Primary intraocular lymphoma: an International Primary Central Nervous System Lymphoma Collaborative Group Report

Abstract Advanced molecular and pathophysiologic characterization of Primary Central Nervous System Lymphoma (PCNSL) has revealed insights into promising targeted therapeutic approaches. Medical imaging plays a fundamental role in PCNSL diagnosis, staging, and response assessment. Institutional imaging variation and inconsistent clinical trial reporting diminishes the reliability and reproducibility of clinical response assessment. In this context, we aimed to: 1) critically review the use of advanced PET and MRI in the setting of PCNSL; 2) provide results from an international survey of clinical sites describing the current practices for routine and advanced imaging, and 3) provide biologically based recommendations from the International PCNSL Collaborative Group (IPCG) on adaptation of standardized imaging practices. The IPCG provides PET and MRI consensus recommendations built upon previous recommendations for standardized brain tumor imaging protocols (BTIP) in primary and metastatic disease. A biologically integrated approach is provided to addresses the unique challenges associated with the imaging assessment of PCNSL. Detailed imaging parameters facilitate the adoption of these recommendations by researchers and clinicians. To enhance clinical feasibility, we have developed both “ideal” and “minimum standard” protocols at 3T and 1.5T MR systems that will facilitate widespread adoption.

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P14.64 Primary Central Nervous System Lymphoma (PCNSL) with HD- MTX based chemotherapy: tolerability and results

Neuro-Oncology ◽

10.1093/neuonc/noz126.299 ◽

2019 ◽

Vol 21 (Supplement_3) ◽

pp. iii82-iii82

Author(s):

A Silvani ◽

E Anghileri ◽

G Simonetti ◽

M Eoli ◽

G Finocchiaro ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Adverse Events ◽

Treatment Strategies ◽

Central Nervous System Lymphoma ◽

Response Assessment ◽

Collaborative Group ◽

Combination Regimen ◽

Group Response ◽

Radiological Data

Abstract BACKGROUND Primary Central nervous system (CNS) lymphomas (PCNSL) are extranodal, malignant non- Hodgkin lymphomas of the diff use large B-cell type, confined to CNS. It accounts for up to 1% of all lymphomas and about 3% of all CNS tumours. The incidence continues to rise in immuno- competent elderly patients.Although the prognosis of PCNSL remains poor, it has improved in the past two decades as a result of better treatment strategies. However, treatment recommendations still result mainly from retrospective series or single-arm phase 2 studies and a few three completed randomised trials. We described a series of patients with newly diagnosis of PCNSL treated with modified-schedula published by Omuro MATERIAL AND METHODS Patients was treated with HD-MTX, procarbazine, vincristine, received four 28-day cycles of chemotherapy, on schedule: HD-MTX (3·5 g/m²) days 1 and 15; Procarbazine (100 mg/m²) on days 1–7. Vincristine (1·4 mg/m²) on days 1 and 15. Toxicity was assessed with Common Terminology Criteria for Adverse Events version 4. The International PCNSL Collaborative Group Response Criteria were used for response assessment. Endpoints include overall survival (OS) and adverse events (AE). RESULTS 21 immunocompetent patients (12 females and 9 males) were retrospectively studied, among 2014 to 2019. Median age at diagnosis 61 years (range 41–76). Median KPS at diagnosis was 70 (range 40–100). Clinical and radiological data and treatment related toxicity were described CONCLUSION Present combination regimen is effective; however, AE incidence is high, and often induces schedule modification

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Primary Central Nervous System Lymphoma of T Cell Origin: A Descriptive Analysis of 45 Cases from the International PCNSL Collaborative Group.

Blood ◽

10.1182/blood.v104.11.1372.1372 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1372-1372

Author(s):

Tamara Shenkier ◽

Jean-Yves Blay ◽

Brian P. O’Neill ◽

Philip Poortmans ◽

Kristoph Janke ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

T Cell ◽

B Cell ◽

Group Performance ◽

Eastern Cooperative Oncology Group ◽

Central Nervous System Lymphoma ◽

Collaborative Group ◽

Serum Lactate Dehydrogenase ◽

Management Approach

Abstract To describe the demographic and tumor related characteristics and outcomes for patients with primary T-cell central nervous system lymphoma (TPCNSL). A retrospective series of patients with TPCNSL was compiled from twelve cancer centers and seven countries. This study involved 35 male and 10 female patients with a median age of 60 years (range 3–84). Twenty (44%) had Eastern Cooperative Oncology Group performance status (PS) of 0 or 1. Twenty six (58%) had involvement of a cerebral hemisphere and sixteen (36%) had lesions of deeper sites in the brain. Two patients had primary spinal cord lesions and one had meningeal disease only. Serum lactate dehydrogenase (LDH) was elevated in 7 of the 22 cases (32%) and cerebrospinal fluid (CSF) protein was elevated above normal in 19 of the 24 cases (79%) with available data. The median disease specific survival (DSS) for all patients was 25 months (95% confidence interval (CI) 11–38 months). The two and five-year DSS were 51 % (CI 35–66 %) and 17 % (CI 6–34 %) respectively. Univariate and multivariate analyses were conducted for the following factors: age (≤ 60 vs. > 60 years), PS (0 or 1 vs. 2, 3 or 4), involvement of deep structures of the central nervous system (no vs. yes), and methotrexate (MTX) use in the primary treatment (yes vs. no). Only PS and MTX use were significantly associated with better outcome with hazard ratios (HR) of 0.2 (CI 0.1–0.4) and 0.4 (CI 0.2–0.8) respectively. This is the largest series ever assembled of TPCNSL. The presentation and outcome appear similar to that of PCNSL of B cell origin. PS 0 or 1 and administration of MTX are associated with better survival. TPCNSL does not appear to require a different therapeutic management approach than B-cell PCNSL.

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International Primary Central Nervous System Lymphoma Collaborative Group (IPCG) Study on Low-Grade Primary Central Nervous System Lymphoma in Immunocompetent Patients.

Blood ◽

10.1182/blood.v106.11.3343.3343 ◽

2005 ◽

Vol 106 (11) ◽

pp. 3343-3343

Author(s):

Agnieszka Korfel ◽

Kristoph Jahnke ◽

Brian P. O’Neill ◽

Jean-Yves Blay ◽

Lauren Abrey ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Performance Status ◽

Eastern Cooperative Oncology Group ◽

Tumor Resection ◽

Central Nervous System Lymphoma ◽

Collaborative Group ◽

Low Grade ◽

Term Outcome ◽

Long Term Outcome

Abstract Low-grade primary central nervous system lymphoma is a very rare subtype of primary central nervous system lymphoma (PCNSL), for which almost no data is currently available. The purpose of this retrospective study was to characterize the clinical presentation, course and outcome of patients with low-grade PCNSL. Forty patients (18 male, 22 female) from 18 cancer centers in five countries were identified with a median age of 58 (range, 19–78) years and a median Eastern Cooperative Oncology Group (ECOG) performance status of 1 (range, 0–4). The mean time to diagnosis was 14.8 months (range, 0.25–84). Thirty-two patients (80%) had a B-cell and eight a T-cell lymphoma. Thirty-seven patients (92.5%) showed involvement of a cerebral hemisphere or deeper brain structures, while two evidenced only leptomeningeal involvement, and one patient had spinal cord disease. Treatment was performed in 39 patients: chemotherapy and radiotherapy in 15 (38%), radiotherapy alone in 12 (30%), chemotherapy alone in 10 (25%), and tumor resection alone in two. The median progression-free survival (PFS), disease-specific survival (DSS) and overall survival (OAS) were 61.5 (range, 0–204), 130 (range, 1–204), and 79 (range, 1–204) months, respectively. An age ≥60 years was associated with a shorter PFS (P = .009), DSS (P = .015) and OAS (P = .001) in multivariate analysis. Low-grade PCNSL differ from the high-grade subtype in pathological, clinical and radiological features. In this study, the long-term outcome was better as compared to the results obtained in PCNSL in general with age ≥60 years adversely affecting survival.

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Ocular manifestations and treatment of central nervous system lymphomas

Neurosurgical FOCUS ◽

10.3171/foc.2006.21.5.10 ◽

2006 ◽

Vol 21 (5) ◽

pp. 1-7 ◽

Cited By ~ 23

Author(s):

Kaan Gündüz ◽

Jose S. Pulido ◽

Colin A. McCannel ◽

Brian Patrick O'Neill

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Systemic Chemotherapy ◽

Central Nervous System Involvement ◽

Central Nervous System Lymphoma ◽

Tumor Formation ◽

Lymphoid Cells ◽

Current Status ◽

Intraocular Lymphoma ◽

High Dose

✓ Intraocular primary central nervous system lymphoma (PCNSL), also called primary intraocular lymphoma (PIOL), is a subset of PCNSL in which lymphoma cells invade the subretinal pigment epithelial space and vitreous cavity with or without central nervous system involvement at the time of ocular diagnosis. The frequency of this rare condition has increased over the past years in immunosuppressed as well as immunocompetent patients. The authors review the current status of PIOL and elaborate on their group's experience with its diagnosis and treatment. The incidence of PIOL is increasing. There is evidence that chronic antigenic stimulation may result in the development of PIOL. Recent advancements in the diagnosis of PIOL include better handling of vitreous specimens for cytological studies, immunocytological investigation for lymphoid cells, flow cytometry, cytokine evaluation, and molecular analysis. Because PIOL has a nonspecific presentation, the differential diagnosis should include infectious and noninfectious causes presenting with vitreitis and/or subepithelial infiltration as well as paraneoplastic syndromes including CRMP-5 optic neuropathies. Given that therapy is long-term and has significant systemic and ocular complications, tissue diagnosis is important. Treatment of PIOL may include systemic chemotherapy in which high-dose methotrexate-based regimens are used as well as intraocular injections of methotrexate and rituximab (anti-CD20 antibody). Cranial and ocular external-beam radiotherapy is being used less often today. Further studies are needed to prevent the tumor formation in terms of eliminating antigenic load and inhibiting B-cell chemokines as well as to determine the optimal local and systemic chemotherapy and immunotherapy options in the management of PIOL.

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