Primary central nervous system lymphoma

Primary central nervous system lymphoma is a rare subtype of extranodal non-Hodgkin lymphoma that accounts for 4% of central nervous system tumours.

Retrospective review of 24 patients diagnosed with primary central nervous system lymphoma between 1990 and 2010. All patients were diagnosed using magnetic resonance imaging and the diagnosis was confirmed surgically.

Of the 24 patients analysed, all except 4 were immunocompetent. Median age at diagnosis was 59.3 years (range 13-79) and the sex ratio (male to female) was 1:1.1. Cognitive decline (in 33.4%) and headache (in 25%) were the most common complaints. Diagnosis was performed In 13 cases (54%) following craniotomy and in the other 11 cases (46%) after stereotactic biopsy. Breakdown by pathology was as follows: 22 cases of B-cell lymphoma (91.6%), 1 case of anaplastic large-cell lymphoma, and 1 case of T-cell lymphoma. Mean survival time was 12.8 months with an overall 1-year survival rate of 37.5%.

Primary central nervous system lymphoma often presents in the sixth decade with cognitive decline, headache, and focal neurological deficits. A single intracranial lesion was present in 75% of the patients (18 cases), and the remaining 25% (6 cases) had between 2 and 4 lesions. Preoperative clinical status was the most important factor determining prognosis.