P14.54 Primary central nervous system lymphoma of the spinal cord: a LOC network cohort study

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Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) mainly affects the brain (>90% of the cases), Very little data can be found in the literature on PCNSL with spinal cord localization. MATERIAL AND METHODS We present a retrospective study based on the French LOC network database. We selected adult immunocompetentpatients, with a histological or cytological diagnosis of PCNSL, and a spinal cord localization at initial diagnosis. RESULTS Of the 2043 PCNSLof the LOC database newly diagnosed since 2011, 14 patients (9 men, median age 68, median Karnofsky performance status 50%)met the selection criteria. The median diagnostic delay was 82 days (min 15-max 1080) compared to 35 days in primary cerebral lymphomas. At diagnosis, walking was impossible in 7/14 patients and 5/14 had indwelling urinary catheter. On MRI, 100% had enlargement of the spinal cord with homogeneous contrast enhancement in 13/14 cases. Spinal cord lesions were unique in 9/14 patients and multiples in 5/14 patients. CSF IL10 level was increased in 6/7 patients. Brain lesions were found in 9/14 patients, located in the posterior fossa in 5/9 cases. The diagnosis was made either on a brain biopsy (N=6), a spinal cord biopsy or surgery (N=5) or the cytologic analysis of the CSF (N=3).4/5 patients had neurological sequel after spinal cord biopsy or surgery. All the patients were treated by high-dose methotrexate-based chemotherapy, followed by spinal cord irradiation (N=1) or autograft (N=2). There was an overall response rate of 71% (complete response in 8/14). 8/14 patients relapsed, 5 in the brain, 2 in the spinal cord, and 1 both in the spinal cord and in the brain. 2-year PFS and OS were 45% and 64%, respectively. Among the long-term responders, 50% remained in wheel chair, while only 10% could walk normally. CONCLUSION Considering the high risk of a spinal cord biopsy,the rarity of the disease, as well as the numerous differential diagnoses, the diagnosis of spinal cord lymphoma is difficult. Searching for other lymphomatous locations or assaying CSF IL10 may be helpful in this disease where delay in diagnosis is often prolonged et can cause irreversible handicap.