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.
Similar content being viewed by others
References
Zimmerman HM (1975) Malignant lymphomas of the nervous system. Acta Neuropathol 6:69–74
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
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
Morales DS, Siatkowski RM, Howard CW, Warman R (2000) Optic neuritis in children. J Pediatr Ophthalmol Strabismus 37:254–259
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
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
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
Fine HA, Mayer RJ (1993) Primary central nervous system lymphoma. Ann Intern Med 119:1093–1104
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
Buggage RR, Chan CC, Nussenblatt RB (2001) Ocular manifestations of central nervous system lymphoma. Curr Opin Oncol 13:137–142
Shibata S (1989) Sites of origin of primary intracerebral malignant lymphoma. Neurosurgery 25:14–19
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
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
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
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
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
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
Nasir S, DeAngelis LM (2000) Update on the management of primary CNS lymphoma. Oncology (Huntingt) 14:228–234
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
Abrey LE, Yahalom J, DeAngelis LM (2000) Treatment for primary CNS lymphoma: the next step. J Clin Oncol 18:3144–3150
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
Author information
Authors and Affiliations
Corresponding author
Rights 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
Received:
Revised:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-006-0093-8