurology 71:1355–1360

    Article  PubMed  CAS  Google Scholar 

  32. Sherman ME, Erozan YS, Mann RB et al (1991) Stereotactic brain biopsy in the diagnosis of malignant lymphoma. Am J Clin Pathol 95(6):878–883

    PubMed  CAS  Google Scholar 

  33. Apuzzo ML, Sabshin JK (1983) Computed tomographic guidance stereotaxis in the management of intracranial mass lesions. Neurosurgery 12(3):277–285

    Article  PubMed  CAS  Google Scholar 

  34. Bellinzona M, Roser F, Ostertag H et al (2005) Surgical removal of primary central nervous system lymphomas presenting as space occupying lesions. Eur J Surg Oncol 31:100–105

    Article  PubMed  CAS  Google Scholar 

  35. Abrey LE, Batchelor TT, Ferreri AJ et al (2005) Report of an international workshop to standardize baseline evaluation and response criteria for primary CNS lymphoma. J Clin Oncol 23:5034–5043

    Article  PubMed  Google Scholar 

  36. Ferreri AJM, Reni M, Pasini F et al (2002) A multicenter study of treatment of primary CNS lymphoma. Neurology 58:1513–1520

    PubMed  CAS  Google Scholar 

  37. DeAngelis LM (1999) Primary central nervous system lymphoma. Curr Opin Neurol 12(6):687–691

    Article  PubMed  CAS  Google Scholar 

  38. Nasir S, DeAngelis LM (2000) Update on the management of primary CNS lymphoma. Oncology 14(2):228–244

    PubMed  CAS  Google Scholar 

  39. O’Neill BP, Dinapoli RP, Kurtin PJ, Habermann TM (1995) Occult systemic non-Hodgkin’s lymphoma (NHL) in patients initially diagnosed as primary central nervous system lymphoma (PCNSL): How much staging is enough?. J Neurooncol 25(1):67–71

    Article  PubMed  Google Scholar 

  40. Mohile NA, Deangelis LM, Abrey LE (2008) The utility of body FDG PET in staging primary central nervous system lymphoma. Neurooncology 10:223–228

    Google Scholar 

  41. Jahnke K, Hummel M, Korfel A et al (2006) Detection of subclinical systemic disease in primary CNS lymphoma by polymerase chain reaction of the rearranged immunoglobulin heavy-chain genes. J Clin Oncol 24:4754–4757

    Article  PubMed  CAS  Google Scholar 

  42. McCann KJ, Ashton-Key M, Smith K et al (2009) Primary central nervous system lymphoma: tumor-related clones exist in the blood and bone marrow with evidence of separate development. Blood 113:4677–4680

    Article  PubMed  CAS  Google Scholar 

  43. Buggage RR, Chan C-C, Nussenblat RB (2001) Ocular manifestations of central nervous system lymphoma. Curr Opin Oncol 13(3):137–142

    Article  PubMed  CAS  Google Scholar 

  44. Ferreri AJ, Blay JY, Reni M et al (2003) Prognostic scoring system for primary CNS lymphomas: the international extranodal lymphoma study group experience. J Clin Oncol 21:266–272

    Article  PubMed  Google Scholar 

  45. Abrey LE, Ben-Porat L, Panageas KS et al (2006) Primary central nervous system lymphoma: the Memorial Sloan-Kettering Cancer Center prognostic model. J Clin Oncol 24:5711–5715

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Stephen E. and Catherine Pappas Center for Neuro-Oncology, Departments of Neurology and Radiation Oncology, Division of Hematology and Oncology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA

    Tracy Batchelor

  2. Blood Brain Barrier and Neuro-Oncology Program, Departments of Neurology and Neurosurgery, Oregon Health Sciences University, Portland, OR, 97239, USA

    Edward Neuwelt

  3. Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, 02114, USA

    R. Gilberto Gonzalez

  4. Department of Neurology, Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital, Harvard Medical School, Fruit Street 55, Boston, MA, 02114, USA

    Daphne L. Wang

Authors
  1. Tracy Batchelor
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  2. Edward Neuwelt
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  3. Daphne L. Wang
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  4. R. Gilberto Gonzalez
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Correspondence to Tracy Batchelor .

Editor information

Editors and Affiliations

  1. , Stephen E. and Catherine Pappas Center f, Massachusetts General Hospital, Fruit St. 55, Boston, 02114, Massachusetts, USA

    Tracy Batchelor

  2. Dept. Neurology, Memorial Sloan-Kettering Cancer Center, York Ave. 1275, New York, 10021-6007, New York, USA

    Lisa M. DeAngelis

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Batchelor, T., Neuwelt, E., Wang, D.L., Gonzalez, R.G. (2012). Clinical and Diagnostic Considerations in Primary Central Nervous System Lymphoma. In: Batchelor, T., DeAngelis, L. (eds) Lymphoma and Leukemia of the Nervous System. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7668-0_6

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