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The outcome of the patients with primary diffuse large B cell lymphoma of central nervous system (PCNSL) still remains poor. The prognostic value of available prognostic scores need to be validated as well as the surgical approach since the improvements made in the field of neurosurgery.

The aim of this study was to evaluate treatment approach and the validity of the International Extranodal Lymphoma Study Group (IELSG) score of the patients with PCNSL.

This study involves 30 patients (13males/17females) with a median age of 53.5 years (range 29-72 years). The patients presented usually with more than two different symptoms which mostly reflected tumor location, and were as following: headache (13 patients), hemiparesis (13 patients), neuropsychiatric (11 patients), dysarthria (8 patients), nausea and vomiting (5 patients), visual symptoms and vertigo (5 patients), and other symptoms occasionally. Five patients (16.7%) had B symptoms, 5 patients (16.7%) had bone marrow infiltration and 13 patients (43.3%) had deep tumor location. According to the Eastern Cooperative Group performance status (ECOG), 9 patients (30%) had ECOG 1–2 and 21 patients (70%) had ECOG 3–4. Regarding the International Prognostic Index (IPI), low score was present in 44.4% of patients, low intermediate in 26%, high-intermediate in 22.2% and 7.4% of patients had high IPI score. The prevalence of the IELSG score was as following: low score had 17.4% of patients, intermediate had 65.2% and 17.4% had high IELSG score. Treatment options included total tumor resection in 13 patients (43.3%), partial tumor resection in 10 patients (33.3%) and biopsy only in 7 patients (23.3%). A customized de Angelis protocol based on high doze methotrexate with/without whole brain irradiation was applied in all patients.

The median overall survival (OS) was 41 months. Median event free survival was 36 months. Overall treatment response was achieved in 22 patients (73.4%), 8 patients (26.6%) had primary resistant disease with lethal outcome within 5 months. Statistical analyzes showed that the patients with ECOG≥3 had worst prognosis than the patients with ECOG 1–2 (Log Rank χ²11.722, p?0.01). Median OS in the group of patients with ECOG 1-2 was 62 months and 23 months in the group of patients with ECOG 3-4 (95% CI 3.118-42.882). The IPI didn`t show prognostic significance in the patients with PCNSL (Log Rank χ² 0.274, p=0.601), however the IELSG score significantly predicted survival in these patients (Log Rank χ² 12.755, p?0.01). The patients who underwent an open surgery with total tumor reduction had significantly longer OS (median 62 months) in comparison to the patients who had partial tumor reduction or biopsy only (Log Rank χ² 5.692, p=0.017) and whose median OS was 23 months (95% CI 1.634-44.366).

Despite that total tumor resection is not standard procedure in the patients with PCNSL, according to our data, it should be considered in patients with good performance status and surgically accessible lesions for resection. The IELSG score should be used as prognostic index in patients with PCNSL.