Abstract BACKGROUND AND OBJECTIVE Primary CNS lymphoma accounts for three percent of all newly diagnosed CNS tumours and 1-3% of all non-Hodgkin’s lymphomas. The oncologic origin of primary intramedullary lymphoma of the spinal cord (PILSC) is an enigma. We aim to determine the clinical manifestations, disease course and management of PILSC. MATERIALS AND METHODS A comprehensive literature search using PubMed Central and Google Scholar, was done, retrieving 17 case reports and two case series of 21 patients with PILSC and included using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, published between 2000 and 2023. RESULTS About 90.48% patients inflicted with PILSC were adult, with male predominance, 61.90%. The most common complaint at presentation was neurological deficit of motor type in 32.14% patients. MRI proved to be helpful in 76.19% cases, with spinal cord enlargement and high signal intensity in 19.04% cases. Adjuvant chemotherapy and radiotherapy was acquired by 52.38% and 38.09% patients. Non-Hodgkin type was reported in 90.47% cases and Hodgkin lymphoma was seen in 9.52% patients. Diffuse large B cell lymphoma was culprit in 41.10% cases. Five out of 21 cases had variable post-operative complications. The average follow-up was 16.16 months. CONCLUSION PILSC is a multifaceted neoplasm in all its clinico-pathological aspects of presentation. Although rare, PILSC can involve adult and paediatric populations with a clear male preponderance. The MRI findings are not reliable to make a preliminary diagnosis. Like other lymphomas, the role of chemoradiotherapy is clearly more significant than surgical intervention in improving patient prognosis. Histopathologically, it can be Non-Hodgkin or Hodgkin, B-cell type or T-cell type and has a wide array of immunohistochemistry positivity.
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