Dg. Kim et al., PRIMARY CENTRAL-NERVOUS-SYSTEM LYMPHOMA - VARIETY OF CLINICAL MANIFESTATIONS AND SURVIVAL, Acta neurochirurgica, 138(3), 1996, pp. 280-289
A retrospective analysis of 23 non-immune compromised patients with pr
imary central nervous system lymphoma (PCNSL) is reported. The patient
s consist of 14 males and 9 females and the median age is 50 years. Mo
st patients presented with focal neurological deficits or symptoms of
increased intracranial pressure (ICP) due to mass effects around the p
eriventricular area. However, there were four cases of diffuse type, o
ne case of intramedullary tumour, and four cases with rapid disease pr
ogression. All the patients underwent histological confirmation by cra
niotomy (16 cases) or stereotactic biopsy (7 cases). The radiation the
rapy started after histological diagnosis in all and 6 cases received
additional chemotherapy. The median survival after diagnosis of overal
l patients was 23 months. Six patients are in disease free status al a
mean of 35 months follow-up. The uni- and multi-variative risk analys
is revealed five favourable factors on survival: (1) age less than 60
years (0.05 < p < 0.1); (2) pre-operative Karnofsky performance scale
(KPS) greater than or equal to 70 (p < 0.05); (3) symptom duration ove
r four weeks (p < 0.05); (4) radiation dosage greater than or equal to
5500 cGy (p<0.05); (5) absence of malignant cells in cerebrospinal fl
uid (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 patie
nts had diffuse recurrence (4 cases), systemic recurrence (2 cases), a
nd drop metastasis (1 case). Although the recurred PCNSL had rapid pro
gression and a grave prognosis, aggressive treatment prolonged the pos
trecurrence survival time significantly. Our experience suggests that
1) there are various clinical and radiological presentations and progr
essions of the disease; 2) treatment method should be decided along wi
th the clinical presentation, progression of disease, and prognostic f
actors; 3) early diagnosis and early detection of recurrence enable th
e start of definitive treatment when the medical condition is still sa
tisfactory.