Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report

S. A. Grimm, C. A. McCannel, A. M P Omuro, A. J M Ferreri, J. Y. Blay, Edward Neuwelt, T. Siegal, T. Batchelor, K. Jahnke, T. N. Shenkier, A. J. Hall, F. Graus, U. Herrlinger, D. Schiff, J. Raizer, J. Rubenstein, N. Laperriere, E. Thiel, Nancy Doolittle, F. M. IwamotoL. E. Abrey

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Abstract

Objective: To describe the demographics, diagnostic details, therapeutic management, and outcome in patients with primary CNS lymphoma (PCNSL) with ocular involvement. Methods: A retrospective study of 221 patients was assembled from 16 centers in seven countries. Only HIV-negative, immunocompetent patients with brain and ocular lymphoma were included; none had systemic lymphoma. Results: Median age at diagnosis was 60. Fifty-seven percent were women. Median Eastern Cooperative Oncology Group performance status was 2. Ocular disturbance and behavioral/cognitive changes were the most common presenting symptoms. Diagnosis of lymphoma was made by brain biopsy (147), vitrectomy (65), or CSF cytology (11). Diagnosis of intraocular lymphoma was made by vitrectomy/choroidal/retinal biopsy (90) or clinical ophthalmic examination (141). CSF cytology was positive in 23%. Treatment information was available for 176 patients. A total of 102 received dedicated ocular therapy (ocular radiotherapy 79, intravitreal methotrexate 22, and both 1) in addition to treatment for their brain lymphoma. Sixty-nine percent progressed at a median of 13 months; sites of progression included brain 52%, eyes 19%, brain and eyes 12%, and systemic 2%. Patients treated with local ocular therapy did not have a statistically significant decreased risk of failing in the eyes (p ≤ 0.7). Median progression free survival and overall survival for the entire cohort were 18 and 31 months. Conclusion: This is the largest reported series of primary CNS lymphoma (PCNSL) with intraocular involvement. Progression free and overall survival was similar to that reported with PCNSL. Dedicated ocular therapy improved disease control but did not affect overall survival.

Original languageEnglish (US)
Pages (from-to)1355-1360
Number of pages6
JournalNeurology
Volume71
Issue number17
DOIs
StatePublished - Oct 21 2008

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Intraocular Lymphoma
Lymphoma
Brain
Vitrectomy
Disease-Free Survival
Cell Biology
Therapeutics
Biopsy
Survival
Methotrexate

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Grimm, S. A., McCannel, C. A., Omuro, A. M. P., Ferreri, A. J. M., Blay, J. Y., Neuwelt, E., ... Abrey, L. E. (2008). Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report. Neurology, 71(17), 1355-1360. https://doi.org/10.1212/01.wnl.0000327672.04729.8c

Primary CNS lymphoma with intraocular involvement : International PCNSL Collaborative Group Report. / Grimm, S. A.; McCannel, C. A.; Omuro, A. M P; Ferreri, A. J M; Blay, J. Y.; Neuwelt, Edward; Siegal, T.; Batchelor, T.; Jahnke, K.; Shenkier, T. N.; Hall, A. J.; Graus, F.; Herrlinger, U.; Schiff, D.; Raizer, J.; Rubenstein, J.; Laperriere, N.; Thiel, E.; Doolittle, Nancy; Iwamoto, F. M.; Abrey, L. E.

In: Neurology, Vol. 71, No. 17, 21.10.2008, p. 1355-1360.

Research output: Contribution to journalArticle

Grimm, SA, McCannel, CA, Omuro, AMP, Ferreri, AJM, Blay, JY, Neuwelt, E, Siegal, T, Batchelor, T, Jahnke, K, Shenkier, TN, Hall, AJ, Graus, F, Herrlinger, U, Schiff, D, Raizer, J, Rubenstein, J, Laperriere, N, Thiel, E, Doolittle, N, Iwamoto, FM & Abrey, LE 2008, 'Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report', Neurology, vol. 71, no. 17, pp. 1355-1360. https://doi.org/10.1212/01.wnl.0000327672.04729.8c
Grimm, S. A. ; McCannel, C. A. ; Omuro, A. M P ; Ferreri, A. J M ; Blay, J. Y. ; Neuwelt, Edward ; Siegal, T. ; Batchelor, T. ; Jahnke, K. ; Shenkier, T. N. ; Hall, A. J. ; Graus, F. ; Herrlinger, U. ; Schiff, D. ; Raizer, J. ; Rubenstein, J. ; Laperriere, N. ; Thiel, E. ; Doolittle, Nancy ; Iwamoto, F. M. ; Abrey, L. E. / Primary CNS lymphoma with intraocular involvement : International PCNSL Collaborative Group Report. In: Neurology. 2008 ; Vol. 71, No. 17. pp. 1355-1360.
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abstract = "Objective: To describe the demographics, diagnostic details, therapeutic management, and outcome in patients with primary CNS lymphoma (PCNSL) with ocular involvement. Methods: A retrospective study of 221 patients was assembled from 16 centers in seven countries. Only HIV-negative, immunocompetent patients with brain and ocular lymphoma were included; none had systemic lymphoma. Results: Median age at diagnosis was 60. Fifty-seven percent were women. Median Eastern Cooperative Oncology Group performance status was 2. Ocular disturbance and behavioral/cognitive changes were the most common presenting symptoms. Diagnosis of lymphoma was made by brain biopsy (147), vitrectomy (65), or CSF cytology (11). Diagnosis of intraocular lymphoma was made by vitrectomy/choroidal/retinal biopsy (90) or clinical ophthalmic examination (141). CSF cytology was positive in 23{\%}. Treatment information was available for 176 patients. A total of 102 received dedicated ocular therapy (ocular radiotherapy 79, intravitreal methotrexate 22, and both 1) in addition to treatment for their brain lymphoma. Sixty-nine percent progressed at a median of 13 months; sites of progression included brain 52{\%}, eyes 19{\%}, brain and eyes 12{\%}, and systemic 2{\%}. Patients treated with local ocular therapy did not have a statistically significant decreased risk of failing in the eyes (p ≤ 0.7). Median progression free survival and overall survival for the entire cohort were 18 and 31 months. Conclusion: This is the largest reported series of primary CNS lymphoma (PCNSL) with intraocular involvement. Progression free and overall survival was similar to that reported with PCNSL. Dedicated ocular therapy improved disease control but did not affect overall survival.",
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T2 - International PCNSL Collaborative Group Report

AU - Grimm, S. A.

AU - McCannel, C. A.

AU - Omuro, A. M P

AU - Ferreri, A. J M

AU - Blay, J. Y.

AU - Neuwelt, Edward

AU - Siegal, T.

AU - Batchelor, T.

AU - Jahnke, K.

AU - Shenkier, T. N.

AU - Hall, A. J.

AU - Graus, F.

AU - Herrlinger, U.

AU - Schiff, D.

AU - Raizer, J.

AU - Rubenstein, J.

AU - Laperriere, N.

AU - Thiel, E.

AU - Doolittle, Nancy

AU - Iwamoto, F. M.

AU - Abrey, L. E.

PY - 2008/10/21

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N2 - Objective: To describe the demographics, diagnostic details, therapeutic management, and outcome in patients with primary CNS lymphoma (PCNSL) with ocular involvement. Methods: A retrospective study of 221 patients was assembled from 16 centers in seven countries. Only HIV-negative, immunocompetent patients with brain and ocular lymphoma were included; none had systemic lymphoma. Results: Median age at diagnosis was 60. Fifty-seven percent were women. Median Eastern Cooperative Oncology Group performance status was 2. Ocular disturbance and behavioral/cognitive changes were the most common presenting symptoms. Diagnosis of lymphoma was made by brain biopsy (147), vitrectomy (65), or CSF cytology (11). Diagnosis of intraocular lymphoma was made by vitrectomy/choroidal/retinal biopsy (90) or clinical ophthalmic examination (141). CSF cytology was positive in 23%. Treatment information was available for 176 patients. A total of 102 received dedicated ocular therapy (ocular radiotherapy 79, intravitreal methotrexate 22, and both 1) in addition to treatment for their brain lymphoma. Sixty-nine percent progressed at a median of 13 months; sites of progression included brain 52%, eyes 19%, brain and eyes 12%, and systemic 2%. Patients treated with local ocular therapy did not have a statistically significant decreased risk of failing in the eyes (p ≤ 0.7). Median progression free survival and overall survival for the entire cohort were 18 and 31 months. Conclusion: This is the largest reported series of primary CNS lymphoma (PCNSL) with intraocular involvement. Progression free and overall survival was similar to that reported with PCNSL. Dedicated ocular therapy improved disease control but did not affect overall survival.

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