Anaplastic large cell lymphoma (ALCL) is a rare variety of non-Hodgkin lymphoma that primarily affects children and young adults and is caused by the fusion of the nucleophosmin 1 and ALK genes, which turns on several signalling pathways. Despite the fact that ALCL frequently affects lymph nodes and extranodal sites, involvement of the central nervous system (CNS) is quite uncommon, with symptoms like pain and fever. The spinal localization may result in neurological impairments. T1WI hypointense lesions with T2WI hyperintensity can be observed on MR images, while bone sequestra can be seen in CT scans. The gold standard for diagnosis is still histology, though. Based on distinctive morphological characteristics, immunohistochemical staining can differentiate ALK-positive ALCL from other ALCLs. The prognosis for ALK-positive ALCL is typically better than ALK-negative ALCL, and treatment options include surgery followed by chemotherapy. ALK inhibitors and CAR-T cell therapy are 2 newly developed targeted treatments that have the potential to improve results. This case report illustrates the rarity of CNS ALCL and the difficulties in diagnosis and treatment by describing a 16-year-old female patient with ALCL affecting the thoracic spine and spreading to the mediastinum and surrounding tissue.
Keywords: ALK-Positive ALCL; Nucleophosmin 1-ALK Fusion Protein; Root Involvement; Thoracic Spine.
© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.