Annals of Hematology

, Volume 94, Issue 3, pp 409–414 | Cite as

Prognostic impact of intraocular involvement in primary CNS lymphoma: experience from the G-PCNSL-SG1 trial

  • Stephan Kreher
  • Felicitas Strehlow
  • Peter Martus
  • Patrick Roth
  • Bernd Hertenstein
  • Alexander Röth
  • Tobias Birnbaum
  • Frank Griesinger
  • Michael Rauch
  • Lothar Kanz
  • Eckhard Thiel
  • Michael Weller
  • Agnieszka Korfel
Original Article


The impact of intraocular involvement (IOL) in primary CNS lymphoma (PCNSL) has not been sufficiently evaluated. Here, we present the analysis of IOL in the only completed randomized phase III trial in PCNSL. The G-PCNSL-SG1 study evaluated the role of whole-brain radiotherapy in primary therapy of PCNSL. Data of the 526 eligible study patients were checked, and clinical characteristics, therapy, and outcome of patients with IOL diagnosed at study inclusion were analyzed. Ophthalmologic examination at study inclusion was performed in 297 patients (56.5 %) of whom IOL was diagnosed in 19 (6.4 %). Clinical characteristics did not significantly differ between patients with IOL (IOL+) and those without (IOL−). The median progression-free survival (PFS) in the IOL+ group was 3.5 months (95 % CI 0.0–7.07) as compared to 8.3 months (95 % CI 4.78–11.78) in the IOL− group (P = 0.004), the median overall survival (OS) was 13.2 months (95 % CI 0.86–25.62) and 20.5 months (95 % CI 15.56–25.5), respectively (P = 0.155). In multivariate analysis, a significantly inferior PFS and OS for IOL+ patients were found. IOL at diagnosis of PCNSL was an independent negative prognostic indicator for PFS and OS in this analysis.


Intraocular lymphoma Primary CNS lymphoma Progression-free survival Overall survival 


Conflict of interest

MW, ET, and AK were the principal investigators; PR, BH, AR, TB, FG, MR, MW, ET, and AK contributed patients to the trial; PM did the statistical analyses; SK, FS, and AK wrote the report; and all authors approved the report. The authors report no potential conflicts of interest.

Supplementary material

277_2014_2212_MOESM1_ESM.doc (40 kb)
Supplementary Table 1 (DOC 40 kb)


  1. 1.
    Korfel A, Schlegel U (2013) Diagnosis and treatment of primary CNS lymphoma. Nat Rev Neurol 9:317–327PubMedCrossRefGoogle Scholar
  2. 2.
    Grimm SA, McCannel CA, Omuro AM et al (2008) Primary CNS lymphoma with intraocular involvement: international PCNSL collaborative group report. Neurology 71:1355–1360PubMedCentralPubMedCrossRefGoogle Scholar
  3. 3.
    Peterson K, Gordon KB, Heinemann MH, DeAngelis LM (1993) The clinical spectrum of ocular lymphoma. Cancer 72:843–849PubMedCrossRefGoogle Scholar
  4. 4.
    Cassoux N, Merle-Beral H, Leblond V et al (2000) Ocular and central nervous system lymphoma: clinical features and diagnosis. Ocul Immunol Inflamm 8:243–250PubMedCrossRefGoogle Scholar
  5. 5.
    Kuker W, Herrlinger U, Gronewaller E, Rohrbach JM, Weller M (2002) Ocular manifestation of primary nervous system lymphoma: what can be expected from imaging? J Neurol 249:1713–1716PubMedCrossRefGoogle Scholar
  6. 6.
    Chan CC, Wallace DJ (2004) Intraocular lymphoma: update on diagnosis and management. Cancer Control 11:285–295PubMedCentralPubMedGoogle Scholar
  7. 7.
    Ferreri AJ, Blay JY, Reni M et al (2002) Relevance of intraocular involvement in the management of primary central nervous system lymphomas. Ann Oncol 13:531–538PubMedCrossRefGoogle Scholar
  8. 8.
    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 21:266–272PubMedCrossRefGoogle Scholar
  9. 9.
    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 24:5711–5715PubMedCrossRefGoogle Scholar
  10. 10.
    Thiel E, Korfel A, Martus P et al (2010) High-dose methotrexate with or without whole brain radiotherapy for primary CNS lymphoma (G-PCNSL-SG-1): a phase 3, randomised, non-inferiority trial. Lancet Oncol 11:1036–1047PubMedCrossRefGoogle Scholar
  11. 11.
    DeAngelis LM, Seiferheld W, Schold SC, Fisher B, Schultz CJ (2002) Combination chemotherapy and radiotherapy for primary central nervous system lymphoma: Radiation Therapy Oncology Group Study 93–10. J Clin Oncol 20:4643–4648PubMedCrossRefGoogle Scholar
  12. 12.
    Poortmans PM, Kluin-Nelemans HC, Haaxma-Reiche H et al (2003) High-dose methotrexate-based chemotherapy followed by consolidating radiotherapy in non-AIDS-related primary central nervous system lymphoma: European Organization for Research and Treatment of Cancer Lymphoma Group phase II trial 20962. J Clin Oncol 21:4483–4488PubMedCrossRefGoogle Scholar
  13. 13.
    Soussain C, Hoang-Xuan K, Taillandier L et al (2008) Intensive chemotherapy followed by hematopoietic stem-cell rescue for refractory and recurrent primary CNS and intraocular lymphoma: Societe Francaise de Greffe de Moelle Osseuse-Therapie Cellulaire. J Clin Oncol 26:2512–2518PubMedCrossRefGoogle Scholar
  14. 14.
    Pels H, Schmidt-Wolf IG, Glasmacher A et al (2003) Primary central nervous system lymphoma: results of a pilot and phase II study of systemic and intraventricular chemotherapy with deferred radiotherapy. J Clin Oncol 21:4489–4495PubMedCrossRefGoogle Scholar
  15. 15.
    Shah GD, Yahalom J, Correa DD et al (2007) Combined immunochemotherapy with reduced whole-brain radiotherapy for newly diagnosed primary CNS lymphoma. J Clin Oncol 25:4730–4735PubMedCrossRefGoogle Scholar
  16. 16.
    Nussenblatt RB, Chan CC, Wilson WH, Hochman J, Gottesman M (2006) International Central Nervous System and Ocular Lymphoma Workshop: recommendations for the future. Ocul Immunol Inflamm 14:139–144PubMedCentralPubMedCrossRefGoogle Scholar
  17. 17.
    Whitcup SM, Chan CC, Buggage RR, Nussenblatt RB, Byrnes GA, Rubin BI (2000) Improving the diagnostic yield of vitrectomy for intraocular lymphoma. Arch Ophthalmol 118:446PubMedGoogle Scholar
  18. 18.
    Hormigo A, Abrey L, Heinemann MH, DeAngelis LM (2004) Ocular presentation of primary central nervous system lymphoma: diagnosis and treatment. Br J Haematol 126:202–208PubMedCrossRefGoogle Scholar
  19. 19.
    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 23:5034–5043PubMedCrossRefGoogle Scholar
  20. 20.
    de Smet MD, Vancs VS, Kohler D, Solomon D, Chan CC (1999) Intravitreal chemotherapy for the treatment of recurrent intraocular lymphoma. Br J Ophthalmol 83:448–451PubMedCentralPubMedCrossRefGoogle Scholar
  21. 21.
    Fishburne BC, Wilson DJ, Rosenbaum JT, Neuwelt EA (1997) Intravitreal methotrexate as an adjunctive treatment of intraocular lymphoma. Arch Ophthalmol 115:1152–1156PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Stephan Kreher
    • 1
  • Felicitas Strehlow
    • 1
  • Peter Martus
    • 2
  • Patrick Roth
    • 3
  • Bernd Hertenstein
    • 4
  • Alexander Röth
    • 5
  • Tobias Birnbaum
    • 6
  • Frank Griesinger
    • 7
  • Michael Rauch
    • 8
  • Lothar Kanz
    • 9
  • Eckhard Thiel
    • 1
  • Michael Weller
    • 3
  • Agnieszka Korfel
    • 1
  1. 1.Department of Hematology & OncologyCampus Benjamin FranklinBerlinGermany
  2. 2.Institute of Clinical Epidemiology and Applied BiostatisticsUniversity TuebingenTuebingenGermany
  3. 3.Department of NeurologyUniversity Hospital ZurichZurichSwitzerland
  4. 4.Department of Hematology & OncologyKlinikum Bremen MitteBremenGermany
  5. 5.Department of Hematology, West German Cancer CenterUniversity Hospital EssenEssenGermany
  6. 6.Department of NeurologyUniversity Hospital GrosshadernMunichGermany
  7. 7.Department of Radiotherapy and Medical OncologyPius Hospital OldenburgOldenburgGermany
  8. 8.Department of Hematology & OncologyEvangelisches Krankenhaus BielefeldBielefeldGermany
  9. 9.University Department of Medicine, Department of Internal MedicineUniversity Hospital TuebingenTuebingenGermany

Personalised recommendations