Primary central nervous system lymphoma: variety of clinical manifestations and survival

A retrospective analysis of 23 non-immune compromised patients with primary central nervous system lymphoma (PCNSL) is reported. The patients consist of 14 males and 9 females and the median age is 50 years. Most patients presented with focal neurological deficits or symptoms of increased intracranial pressure (ICP) due to mass effects around the periventricular area. However, there were four cases of diffuse type, one case of intramedullary tumour, and four cases with rapid disease progression. All the patients underwent histological confirmation by craniotomy (16 cases) or stereotactic biopsy (7 cases). The radiation therapy started after histological diagnosis in all and 6 cases received additional chemotherapy. The median survival after diagnosis of overall patients was 23 months. Six patients are in disease free status at a mean of 35 months follow-up. The uni- and multi-variative risk analysis revealed five favourable factors on survival: (1) age less than 60 years (0.05 < p < 0.1); (2)pre-operative Karnofsky performance scale (KPS) > or = 70 (p < 0.05); (3) symptom duration over four weeks (p < 0.05); (4) radiation dosage > or = 5500 cGy (p < 0.05); (5)absence of malignant cells in cerebrospinal fluid (CSF) (p < 0.05). The most frequent site of recurrence was the local area as shown in seven cases out of 11 recurrent cases. But six patients had diffuse recurrence (4 cases), systemic recurrence (2 cases), and drop metastasis (1 case). Although the recurred PCNSL had rapid progression and a grave prognosis, aggressive treatment prolonged the postrecurrence survival time significantly. Our experience suggests that 1) there are various clinical and radiological presentations and progressions of the disease; 2) treatment method should be decided along with the clinical presentation, progression of disease, and prognostic factors; 3) early diagnosis and early detection of recurrence enable the start of definitive treatment when the medical condition is still satisfactory.