Affiliation:
1. Pediatric Hematology and Oncology and NHL‐BFM Study Centre University Medical Centre Hamburg‐Eppendorf Hamburg Germany
2. Department of Pediatric Hematology and Oncology Children's Hospital, Hannover Medical School Hannover Germany
3. Department of Pediatric Hematology and Oncology, St. Anna Children's Hospital Medical University of Vienna Vienna Austria
4. Pediatric Hematology and Oncology Charles University and University Hospital Motol Prague Czech Republic
5. Pediatric Hematology‐Oncology Unit, Division of Pediatrics, Department Woman‐Mother‐Child University Hospital of Lausanne and University of Lausanne Lausanne Switzerland
6. Pediatric Hematology and Oncology and NHL‐BFM Study Centre University Hospital Muenster Muenster Germany
7. St. Anna Children's Cancer Research Institute Vienna Austria
Abstract
AbstractBackgroundSpinal 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.MethodsWe 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.ResultsFifty‐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.Conclusion1.2% 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.
Funder
Deutsche Kinderkrebsstiftung