BACKGROUND: Spinal cord compression is a rare presentation of non-Hodgkin lymphoma (NHL) in children. We aimed to describe the prevalence, histological subtypes, clinical presentation, therapy, and outcome of those children in a population-based cohort. The chemotherapy regimen remained comparable over time. METHODS: We retrospectively identified all children and adolescents with paresis as initial manifestations of the NHL between January 1990 and December 2020 from the NHL-BFM database. Characteristics, therapy, and outcome data were gathered from the database and patient files. RESULTS: Fifty-seven of 4779 children (1.2%) presented with initial paresis due to spinal cord compression. The median age was 10.3 years (range, 3.1-18.0 years), and 33% were female. Initial symptoms were paresis/weakness (n = 50, 88%), back pain (n = 33, 58%), paresthesia (n = 23, 40%), and bladder dysfunction and/or constipation (n = 22, 39%), persisting for a median of 14 days before diagnosis. Subtype distribution was mature B-NHL (n = 41, 72%), precursor B-lymphoblastic lymphoma (LBL) (n = 12, 21%), anaplastic large cell lymphoma (ALCL) (n = 3, 5%), and T-LBL (n = 1, 2%). Initial emergency therapy included surgery (70%) and/or chemotherapy/steroids (63%). Five-year event-free survival and overall survival (80% ± 5% and 82% ± 5%, respectively) were comparable with all other NHL patients. Neurological symptoms persisted in approximately one-third of surviving patients at the last follow-up. CONCLUSION: 1.2% of pediatric NHL patients presented with paresis from spinal cord compression mainly due to B-cell lymphomas. Neurological sequelae were observed in one-third of surviving patients.

Department of Pediatric Hematology and Oncology Children's Hospital Hannover Medical School Hannover Germany

Department of Pediatric Hematology and Oncology St Anna Children's Hospital Medical University of Vienna Vienna Austria

Pediatric Hematology and Oncology and NHL BFM Study Centre University Hospital Muenster Muenster Germany

Pediatric Hematology and Oncology and NHL BFM Study Centre University Medical Centre Hamburg Eppendorf Hamburg Germany

Pediatric Hematology and Oncology Charles University and University Hospital Motol Prague Czech Republic

Pediatric Hematology Oncology Unit Division of Pediatrics Department Woman Mother Child University Hospital of Lausanne and University of Lausanne Lausanne Switzerland

St Anna Children's Cancer Research Institute Vienna Austria

Zobrazit více v PubMed

Kurucu N, Akyuz C, Varan A, et al. Primary paraspinal and spinal epidural non‐Hodgkin lymphoma in childhood. J Pediatr Hematol Oncol. 2021;43(3):e395‐e400.

Pui CH, Dahl GV, Hustu HO, Murphy SB. Epidural spinal cord compression as the initial finding in childhood acute leukemia and non‐Hodgkin lymphoma. J Pediatr. 1985;106(5):788‐792.

Mora J, Wollner N. Primary epidural non‐Hodgkin lymphoma: spinal cord compression syndrome as the initial form of presentation in childhood non‐Hodgkin lymphoma. Med Pediatr Oncol. 1999;32(2):102‐105.

Dho YS, Kim H, Wang KC, et al. Pediatric spinal epidural lymphoma presenting with compressive myelopathy: a distinct pattern of disease presentation. World Neurosurg. 2018;114:e689‐e697.

Jaffe ESHN, Stein H, Vardiman JW, eds. World Health Organization classification of tumours. Pathology and genetics. Tumours of hematopoietic and lymphoid Tissues. 3rd ed. IARC; 2001. Revised.

Swerdlow SH, Campo E, Harris NL, eds. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. IARC; 2008.

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, eds. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. IARC; 2017. Revised.

Murphy SB. Classification, staging and end results of treatment of childhood non‐Hodgkin's lymphomas: dissimilarities from lymphomas in adults. Semin Oncol. 1980;7(3):332‐339.

Rosolen A, Perkins SL, Pinkerton CR, et al. Revised international pediatric non‐Hodgkin lymphoma staging system. J Clin Oncol. 2015;33(18):2112‐2118.

Reiter A, Schrappe M, Tiemann M, et al. Improved treatment results in childhood B‐cell neoplasms with tailored intensification of therapy: a report of the Berlin‐Frankfurt‐Munster Group Trial NHL‐BFM 90. Blood. 1999;94(10):3294‐3306.

Reiter A, Schrappe M, Ludwig WD, et al. Intensive ALL‐type therapy without local radiotherapy provides a 90% event‐free survival for children with T‐cell lymphoblastic lymphoma: a BFM group report. Blood. 2000;95(2):416‐421.

Seidemann K, Tiemann M, Schrappe M, et al. Short‐pulse B‐non‐Hodgkin lymphoma‐type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin‐Frankfurt‐Munster group trial NHL‐BFM 90. Blood. 2001;97(12):3699‐3706.

Woessmann W, Seidemann K, Mann G, et al. The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B‐cell neoplasms: a report of the BFM group study NHL‐BFM95. Blood. 2005;105(3):948‐958.

Burkhardt B, Woessmann W, Zimmermann M, et al. Impact of cranial radiotherapy on central nervous system prophylaxis in children and adolescents with central nervous system‐negative stage III or IV lymphoblastic lymphoma. J Clin Oncol. 2006;24(3):491‐499.

Landmann E, Burkhardt B, Zimmermann M, et al. Results and conclusions of the European Intergroup EURO‐LB02 trial in children and adolescents with lymphoblastic lymphoma. Haematologica. 2017;102(12):2086‐2096.

Brugieres L, Le Deley MC, Rosolen A, et al. Impact of the methotrexate administration dose on the need for intrathecal treatment in children and adolescents with anaplastic large‐cell lymphoma: results of a randomized trial of the EICNHL group. J Clin Oncol. 2009;27(6):897‐903.

Mussolin L, Le Deley MC, Carraro E, et al. Prognostic factors in childhood anaplastic large cell lymphoma: long term results of the international ALCL99 trial. Cancers (Basel). 2020;12(10):2747.

Meinhardt A, Burkhardt B, Zimmermann M, et al. Phase II window study on rituximab in newly diagnosed pediatric mature B‐cell non‐Hodgkin's lymphoma and Burkitt leukemia. J Clin Oncol. 2010;28(19):3115‐3121.

Kaplan EL, Meier P. Nonparametric‐estimation from incomplete observations. J Am Stat Assoc. 1958;53(282):457‐481.

Kalbfleisch JD, Prentice RL. The statistical analysis of failure time data. John Wiley & Sons; 1980.

Gray RJ. A class of K‐sample tests for comparing the cumulative incidence of a competing risk. Ann Stat. 1988;16(3):1141‐1154.

Burkhardt B, Klapper W, Wößmann W. Non‐Hodgkin‐lymphome. In: Charlotte Niemeyer AE, ed. Pädiatrische Hämatologie und Onkologie. 2nd ed. Springer Berlin; 2018.

Patte C, Auperin A, Michon J, et al. The Societe Francaise d'Oncologie Pediatrique LMB89 protocol: highly effective multiagent chemotherapy tailored to the tumor burden and initial response in 561 unselected children with B‐cell lymphomas and L3 leukemia. Blood. 2001;97(11):3370‐3379.

Patel JL, Smith LM, Anderson J, et al. The immunophenotype of T‐lymphoblastic lymphoma in children and adolescents: a Children's Oncology Group report. Br J Haematol. 2012;159(4):454‐461.

Ducassou S, Ferlay C, Bergeron C, et al. Clinical presentation, evolution, and prognosis of precursor B‐cell lymphoblastic lymphoma in trials LMT96, EORTC 58881, and EORTC 58951. Br J Haematol. 2011;152(4):441‐451.

Quraishi NA, Palliyil N, Hassanin MA, D'Aquino D, Shetaiwi A, Walker D. Malignant spinal cord compression in the paediatric population‐a systematic review, meta‐analysis. Eur Spine J. 2023;32(12):4306‐4313.

Kraal K, Blom T, van Noesel M, et al. Treatment and outcome of neuroblastoma with intraspinal extension: a systematic review. Pediatr Blood Cancer. 2017;64(8).

De Bernardi B, Pianca C, Pistamiglio P, et al. Neuroblastoma with symptomatic spinal cord compression at diagnosis: treatment and results with 76 cases. J Clin Oncol. 2001;19(1):183‐190.

Hoover M, Bowman LC, Crawford SE, et al. Long‐term outcome of patients with intraspinal neuroblastoma. Med Pediatr Oncol. 1999;32(5):353‐359.

Angelini P, Plantaz D, De Bernardi B, Passagia JG, Rubie H, Pastore G. Late sequelae of symptomatic epidural compression in children with localized neuroblastoma. Pediatr Blood Cancer. 2011;57(3):473‐480.

McGirt MJ, Chaichana KL, Atiba A, et al. Incidence of spinal deformity after resection of intramedullary spinal cord tumors in children who underwent laminectomy compared with laminoplasty. J Neurosurg Pediatr. 2008;1(1):57‐62.

Hayes FA, Thompson EI, Hvizdala E, O'Connor D, Green AA. Chemotherapy as an alternative to laminectomy and radiation in the management of epidural tumor. J Pediatr. 1984;104(2):221‐224.

McDonald AC, Nicoll JA, Rampling RP. Non‐Hodgkin's lymphoma presenting with spinal cord compression; a clinicopathological review of 25 cases. Eur J Cancer. 2000;36(2):207‐213.