Background Primary central nervous system lymphoma (PCNSL) is a type of non-Hodgkin lymphoma which starts in the brain or spinal cord. PCNSL is more common in adults, typically in patients in their 50s and 60s, and its incidence has been increasing.
Purpose To analyse the neurological, radiological and clinical manifestations, and treatment and evolution of a series of patients diagnosed with PCNSL.
Material and methods Retrospective observational study of patients diagnosed with PCNSL from 2008 to 2014 in a second level hospital. All medical records were reviewed as well as all medical information from reference centres where some patients were transferred to receive their treatments.
Data collected: sex, age, lactate dehydrogenase level, CSF protein levels and global survival.
Results 10 patients were included in the study, the majority were male (70%) and mean age was 69.5 years. Initial clinical manifestations: dizziness and instability (40%), disorders of consciousness (20%), changes of behaviour (20%), cephalea (10%) and partial (focal) epilepsy seizure (10%).
All patients were immunocompromised. 4 patients presented elevated lactate dehydrogenase levels (500-1600 U/L) and another 4 presented high CSF protein levels (45-133 mg/dL) with normal cytological study. Neuroimaging studies showed unique tumoral lesions in 8 patients, with multicentric lesions in 2 cases. Tumoral biopsies were performed in 6 patients, spinal cord biopsy in 4 and extension study in 7 patients.
Principal treatments were: corticosteroids (dexamethasone, oral and intravenous administration; 100%), surgical intervention (20%), cytostatic treatment (high dose methotrexate, intravenous regimen, high dose methotrexate intravenous plus cytarabine regimen, and high dose methotrexate intravenous plus cytarabine plus carmustine regimen; 80%), radiotherapy (30%) and spinal cord transplantation (10%). 7 patients died during the study. Mean global survival was 9.1 months and survival of patients after surgical intervention was 22.5 months.
Conclusion PCNSL continues to be a malignancy with a poor prognosis in our work environment. Because the mainstay of treatment for many patients is high dose methotrexate intravenously, they must be educated carefully about the drugs to be avoided in the week prior to chemotherapy and about the fluid and intensive monitoring requirements of their inpatient stay.
No conflict of interest.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.