P14.54 Primary central nervous system lymphoma of the spinal cord: a LOC network cohort study

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii48-ii48
Author(s):  
N Valyraki ◽  
G Ahle ◽  
E Tabouret ◽  
R Houot ◽  
F Jardin ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Karnofsky Performance Status ◽  
Performance Status ◽  
Diagnostic Delay ◽  
Central Nervous System Lymphoma ◽  
High Dose ◽  
Delay In Diagnosis ◽  
The Brain

Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) mainly affects the brain (>90% of the cases), Very little data can be found in the literature on PCNSL with spinal cord localization. MATERIAL AND METHODS We present a retrospective study based on the French LOC network database. We selected adult immunocompetentpatients, with a histological or cytological diagnosis of PCNSL, and a spinal cord localization at initial diagnosis. RESULTS Of the 2043 PCNSLof the LOC database newly diagnosed since 2011, 14 patients (9 men, median age 68, median Karnofsky performance status 50%)met the selection criteria. The median diagnostic delay was 82 days (min 15-max 1080) compared to 35 days in primary cerebral lymphomas. At diagnosis, walking was impossible in 7/14 patients and 5/14 had indwelling urinary catheter. On MRI, 100% had enlargement of the spinal cord with homogeneous contrast enhancement in 13/14 cases. Spinal cord lesions were unique in 9/14 patients and multiples in 5/14 patients. CSF IL10 level was increased in 6/7 patients. Brain lesions were found in 9/14 patients, located in the posterior fossa in 5/9 cases. The diagnosis was made either on a brain biopsy (N=6), a spinal cord biopsy or surgery (N=5) or the cytologic analysis of the CSF (N=3).4/5 patients had neurological sequel after spinal cord biopsy or surgery. All the patients were treated by high-dose methotrexate-based chemotherapy, followed by spinal cord irradiation (N=1) or autograft (N=2). There was an overall response rate of 71% (complete response in 8/14). 8/14 patients relapsed, 5 in the brain, 2 in the spinal cord, and 1 both in the spinal cord and in the brain. 2-year PFS and OS were 45% and 64%, respectively. Among the long-term responders, 50% remained in wheel chair, while only 10% could walk normally. CONCLUSION Considering the high risk of a spinal cord biopsy,the rarity of the disease, as well as the numerous differential diagnoses, the diagnosis of spinal cord lymphoma is difficult. Searching for other lymphomatous locations or assaying CSF IL10 may be helpful in this disease where delay in diagnosis is often prolonged et can cause irreversible handicap.

scholarly journals NQPC-06 FUNCTIONAL OUTCOMES OF INITIAL TREATMENTS FOR PATIENTS WITH PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA ASSESSED BY ADL AND NEUROCOGNITIVE FUNCTION

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii30-ii30
Author(s):  
Mikiko Taku ◽  
Keiichi Kobayashi ◽  
Yuki Yamagishi ◽  
Kuniaki Saito ◽  
Daisuke Shimada ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Induction Therapy ◽  
Social Adjustment ◽  
Karnofsky Performance Status ◽  
Performance Status ◽  
Central Nervous System Lymphoma ◽  
Neurocognitive Function ◽  
Remission Induction ◽  
High Dose

Abstract BACKGROUNDS Primary central nervous system lymphoma (PCNSL) frequently causes severe damage of activities of daily living (ADL) and neurocognitive function (NCF) due to extensive brain infiltration, necessitating their appropriate assessment and measures even in clinical practice. Since few studies have focused on the changes in the level of ADL and NCF in the course of PCNSL treatment, we retrospectively analyzed the effect of initial treatment of PCNSL in view of ADL and NCF. METHODS Among 55 patients (13 male/9 female) with newly-diagnosed PCNSL treated in our institution from January 2014 to June 2019, 22 were evaluated with both ADL and NCF. Remission induction therapies consisted of high-dose methotrexate alone (two patients), R-MPV (rituximab, methotrexate, procarbazine, and vincristine)(17 patients), and R-MPV+radiaotherapy (three patients), according to the patients’ conditions. Rehabilitation staffs intervened from the beginning, providing specific exercises and periodically evaluating scores of Karnofsky Performance Status (KPS) and Mini Mental State Examination (MMSE). RESULTS Mean age was 68.4 yo (range 34 to 85). After induction therapies, there were 11 complete responses (CRs), eight partial responses (PRs), and three progressive diseases (PDs). Both KPS and MMSE scores improved after induction therapy, from median 70 (40–90) to 80 (50–90), and from 24 (0–30) to 27(0–30), respectively. Among three patients who underwent RT, MMSE declined in two (one CR/one PR). CONCLUSIONS Case-adjusted induction therapies resulted in significant radiographical responses, and the longitudinal evaluation of ADL and NCF by rehabilitation staffs could validate their maintenance or improvement over time through effective treatments and early rehabilitation intervention. However, three was difficulty in assessing patients with higher brain dysfunction such as aphasia and social adjustment disorder. Further study is needed to include more patients and to explore more appropriate evaluation batteries and timings during and after completion of induction therapy for PCNSL.

scholarly journals An overlooked cause of longitudinally extensive spinal cord lesions: Primary central nervous system lymphoma

2020 ◽  
Author(s):  
Meng Wang ◽  
Baochang Qi ◽  
Jinming Han ◽  
Chunjie Guo ◽  
Limei Qu ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Lymphoma ◽  
High Dose Chemotherapy ◽  
Lower Limbs ◽  
Whole Body ◽  
Sensory Loss ◽  
High Dose ◽  
Spinal Cord Lesions

Abstract Background: Primary central nervous system lymphoma (PCNSL ) is a rare and aggressive malignant tumor. It is easy to be misdiagnosed due to its low incidence and unspecific presentations in clinical practice. PCNSL mainly occurs intracranially in the brain while spinal cord is rarely involved. Case presentation: Here we report a 76-year-old woman who had a suspicious tumor history and presented retardant paralysis, bladder dysfunction and sensory loss of the lower limbs. Magnetic resonance imaging (MRI) of the thoracic spine disclosed longitudinally extensive lesions extending from thoracic 4 (T4) to lumbar 1 (L1) vertebral level with an enhanced nodular lesion noting at levels of T10 and T11 . In order to further identify the cause, the whole body 18 F-fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET)/computed tomography (CT) was performed and showed a hypermetabolic nodule corresponded to MRI enhancing lesions, which further suggesting the possibility of a tumor. The patient then underwent a surgical resection and spinal cord biopsy confirmed the diagnosis of non-Hodgkin's lymphoma (diffuse large B-cell type). The patient then received a high-dose chemotherapy based on methotrexate combined with Rituximab. Unfortunately, the symptoms of this patient have not been improved significantly after three rounds of chemotherapy. Conclusion: Our case indicates that PCNSL may also serve as a possible cause for longitudinally extensive spinal cord lesions, especially the patients who had a suspicious tumor history, MRI enhancing lesion s in the spinal cord corresponded to hypermetabolic nodules on 18 F-FDG- PET/CT at the same level.

Sequential High Dose Immuno-Chemotherapy Followed by Autologous Peripheral Blood Stem Cell Transplantation for Patients with Untreated Primary Central Nervous System Lymphoma - a Multicentre Study by the Collaborative PCNSL Study Group Freiburg

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 302-302 ◽  
Author(s):  
Gerald Illerhaus ◽  
Kristina Fritsch ◽  
Gerlinde Egerer ◽  
Monika Lamprecht ◽  
Nikolas von Bubnoff ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Stem Cell ◽  
Complete Remission ◽  
Performance Status ◽  
Central Nervous System Lymphoma ◽  
Infectious Complications ◽  
Induction Treatment ◽  
High Dose ◽  
Grade 3

Abstract Abstract 302 Background: Primary central nervous system lymphoma (PCNSL) is an aggressive non-Hodgkin′s lymphoma with poor prognosis. Addition of methotrexate (MTX) to whole brain radiotherapy (WBRT) has improved the prognosis of patients (pts) with PCNSL, but a significant proportion are still not cured. Preliminary reports suggested that dose-intensified chemotherapy (HDT) and autologous stem-cell transplantation (ASCT) are highly effective in the treatment of newly-diagnosed PCNSL in younger pts. To strengthen the evidence of this approach, we initiated a prospective multicenter phase II study with early HDT and ASCT to investigate efficacy, safety and survival. This trial is registered at ClinicalTrials.gov (NCT 00647049). Methods: Immunocompetent pts <65 years with untreated biopsy proven PCNSL were eligible. Induction treatment consisted of 4 repetitive cycles of MTX (8g/m2) and 2 cycles cytarabine (2×3g/m2) and thiotepa (TT, 40mg/m2). Rituximab (375mg/m2) was added by amendmend after 2 included patients; it was given on day −7 before induction treatment and before each chemotherapy cycle. After the 2nd cycle cytarabine/TT stem-cells were collected after mobilisation with rG-CSF. The HDT regimen included carmustine (400mg/m2) and TT (4×5mg/kgBW) prior ASCT. Primary endpoint was complete remission (CR) 30 days after ASCT. Secondary end-points were overall-survival (OS), duration of response and toxicity. Patients not in complete remission after HDT and ASCT received WBRT. Results: From 2007 to 2011 79 pts (44 female, 35 male) were enrolled from 18 German centers and evaluable for analysis (median age 55 years, range 20–66). Seventy eight pts had aggressive B-cell lymphomas and one T-cell-lymphoma. Median Karnofsky performance status at diagnosis was 90% (range 30–100). After induction treatment, 73 of 76 (96%) evaluable pts responded, (26,9% CR, 55,7 PR). Seventy-three pts (96%) received HDT and ASCT according to protocol. Six pts were treated off-protocol due to low performance status (n=1), progressive disease (n=1) and infectious complications (n=4). Regarding the primary endpoint, CR was achieved in 77% and partial remission (PR) in 14% of patients (overall response rate 91%) after HDT and ASCT. Ten pts in PR after HDT and ASCT received consolidating WBRT. After a median follow-up of 28.8 months (range 1–63 mo) 1 and 2 years OS was 92% and 87%, respectively. Myelotoxicity was the most frequent CTC grade 3–4 toxicity with grade 3–4 infections in 41/73 pts (56,2%) during the transplant-phase. Two patients had lethal infectious complications during induction treatment with cytarabine/TT, three further pts died after HDT and ASCT due to severe infection (n=1), renal failure (n=1) and pneumonitis (n=1). Further results will be presented. Conclusion: Sequential MTX-based immuno-chemotherapy followed by carmustine/TT containing HDT and ASCT is highly effective and feasible in younger patients. Treatment related toxicity is of concern and comparable to non-high-dose protocols. Further randomised trials to compare HDT with conventional CT are needed. Disclosures: Illerhaus: Riemser: Honoraria.

scholarly journals ML-16 First clinical experience of administration of Tirabrutinib for the patients with newly diagnosed primary central nervous system lymphoma

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi24-vi24
Author(s):  
Sho Onodera ◽  
Jiro Akimoto
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Performance Status ◽  
Central Nervous System Lymphoma ◽  
Clinical Implications ◽  
High Dose ◽  
Newly Diagnosed ◽  
Dose Methotrexate ◽  
Early Progression ◽  
Inhibitory Drug

Abstract Tirabrutinib (TIR), a Burton’s tyrosine kinase inhibitory drug, has been approved in Japan for treating relapsed/refractory primary central nervous system lymphoma (PCNSL). The authors recently encountered three patients with newly diagnosed refractory PCNSL using TIR. Three patients, 48, 78 and 88 years-old males, diagnosed with PCNSL by histologically verification were firstly treated with high dose Methotrexate based chemotherapy (HD-MTX) and/or radiotherapy, however these cases were refractory for these standard treatments, demonstrated early cerebrospinal fluid dissemination or accompanied with severe adverse event. The authors decided to administrate TIR to these patients with a full informed consent. TIR demonstrated dramatic reduction of the volume of tumor on MRI within one month after administration of TIR, and improved the patient’s performance status. However, one case demonstrated liver dysfunction and multiple brain abscess due to aspergillus infection, and one case demonstrated early progression of the tumor 49 days after starting TIR. Administration of TIR for the patients with newly diagnosed refractory PCNSL demonstrated a rapid and dramatic clinical response, and presented with several clinical implications for this complicated condition.

Significance of MYC rearrangement and chemotherapy type on survival outcomes of patients with central nervous system lymphoma.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 7548-7548
Author(s):  
Natalie Sophia Grover ◽  
Allison Mary Deal ◽  
Stephanie Mathews ◽  
Ashley Freeman ◽  
Christopher Dittus ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Statistical Significance ◽  
Performance Status ◽  
Central Nervous System Lymphoma ◽  
B Cell Lymphoma ◽  
Poor Performance Status ◽  
High Dose ◽  
Single Institution ◽  
Significant Difference

7548 Background: Central nervous system lymphoma (CNSL) has a poor prognosis and an optimal treatment regimen has not been established. Due to the rarity of this disease and frequently poor performance status at diagnosis, there have been few prospective therapeutic clinical trials in this patient population. We therefore performed a retrospective analysis of prognostic factors and treatment outcomes of patients with CNSL treated at a single institution. Methods: Pathology records were used to identify patients diagnosed with CNSL from 1/1/2005 to 9/1/2016 at the University of North Carolina Cancer Hospital. Information about demographics, disease characteristics, treatment, and outcomes was gathered from the electronic medical record. Overall (OS) and progression free survival (PFS) were estimated using the Kaplan-Meier method. Results: We identified 100 patients with CNSL. 49% had primary CNSL (PCNSL). 78% of cases were diffuse large B cell lymphoma. Out of 51 patients evaluated for MYC translocation by FISH, 13 were positive (3 PCNSL and 10 secondary CNSL). Out of 74 patients treated with chemotherapy, 51% received methotrexate (MTX), procarbazine, and vincristine (MPV), with or without rituximab, 28% were treated with other high dose MTX based regimens, with or without rituximab, and 20% received a non-MTX based regimen. There was no significant difference in OS between PCNSL and secondary CNSL (13.7 vs 7.9 months, p = 0.97). Patients with MYC translocation had a worse OS compared to those without MYC translocation (5.1 vs 29.5 months, p = 0.004). Patients treated with MPV had a longer PFS compared to those treated with other high dose MTX based regimens or those who were treated with a non-MTX based regimen (19.1 vs 10.9 vs 3.9 months, p = 0.05), but difference in OS did not reach statistical significance (29.5 vs 22.4 vs 10.6 months, p = 0.12). Conclusions: In this single institution analysis of CNSL, MYC translocation was associated with worse survival. MPV was associated with improved PFS compared to other chemotherapy regimens. Further prospective studies are needed comparing MPV to other MTX-based regimens in CNSL.

Outcomes of primary central nervous system lymphoma in the era of immunotherapy: Cleveland Clinic experience.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e13516-e13516
Author(s):  
Suresh Kumar Balasubramanian ◽  
Philipp Schmitt ◽  
Meena Sadaps ◽  
Vidhya Karivedu ◽  
Debra Kangisser ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Proportional Hazards ◽  
Performance Status ◽  
Brain Lesion ◽  
Cleveland Clinic ◽  
Central Nervous System Lymphoma ◽  
Cox Proportional Hazards ◽  
High Dose

e13516 Background: Primary central nervous system lymphoma (PCNSL), a form of extranodal non-Hodgkin lymphoma represents 3% of primary CNS tumors. It is aggressive but typically confined to the CNS. Despite improvements in the management of PCNSL, more than 50% of patients eventually relapse. There are limited data on PCNSL from larger cohort studies. Methods: With IRB approval, the Cleveland Clinic Neuro-Oncology Center database was used to identify patients treated between 2006-2015 for PCNSL. Overall survival (OS) from the diagnosis of PCNSL and progression free survival (PFS) were the primary and secondary end points respectively. Cox proportional hazards models were used for data analysis. Results: 86 PCNSL patients were included in the analysis. Only 5% (4/76) were HIV positive. The median age of diagnosis was 63 (range 15 - 86) and 50% were males. 88% of patients presented only with brain lesion, 8% only in eye and 4% had both brain and eye involvement. 15% of patients (12/81) had positive CSF findings. Treatment included: chemotherapy (CT) alone (39% of patients); chemoimmunotherapy (CIT) (32%); CIT with radiotherapy (RT) (13%); RT alone (11%); CT with RT (4%); and immunotherapy (IT) alone (1%). Among 23 patients (31%) who received RT upfront, 74% had WBRT (n = 17). The most common upfront therapy was high dose methotrexate (HD MTX) (44%), followed by HD MTX with rituximab (23%), RTOG 0227 (12%), RTOG 1114 (11%) and rest 14% included rituximab or temozolomide or other cytotoxic chemotherapy alone or in combinations. The most common relapse site was brain (72%), followed by eyes (12%) and spine (8%). The median follow-up was 26 months. At last follow up, 41% had died and 93% of which were PCNSL-related. The median PFS and OS were 17.7 months and 84 months, respectively. There was a trend towards superior PFS in upfront IT vs. no IT (20 vs. 14 months, p 0.08). Better performance status (KPS > 80 vs. < 80, HR 0.42 (p = 0.033)) and PFS ≥ 24 months compared to ≤ 24 months (p = 0.0012) were associated with improved OS. Conclusions: We report a large single institution cohort of PCNSL patients treated in the era of immunotherapy. In our cohort, better KPS (≥80) and PFS ≥ 24 months had improved OS. Upfront IT showed a trend towards improved PFS.

scholarly journals Consecutive single-institution case series of primary central nervous system lymphoma treated by R-MPV or high-dose methotrexate monotherapy

2020 ◽  
Vol 50 (9) ◽  
pp. 999-1008 ◽  
Author(s):  
Nobuyoshi Sasaki ◽  
Keiichi Kobayashi ◽  
Kuniaki Saito ◽  
Saki Shimizu ◽  
Kaori Suzuki ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Mini Mental State Examination ◽  
Karnofsky Performance Status ◽  
Performance Status ◽  
Central Nervous System Lymphoma ◽  
Whole Brain Radiotherapy ◽  
High Dose ◽  
High Dose Methotrexate ◽  
Brain Radiotherapy ◽  
Dose Methotrexate

Abstract Objective The optimal regimen for use of high dose-methotrexate-based chemotherapy in primary central nervous system lymphoma is still under debate. We conducted a retrospective study to evaluate the treatment outcome of a combination immunochemotherapy consisting of rituximab, methotrexate, procarbazine and vincristine followed by with or without whole brain radiotherapy and consolidation cytarabine, in comparison with high dose-methotrexate monotherapy followed by full dose whole brain radiotherapy. Methods Newly diagnosed primary central nervous system lymphoma patients treated with either rituximab, methotrexate, procarbazine and vincristine or high dose-methotrexate in Kyorin University Hospital were identified, and the response rates and survival were compared. Toxicities, post-treatment transition of Mini-Mental State Examination, Karnofsky performance status score, Fazekas scale and prognostic factors were analysed in the rituximab, methotrexate, procarbazine and vincristine group. Results Ninety-five patients treated with rituximab, methotrexate, procarbazine and vincristine (n = 39) or high dose-methotrexate (n = 56) were analysed. The complete response/complete response unconfirmed rate was significantly higher in the rituximab, methotrexate, procarbazine and vincristine group (74.4 vs. 15.4%, P &lt; 0.001). Accordingly, both median progression-free survival and overall survival were significantly longer in the rituximab, methotrexate, procarbazine and vincristine group (median progression-free survival: unreached vs. 14.75 months, P &lt; 0.001) (median overall survival: unreached vs. 63.15 months, P = 0.005). Although the rate of grade 3/4 hematologic toxicities was high both during rituximab, methotrexate, procarbazine and vincristine and consolidation cytarabine, the rate of grade 3/4 infections was low, and no treatment related deaths were observed. Deterioration in Karnofsky performance status or Mini-Mental State Examination was rare, except on disease recurrence. Although whole brain radiotherapy was associated with Fazekas scale deterioration, its association with Karnofsky performance status or Mini-Mental State Examination deterioration was not significant. Conclusions Rituximab, methotrexate, procarbazine and vincristine was apparently promising in comparison with high dose-methotrexate monotherapy with manageable toxicity in this retrospective study, and further investigation is warranted.

scholarly journals Risk of Death, Relapse or Progression, and Loss of Life Expectancy at Different Progression-Free Survival Milestones in Primary Central Nervous System Lymphoma

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1699-1699
Author(s):  
Jorne Lionel Biccler ◽  
Kerry J Savage ◽  
Peter de Nully Brown ◽  
Judit Jørgensen ◽  
Thomas Stauffer Larsen ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Performance Status ◽  
Central Nervous System Lymphoma ◽  
Progression Free Survival ◽  
High Dose ◽  
Ecog Performance Status ◽  
Free Survival ◽  
Risk Of Death ◽  
Background Population

Abstract Primary Central Nervous System Lymphoma (PCNSL) is a rare aggressive non-Hodgkin lymphoma involving exclusively the central nervous system (CNS). The majority of PCNSLs are diffuse large B-cell lymphomas (DLBCL), but treatment and prognosis differ from systemic DLBCL due to differences in biology and the difficulty of delivering effective therapies with high penetration across the blood-brain barrier (BBB). While PCNSL often responds to initial therapy, relapses are common even after achieving a complete remission. The aims of this study were to estimate the risk of death or relapse and the loss of life expectancy in PCNSL after primary treatment with high-dose methotrexate (HD-MTX) containing regimens. Outcomes were assessed at baseline and for patients reaching pre-defined milestones of progression-free survival (PFS). Data on PCNSL patients were extracted from the nationwide Danish lymphoma register. The inclusion criteria were I) histologically-proven DLBCL morphology, II) involvement restricted to parenchymal or leptomeningeal CNS involvement without ocular involvement, III) treatment protocols containing HD-MTX, and IV) diagnosis between 2000-2017. PFS was defined as the time from diagnosis until death, relapse/progression, or end-of-treatment response assessment for patients with stable or progressive disease at the response assessment. The five-year PFS event probability risk was estimated for all patients and conditional on patients reaching different PFS milestones. The five-year restricted loss of lifetime (5y-RLEL) was defined as the numeric difference in the number of days patients and individuals from a background population are expected to live in the following five year period. This was estimated for all patients and for subsets of patients free of PFS events after one (PFS1), two (PFS2), or three (PFS3) years. Additionally, the results were stratified according to gender, ECOG performance status 0-1/> 1, elevated LDH status, treatment with/without rituximab, and age at diagnosis ≤60/>60 years. The survival of an age- and gender-matched general population was calculated by using life tables from the Human Mortality Database. In total 253 patients were included in the analyses; 60% were male, median age at diagnosis was 66 (range 27 - 85), 46% had an ECOG performance status > 1, and 33% had elevated LDH levels. Consolidation therapy (radiotherapy and/or high-dose therapy with autologous stem cell transplantation) was used in 23% of patients and 36% received rituximab in first line. The median follow-up was 6.9 years (range 0.7 - 17.7), the 5-year overall survival was 35% (95% CI 29-42), and the five-year PFS was 28% (95% CI 22-34). Patients reaching PFS1 had a 51% (95% CI 41-61) probability of a PFS event in the following five years (Figure 1A). After the PFS1 milestone, the five-year probability of a PFS event did not change substantially (Figure 1A) and the event probability remained high even after three years of PFS. On average, the PCNSL patients lost 2.2 living years (95% CI 1.9 - 2.4) in the five years after first pathologic diagnosis of PCNSL (Figure 1B). At PFS1, the 5y-RLEL decreased to 1.0 years (95% CI 0.7 - 1.3) (Figure 1B). The achievement of later PFS milestones only led to minor additional decreases in 5y-RLEL (PFS3: 0.7 years [95% CI 0.3 - 1.1]) (Figure 1B). The 5y-RLEL estimates were substantially larger for patients with an ECOG performance status > 1 vs patients with an ECOG performance status ≤ 1 (Figure 1B). Outcome differences between risk factor defined subgroups decreased after PFS1 and later PFS milestones (Figure 1B). The outlook of PCNSL patients treated with HD-MTX-based therapy improves significantly given a progression-free survival of one year, after which baseline adverse risk factors lose prognostic impact over time. However, in contrast to systemic DLBCL, survival does not normalize to the background population even after several years without PFS event. By the time of the ASH, updated results that include patients from the population-based lymphoma database in British Columbia (Canada) will be presented. Disclosures No relevant conflicts of interest to declare.

scholarly journals Primary central nervous system lymphoma

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 379-385 ◽  
Author(s):  
Tracy T. Batchelor
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Hodgkin Lymphoma ◽  
Central Nervous System Lymphoma ◽  
The Body ◽  
Immunocompetent Host ◽  
Non Hodgkin Lymphoma ◽  
Organ Specific ◽  
The Brain

Abstract Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma (NHL) confined to the brain, leptomeninges, eyes, or spinal cord. The majority of PCNSL cases occur in the immunocompetent host, the focus of this review. The prognosis of PCNSL is inferior to that of other NHL subtypes including other organ-specific subtypes of extranodal NHL. The 5- and 10-year survival proportions for PCNSL are 29.3% and 21.6%, respectively. The diagnosis and management of PCNSL differs from that of other primary brain cancers and NHL in other parts of the body.

Primary central nervous system lymphoma: presentation, diagnosis, and staging

2006 ◽  
Vol 21 (5) ◽  
pp. 1-9 ◽  
Author(s):  
April F. Eichler ◽  
Tracy T. Batchelor
Keyword(s):  
Spinal Cord ◽  
Central Nervous System ◽  
Nervous System ◽  
Mr Imaging ◽  
Central Nervous System Lymphoma ◽  
Serum Lactate Dehydrogenase ◽  
Periventricular White Matter ◽  
Contrast Enhanced ◽  
The Brain ◽  
Immunocompetent Patients

✓ Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that affects the brain, spinal cord, leptomeninges, and eyes. The clinical presentation and neuroimaging appearance of PCNSL differ in immunocompetent patients and in those with acquired immunodeficiency syndrome (AIDS). A magnetic resonance (MR) image of the brain in immunocompetent patients with PCNSL typically demonstrates one or more homogeneously enhancing lesions located in the periventricular white matter, characteristically spanning the corpus callosum. In patients with AIDS, multiple ring-enhancing lesions are more common. After neuroimages raising the suspicion of PCNSL are obtained, a definitive diagnosis should be established in both immunocompetent and AIDS patients by performing pathological analysis of cerebrospinal fluid (CSF), vitreous fluid, or a biopsy specimen. Brain biopsy sampling remains the gold standard for PCNSL diagnosis in all patients, although the possibility of establishing routine, minimally invasive diagnostic procedures in which Epstein–Barr virus polymerase chain reaction (PCR) analysis of the CSF and nuclear imaging are used is currently under investigation in the population of patients with AIDS. At the time of diagnosis, the patient should undergo further evaluation, which should include a physical examination, ophthalmic evaluation with a slit-lamp examination, serum lactate dehydrogenase levels, human immunodeficiency virus testing, computed tomography scans of the chest/abdomen/pelvis, bone marrow biopsy sampling, contrast-enhanced brain MR imaging, and lumbar puncture (LP). Testicular ultrasonography studies should be considered in men. In patients who cannot undergo LP or in those with evidence of spinal cord dysfunction, contrast-enhanced MR imaging of the entire spine should be considered.

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