Purpose: Primary central nervous system lymphoma (PCNSL) mainly affects the brain (>90% cases); there are very little data pertaining to PCNSL involving the spinal cord.
Methods: We retrospectively selected from the French LOC network database adult immunocompetent patients diagnosed with PCNSL involving the spinal cord between 2011 and 2022.
Results: Of the 2043 patients records retrieved from the database, 16 patients (median age: 62.5 years, median Karnofsky performance status: 40) satisfied our study's selection criteria. The median diagnostic delay was 97 days. Upon diagnosis, 10 patients could not walk, and seven had an indwelling urinary catheter. All of the patients had undergone MRI that revealed expansive lesion(s) with homogeneous contrast enhancement. Brain lesions were found in nine patients and CSF IL-10 was increased in 9 of 10 patients. The diagnosis of PCNSL was made based on brain biopsy (n=6), spinal cord biopsy or surgery (n=6) or cytologic analysis of CSF (n=4). All the patients were treated with high-dose methotrexate-based chemotherapy, followed by spinal cord irradiation (n=1) or autograft (n=4). The median PFS and OS were 29 and 51 months, respectively. Among the responders, 33% remained in a wheelchair, and only 25% regained the ability to walk without assistance.
Conclusion: Considering the high risks associated with a spinal cord biopsy, the rarity of the disease and the lack of specificity of its clinicoradiological presentation, the diagnosis of spinal cord lymphoma is often delayed. Searching for other lymphomatous locations or assaying CSF IL-10 may be helpful in this disease, which can cause irreversible handicap.
Keywords: Intramedullary lymphoma; Primary central nervous system lymphoma; Spinal cord lymphoma.
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