Skip to main content

Advertisement

Log in

Primary central nervous system non-Hodgkin lymphoma in childhood presenting as bilateral optic neuritis

  • Case Report
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Inroduction

Primary central nervous system lymphoma is a very rare condition in pediatric patients.

Case report

We describe the case of a 10-year old girl who presented with acute bilateral vision impairment. At the time of presentation, the only positive finding was optic disk swelling, and the brain MRI scan was normal. Seventeen months later, she developed a large-cell non-Hodgkin lymphoma in the brain, with no evidence of neoplasia elsewhere. Immunodeficiencies and Epstein–Barr virus infection could not be demonstrated. The patient was successfully treated with a combination of cyclophosphamide, etoposide, vincristine, methotrexate, and cytarabine, plus intrathecal chemotherapy. Craneospinal irradiation was not used.

Outcome

The patient’s condition is still in remission 68 months after completing the treatment.

Conclusion

This case is the only non-Hodgkin lymphoma with primary central nervous system location treated in our institution in the last 10 years and represents less than 0.5% of our non-Hodgkin lymphoma series. Due to its rare occurrence, not much is known about the clinical features and treatment outcome of primary central nervous system lymphoma in pediatric patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Zimmerman HM (1975) Malignant lymphomas of the nervous system. Acta Neuropathol 6:69–74

    MathSciNet  Google Scholar 

  2. Krogh-Jensen M, D’Amore F, Jensen MK, Christensen BE, Thorling K, Pedersen M, Johansen P, Boesen AM, Andersen E (1995) Clinicopathological features, survival and prognostic factors of primary central nervous system lymphomas and primary malignant brain tumors in a well-defined geographical area. Population based data from the Danish Lymphoma Registry, LYFO, and the Danish Cancer Registry. Leuk Lymphoma 19:223–233

    PubMed  CAS  Google Scholar 

  3. Corn BW, Marcus SM, Topham A, Hauck W, Curran WJ Jr (1997) Will primary central nervous system lymphoma the most frequent tumor diagnosed in the year 2000? Cancer 79:2409–2413

    Article  PubMed  CAS  Google Scholar 

  4. Morales DS, Siatkowski RM, Howard CW, Warman R (2000) Optic neuritis in children. J Pediatr Ophthalmol Strabismus 37:254–259

    PubMed  CAS  Google Scholar 

  5. Kosuda S, Kusano S, Ishihara S, Nawashiro H, Shima K, Kamata N, Suzuki K, Ichihara K (2003) Combined 201T1 and 67Ga brain SPECT in patients with suspected central nervous system lymphoma or germinoma: clinical and economic value. Ann Nucl Med 17:359–367

    Article  PubMed  Google Scholar 

  6. George DH, Scheithauer BW, Aker FV, Kurtin PJ, Burger PC, Cameselle-Teijeiro J, McLendon RE, Parisi JE, Paulus W, Roggendorf W, Sotelo C (2001) Primary anaplastic large cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression. Am J Surg Pathol 27:487–493

    Article  Google Scholar 

  7. Sandlund JT, Murphy SB, Santana VM, Behm F, Jones D, Berard CW, Furman WL, Ribeiro R, Crist WM, Greenwald C, Chen G, Walter A, Pui CH (2000) CNS involvement in children with newly diagnosed non-Hodgkin’s lymphoma. J Clin Oncol 18:3018–3024

    PubMed  CAS  Google Scholar 

  8. Fine HA, Mayer RJ (1993) Primary central nervous system lymphoma. Ann Intern Med 119:1093–1104

    PubMed  CAS  Google Scholar 

  9. Cassoux N, Merle-Beral H, Leblond V, Bodaghi B, Milea D, Gerber S, Fardeau C, Reux I, Xuan KH, Chan CC, LeHoang P (2000) Ocular and central nervous system lymphoma: clinical features and diagnosis. Ocul Immunol Inflamm 8:243–250

    Article  PubMed  CAS  Google Scholar 

  10. Buggage RR, Chan CC, Nussenblatt RB (2001) Ocular manifestations of central nervous system lymphoma. Curr Opin Oncol 13:137–142

    Article  PubMed  CAS  Google Scholar 

  11. Shibata S (1989) Sites of origin of primary intracerebral malignant lymphoma. Neurosurgery 25:14–19

    Article  PubMed  CAS  Google Scholar 

  12. Larocca LM, Capello D, Rinelli A, Nori S, Antinori A, Gloghini A, Cingolani A, Migliazza A, Saglio G, Cammilleri-Broet S, Raphael M, Carbone A, Gaidano G (1998) The molecular and phenotypic profile of primary central nervous system lymphoma identifies distinct categories of the disease and is consistent with histogenetic derivation from germinal center-related B cells. Blood 92:1011–1019

    PubMed  CAS  Google Scholar 

  13. Paulus W, Jellinger K, Hallas C, Ott G, Muller-Hermelink HK (1993) Human herpesvirus-6 and Epstein–Barr virus genome in primary cerebral lymphomas. Neurology 43:1591–1593

    PubMed  CAS  Google Scholar 

  14. Nelson DF, Martz KL, Bonner H, Nelson JS, Newall J, Kerman HD, Thomson JW, Murray KJ (1992) Non-Hodgkin’s lymphoma of the brain: can high dose, large volume radiation therapy improve survival? Report on a prospective trial by the radiation therapy oncology group (RTOG): RTOG 8315. Int J Radiat Oncol Biol Phys 23:9–17

    PubMed  CAS  Google Scholar 

  15. Loeffler JS, Ervin TJ, Mauch P, Skarin A, Weinstein HJ, Canellos G, Cassady JR (1985) Primary lymphoma of the central nervous system: patterns of failure and factors that influence survival. J Clin Oncol 3:490–494

    PubMed  CAS  Google Scholar 

  16. Sandor V, Stark-Vancs V, Pearson D, Nussenblat R, Whitcup SM, Brouwers P, Patronas N, Heiss J, Jaffe E, deSmet M, Kohler D, Simon R, Wittes R (1998) Phase II trail of chemotherapy alone for primary CNS and intraocular lymphoma. J Clin Oncol 16:3000–3006

    PubMed  CAS  Google Scholar 

  17. Korfel A, Martus P, Nowrousian MR, Hossfeld DK, Kirchen H, Brucher J, Stelljes M, Birkmann J, Peschel C, Pasold R, Fischer L, Jahnke K, Thiel E; German Primary Central Nervous System Lymphoma Study Group (G-PCNSL-SG) (2005) Response to chemotherapy and treating institution predict survival in primary central nervous system lymphoma. Br J Haematol 128:177–183

    Article  PubMed  Google Scholar 

  18. Nasir S, DeAngelis LM (2000) Update on the management of primary CNS lymphoma. Oncology (Huntingt) 14:228–234

    CAS  Google Scholar 

  19. Murphy SB, Bowman WP, Abromowitch M, Mirro J, Ochs J, Rivera G, Pui CH, Fairclough D, Berard CW (1986) Results of treatment of advanced-stage Burkitt’s lymphoma and B cell (SIg+) acute lymphoblastic leukemia with high-dose fractionated cyclophosphamide and coordinated high-dose methotrexate and cytarabine. J Clin Oncol 4:1732–1739

    PubMed  CAS  Google Scholar 

  20. Abrey LE, Yahalom J, DeAngelis LM (2000) Treatment for primary CNS lymphoma: the next step. J Clin Oncol 18:3144–3150

    PubMed  CAS  Google Scholar 

  21. Bessell EM, Lopez-Guillermo A, Villa S, Verger E, Nomdedeu B, Petit J, Byrne P, Montserrat E, Graus F (2002) Importance of radiotherapy in the outcome of patients with primary CNS lymphoma: an analysis of the CHOD/BVAM regimen followed by two different radiotherapy treatments. J Clin Oncol 20:231–236

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Aurora Medina-Sanson.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Medina-Sanson, A., Chico-Ponce de León, F., Cabrera-Muñoz, M. et al. Primary central nervous system non-Hodgkin lymphoma in childhood presenting as bilateral optic neuritis. Childs Nerv Syst 22, 1364–1368 (2006). https://doi.org/10.1007/s00381-006-0093-8

Download citation

  • Received:

  • Revised:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-006-0093-8

Keywords

Navigation