Absence of enhancement in a lesion does not preclude primary central nervous system T-cell lymphoma:A case report

BACKGROUND Primary central nervous system lymphoma(PCNSL)is a non-Hodgkin lymphoma that originates in the central nervous system(CNS)and is exclusively limited to the CNS.Although most PCNSLs are diffuse large B-cell lymphomas,primary CNS T-cell lymphomas(PCNSTLs)are rare.PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging(MRI).To the best of our knowledge,non-enhancing PCNSTL has not been reported previously.CASE SUMMARY A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks.Initial MRI showed asymmetric T2-hyperintense lesions within the brain.No enhancement was observed on the contrast-enhanced T1 image.The initial diagnosis was neuro-Behçet’s disease.Despite high-dose steroid therapy,no alterations in the lesions were identified on initial MRI.The patient’s symptoms deteriorated further.An MRI performed one month after the initial scan revealed an increased lesion extent.Subsequently,brain biopsy confirmed the diagnosis of PCNSTL.The patient underwent definitive combined chemoradiotherapy.However,the patient developed bacteremia and died of septic shock approximately three months after diagnosis.CONCLUSION The absence of enhancement in the lesion did not rule out PCNSTL.A biopsy approach is advisable for pathological confirmation.

High-dose methotrexate and zanubrutinib combination therapy for primary central nervous system lymphoma

In this editorial I comment on the article,published in the current issue of the World Journal of Clinical Oncology.Primary central nervous system lymphoma(PCNSL)is a disease of elderly and immunocompromised patients.The authors reported clinical results of 19 patients with PCNSL treated with zanubrutinib/high dose methotrexate(HD-MTX)until disease progression.They demonstrated that the combination of zanubrutinib with HD-MTX led to a marked clinical response and tolerability among these patients.They also observed that cerebrospinal fluid liquid biopsy to detect circulating tumor DNA may be a good option for evaluating treatment response and tumor burden in patients with PCNSL.PCNSL is a challenging disease for treatment as these patients present with different neurological states and comorbidities.Treatment has evolved over the years from whole brain radiotherapy to HD-MTX followed by autologous stem cell transplant.Gradually,treatment of patients with PCNSL is going to become individualized.

Clinical outcomes of newly diagnosed primary central nervous system lymphoma treated with zanubrutinib-based combination therapy

BACKGROUND High-dose methotrexate(HD-MTX)combined with other chemotherapeutic agents is an effective treatment for patients with newly diagnosed primary central nervous system lymphoma(PCNSL);however,some patients have adverse reactions.AIM To retrospectively evaluate disease outcomes and mutational profiles in newly diagnosed PCNSL patients treated with a zanubrutinib/HD-MTX combination regimen.METHODS Nineteen newly diagnosed PCNSL patients were treated with zanubrutinib/HDMTX until disease progression,intolerable toxicities,or physician/patientdirected withdrawal.Safety and efficacy were assessed per the CTCAE v5.0 and RECIST v1.1 criteria,respectively.The primary endpoint was the objective response rate(ORR),and the secondary endpoints were progression-free survival,overall survival(OS),and safety.RESULTS The median follow-up duration was 14.7 mo(range,3.9–30 mo).The ORR for all patients was 84.2%,and 2-year progression-free-and OS rates were 75.6%and 94.1%,respectively.All patients completed the induction phase,and nine patients underwent autologous stem cell transplantation as consolidation therapy,resulting in an ORR of 88.9%.Ten patients received zanubrutinib as maintenance therapy and achieved an ORR of 80%.All patients showed an acceptable safety profile.The sequencing results for cerebrospinal fluid(CSF)and tumor tissue showed that PIM1 mutations were the most frequent genetic alterations.Circulating tumor DNA was correlated with disease relapse and response.CONCLUSION Our empirical observations demonstrated that the combination of zanubrutinib with HD-MTX yielded a marked clinical response and tolerability among newly diagnosed PCNSL patients.Non-invasive CSF liquid biopsy profiling may be feasible for evaluating treatment response and tumor burden.

Distinctive magnetic resonance imaging features in primary central nervous system lymphoma:A case report

BACKGROUND Primary central nervous system lymphoma(PCNSL)is a rare malignant tumor originating from the lymphatic hematopoietic system.It exhibits unique imaging manifestations due to its biological characteristics.CASE SUMMARY Magnetic resonance imaging(MRI)with diffusion-weighted imaging(DWI),perfusion-weighted imaging(PWI),and magnetic resonance spectroscopy was performed.The imaging findings showed multiple space-occupying lesions with low signal on T1-weighted imaging,uniform high signal on T2-weighted imaging,and obvious enhancement on contrast-enhanced scans.DWI revealed diffusion restriction,PWI demonstrated hypoperfusion,and spectroscopy showed elevated choline peak and decreased N-acetylaspartic acid.The patient's condition significantly improved after hormone shock therapy.CONCLUSION This case highlights the distinctive imaging features of PCNSL and their importance in accurate diagnosis and management.

B-scan ultrasound and cytology of the vitreous in primary central nervous system lymphoma with vitreoretinal involvement

AIM: To evaluate the diagnostic value of B-scan ultrasound and explore the cytological characteristics of patients with vitreoretinal lymphoma(VRL) and primary central nervous system lymphoma(PCNSL).METHODS: The clinical data and pathologic specimens from patients with VRL diagnosed at the North Huashan Hospital from 2016 to 2017 were retrospectively reviewed. The patients were diagnosed by slit lamp ophthalmoscopy, B-scan ultrasound, cytology of the vitreous, which was obtained by vitrectomy, and cytokine measurements of interleukin(IL)-10 and IL-6.RESULTS: Twenty-six eyes(19.4%) out of 134 eyes of 67 patients(47 men and 20 women) with PCNSL were diagnosed with VRL by B-scan ultrasound, and 14 eyes(10.4%) were diagnosed by slit lamp ophthalmoscopy. Twenty-four eyes(17.9%) of 17 patients were confirmed as having VRL with cytology. No difference in the association between intracranial lesion location and ocular involvement was found. VRL patients had higher levels of vitreous IL-10 and IL-10/IL-6 when compared with macular hole cases, but the difference was not statistically significant.CONCLUSION: A total of 25.4% of the PCNSL patients had VRL, B-scan ultrasound examination had characteristic features and is recommended over slit lamp ophthalmoscopy for the screening diagnosis of PCNSL with intraocular involvement. Moreover, the cytological and immunohistochemical analyses performed after 25-gauge diagnostic vitrectomy were accurate diagnostic techniques.

Imaging of hemorrhagic primary central nervous system lymphoma: A case report

BACKGROUND A primary central nervous system lymphoma(PCNSL)presenting with massive hemorrhage is a rare occurrence that is difficult to distinguish from a high-grade glioblastoma.Comprehensive descriptions of the imaging characteristics of such tumors have not yet been reported.Herein,we reported a case of a PCNSL with massive hemorrhage by presenting the imaging features of computed tomography(CT)imaging and structural and perfusion magnetic resonance imaging(MRI).CASE SUMMARY A 48-year-old man presented with headache lasting for 10 d.CT of the brain showed a round,heterogeneous,high-density lesion with surrounding edema in the right temporal lobe.For further diagnosis,a series of MRI examinations of the brain were subsequently performed,and a hemorrhagic lesion with ring-like enhancement was determined.The whole lesion was relatively hypoperfused on arterial spin labeling images.Surgical resection of the lesion and histopathological examination confirmed that the lesion was a diffuse large B-cell lymphoma with massive hemorrhage.CONCLUSION PCNSLs with hemorrhage occur very rarely,and structural and perfusion MRI examinations are requested exceedingly rarely.This case provided insight into some characteristics of a hemorrhagic lymphoma on CT and MRI examinations.Perfusion MRI examination may be useful for the differential diagnosis of PCNSLs and other brain tumors.

Fotemustine-based therapy in combination with rituximab as a first-line induction chemotherapy followed by WBRT for newly diagnosed primary central nervous system lymphoma: a prospective phase II trial

Objective:This study aimed to evaluate the safety,efficacy,and feasibility of the rituximab,fotemustine,pemetrexed,and dexamethasone(R-FPD)regimen followed by whole-brain radiotherapy(WBRT)for patients with primary central nervous system lymphoma(PCNSL).Methods:A prospective,single-center phase II clinical trial was conducted.Patients with PCNSL newly diagnosed at the First Affiliated Hospital of Zhengzhou University between July 2018 and July 2020 were studied.The R-FPD regimen consisted of rituximab(375 mg/m2 i.v.on D0),fotemustine(100 mg/m2 i.v.on D1),pemetrexed(600 mg/m2 i.v.on D1),and dexamethasone(40 mg i.v.on D1-5).Patients 60 years or younger who showed a complete response(CR)were treated with 23.4 Gy of WBRT after the end of chemotherapy;those older than 60 years with CR were treated with a wait-and-see approach;and those who did not show CR after the 4th cycle of chemotherapy were given salvage WBRT 30 Gy+local tumor field irradiation up to 45 Gy,regardless of age.Results:A total of 30 patients were included.After 2 cycles,the objective response rate(ORR)was 96.5%(28/29,1 CR,27 PR,0 SD,and 1 PD).After 4 cycles,the ORR was 73.1%(19/26,11 CR,8 PR,4 SD,and 3 PD).After WBRT,the ORR was 90.9%(10/11,7 CR,3 PR,and 1 SD).The grade III and IV toxicity responses were mainly leukopenia(20.0%),thrombocytopenia(23.3%),and anemia(10.0%).Conclusions:Fotemustine-based therapy in combination with rituximab chemotherapy followed by WBRT can improve outcomes,providing ORR benefits and favorable tolerability in patients newly diagnosed with PCNSL.

Primary isolated central nervous system acute lymphoblastic leukemia with BCR-ABL1 rearrangement:A case report

BACKGROUND BCR-ABL1 fusion gene is associated with a poor prognosis and a high incidence in central nervous system(CNS)leukemia.CNS invasion which detected at the initial diagnosis is commonly with bone marrow infiltration.It is uncommon for the leukemia cells to be located primarily in the CNS without bone marrow involvement.CASE SUMMARY We here report the rare initial presentation of CNS-restricted BCR-ABL-positive acute lymphoblastic leukemia in a 30-year-old female patient who clinically manifested with leukemic meningitis,with no involvement in peripheral blood or bone marrow.Identification of abnormal phenotypes of blast cells,and BCR-ABL1 rearrangement in the cerebrospinal fluid alone established the diagnosis of primary CNS-isolated acute lymphocytic leukemia.The patient received a combination of intrathecal therapy and high-dose chemotherapy.But the benefits of the treatments were short-lived and she experienced recurrence.CONCLUSION Flow cytometry in combination with molecular genetic analysis improved diagnostic accuracy.New approaches that may enhance the efficacy of the existing therapies and cure CNS leukemia are required.

Primary central nervous system lymphoma presenting as a single choroidal lesion mimicking metastasis:A case report

BACKGROUND Primary choroidal lymphoma is usually an indolent B-cell lymphoma and rarely progresses to extraocular sites.Herein,we report a case of primary choroidal lymphoma diagnosed as diffuse large B-cell lymphoma(DLBL),which progressed to the brain parenchyma after 4 mo.CASE SUMMARY A 78-year-old man presented with diminution of vision in his right eye.A choroidal lesion suspected of metastatic lesion was observed in the right eye by ophthalmologic examination.To discover the primary tumor,imaging investigations were performed but no malignant lesion was detected.After 4 mo,the patient returned to the clinic presenting with neurological symptoms.Brain magnetic resonance imaging revealed an abnormal contrast-enhancing mass in the left cerebellum.A stereotactic biopsy was performed,and DLBL was confirmed.The patient received the high dose methotrexate-based chemotherapy and he achieved complete remission.CONCLUSION Primary choroidal lymphoma is usually known to have a benign clinical course without systemic involvement.We present a rare case of primary choroidal lymphoma diagnosed as DLBL that progressed to the brain parenchyma within months.

Diffuse Large B-Cell Lymphoma of the Central Nervous System. Immunophenotype, Clinicopathological Features and Differential Diagnosis

Background: Diffuse large B-cell lymphomas of the central nervous system (DLBCL CNS) represent less than 1% of all lymphomas and between 2% and 3% of all cerebral tumors. They occur in adults of 60 years of age or more. The objective of this work is to describe the clinical-pathological characteristics, the immunophenotype and the differential diagnosis. Clinical Case: From the files of the surgical pathology unit we found four cases of primary diffuse large B cell lymphoma of the central nervous system in a 6-year period. Three corresponded to women over 47 years of age and the other to a 42-year-old man. The time of evolution was between 2 and 4 months. The symptoms were headache, blurred vision, hemiparesis, and seizures. Localization was in the pineal region, the frontal, parietal regions, and the right thalamus. Morphologically, large lymphoid cells with a diffuse growth pattern and necrosis were observed. Immunohistochemical markers, such as CD 20 and bcl2 were positive, one was positive to CD3. Expression of bcl6 and CD 10 was positive in one case, and MUM-1 was positive in three cases. All the cases were negative for Epstein-Barr virus. Conclusions: The diffuse large-B cell lymphoma of the central nervous system is rare. Its average age of presentation is at 60 years or older. The localization is in the pineal, frontal, parietal and thalamic regions. Three cases were originated by activated B lymphocyte (MUM-1 expression) and other from the Germinal Center (GC) (CD 10 expression). The clinical course was bad. The four patients died shortly after the diagnosis.

Analysis of the genomic landscape of primary central nervous system lymphoma using whole-genome sequencing in Chinese patients

Primary central nervous system lymphoma(PCNSL)is an uncommon non-Hodgkin’s lymphoma with poor prognosis.This study aimed to depict the genetic landscape of Chinese PCNSLs.Whole-genome sequencing was performed on 68 newly diagnosed Chinese PCNSL samples,whose genomic characteristics and clinicopathologic features were also analyzed.Structural variations were identified in all patients with a mean of 349,which did not significantly influence prognosis.Copy loss occurred in all samples,while gains were detected in 77.9%of the samples.The high level of copy number variations was significantly associated with poor progression-free survival(PFS)and overall survival(OS).A total of 263 genes mutated in coding regions were identified,including 6 newly discovered genes(ROBO2,KMT2C,CXCR4,MYOM2,BCLAF1,and NRXN3)detected in≥10%of the cases.CD79B mutation was significantly associated with lower PFS,TMSB4X mutation and high expression of TMSB4X protein was associated with lower OS.A prognostic risk scoring system was also established for PCNSL,which included Karnofsky performance status and six mutated genes(BRD4,EBF1,BTG1,CCND3,STAG2,and TMSB4X).Collectively,this study comprehensively reveals the genomic landscape of newly diagnosed Chinese PCNSLs,thereby enriching the present understanding of the genetic mechanisms of PCNSL.

Bruton’s tyrosine kinase inhibitors in primary central nervous system lymphoma:New hopes on the horizon

In this editorial,we comment on the article by Wang et al.This manuscript explores the potential synergistic effects of combining zanubrutinib,a novel oral inhibitor of Bruton’s tyrosine kinase,with high-dose methotrexate(HD-MTX)as a therapeutic intervention for primary central nervous system lymphoma(PCNSL).The study involves a retrospective analysis of 19 PCNSL patients,highlighting clinicopathological characteristics,treatment outcomes,and genomic biomarkers.The results indicate the combination’s good tolerance and strong antitumor activity,with an 84.2%overall response rate.The authors emphasize the potential of zanubrutinib to modulate key genomic features of PCNSL,particularly mutations in myeloid differentiation primary response 88 and cluster of differentiation 79B.Furthermore,the study investigates the role of circulating tumor DNA in cerebrospinal fluid for disease surveillance and treatment response monitoring.In essence,the study provides valuable insights into the potential of combining zanubrutinib with HD-MTX as a frontline therapeutic regimen for PCNSL.The findings underscore the importance of exploring alternative treatment modalities and monitoring genomic and liquid biopsy markers to optimize patient outcomes.While the findings suggest promise,the study’s limitations should be considered,and further research is needed to establish the clinical relevance of this therapeutic approach for PCNSL.

Primary angiitis of the central nervous system： a case report

中央神经系统的主要 angiitis 是我们代表了的稀罕、困难的 entity.Here 有对在明确的 diagnosis.The 50岁的孩子男性前的类固醇的小反应的一个病人的临床、病理学的特征有一堂波动 的疾病功课因为超过 3 years.He 介绍了视觉混乱，抓住，认知缺陷，睡眠过度，不稳定的步法，语言困难，咽下困难，和 incontinence.Magnetic 回声成像出现了多重， supratentorial 和 infratentorial 反常信号，当时

A 5-year-old child presenting with tumor-like primary angiitis of the central nervous system

Introduction:Primary angiitis of the central nervous system(PACNS)is a vasculitis confined to the CNS.A small proportion of the lesions may present as a tumor-like mass,which is rarely seen in children.Case presentation:A 5-year-old girl was admitted to our hospital because of an intermittent headache.Brain imaging suggested a space-occupying lesion in the right cerebral hemisphere.The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy.Her condition improved after immunotherapy.Conclusion:Pediatricians should consider the possibility of PACNS when encountering intracranial tumor-like lesions.Early diagnosis of tumor-like PACNS and prompt immunotherapy could improve the long-term prognosis and avoid surgery.

Headache that Never Went Away

Central nervous system (CNS) vasculitis encompasses a broad range of diseases characterized by inflammation of blood vessels within the brain, spinal cord, and meninges. This review focuses on Primary Angiitis of the CNS (PACNS), a unique form affecting only the CNS without other organ involvement. PACNS primarily affects the small- and medium-sized arteries in the brain, leading to neurological symptoms. Early recognition and intervention are essential for optimal management. This case report of a 25-year-old male highlights the diagnostic challenges, where a multidisciplinary approach was necessary to diagnose PACNS, given initial negative results. The paper also explores the etiology and pathogenesis of PACNS and discusses the diagnostic criteria proposed by researchers. Medical professionals primarily base management strategies on induction and maintenance therapies, utilizing a combination of glucocorticoids and immunosuppressants as the standard treatment. However, the exact dosages and administration methods need further research due to the absence of randomized clinical trials. The review underscores the need for continued research to enhance diagnostic and treatment protocols, given the severity of PACNS.

Primary central nervous system lymphoma:status and advances in diagnosis,molecular pathogenesis,and treatment

Primary central nervous system lymphoma(PCNSL)is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes.This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients.The authors retrieved information from the PubMed database up to September 2019.The annual incidence of PCNSL increased over the last four decades.The prognosis of PCNSL has improved mainly due to the introduction and wide-spread use of high-dose methotrexate,which is now the backbone of all first-line treatment polychemotherapy regimens.Gene expression profiling and next-generation sequencing analyses have revealed mutations that induce activation of nuclear factor-kB,B cell antigen receptor,and Janus kinases/signal transducer and activator of transcription proteins signal pathways.Some novel agents are investigated in the treatment of relapsed PCNSL including immunotherapy and targeted therapy.In particular,lenalidomide and ibrutinib have demonstrated durable efficiency.Treatment of PCNSL has evolved in the last 40 years and survival outcomes have improved in most patient groups,but there is still room to improve outcome by optimizing current chemotherapy and novel agents.

Clinical utility of cytokine analysis in the diagnosis and efficacy monitoring of vitreoretinal lymphoma

AIM:To investigate the value of cytokine analysis in aqueous humor(AH)for discriminating vitreoretinal lymphoma(VRL)from uveitis and for evaluating the efficacy of intravitreal methotrexate(MTX)injections.METHODS:This retrospective study was done on 28 VRL patients between 2013 and 2019.AH interleukin(IL)-10,IL-6,IL-8,vascular endothelial growth factor(VEGF),and vascular cell adhesion molecules(VCAM)were measured in 28 VRL patients and 38 uveitis patients.As to the respective examinations for distinguishing VRL from uveitis,the diagnostic accuracy was evaluated by receiver operating characteristic(ROC)curve analysis.The response to treatment was monitored by observing changes in bestcorrected visual acuity(BCVA),ocular manifestation,and AH cytokine levels in 21 patients with VRL who had undergo multiple intravitreal injections of MTX.RESULTS:Compared with uveitis patients,VRL patients had higher IL-10 level(P<0.001)and IL-10/IL-6 ratio(P<0.001),whereas patients with uveitis had significantly higher IL-6 level than those with VRL(P=0.003).An ROC analysis was used to identify the diagnostic threshold values for VRL,and it was found that optimal sensitivity and specificity improved to 94.1%and 100%,respectively,for IL-10/IL-6>1.55 and 88.2%and 81.1%,respectively,for IL-10>76.7 pg/m L.In 21 patients who had undergo repeated injections,improvements in BCVA,clinical remission of VRL and continuous decrease in cytokine levels over time were observed.In those patients,the BCVA correlated with the aqueous levels of IL-10 and IL-6 during the course of disease treatment.CONCLUSION:The combination of the aqueous cytokine profiles can be instrumental for conventional diagnostic methods and for progression monitoring and treatment response.

Primary central nervous system lymphoblastic B cell lymphoma located at cerebellum in a child: A case report and literature review

Introduction:Primary central nervous system lymphoma(PCNSL)is extremely rare in pediatric population.We reported a case of PCNSL in a 3-year-old girl and reviewed the literature in the past three decades.Case presentation:A 3-year-old girl presented with gait disturbance.A contrast-enhanced magnetic resonance image of the brain showed a solitary bulky mass in the left cerebellar hemisphere,hydrocephalus and cerebellar tonsillar hernia.Surgical resection was performed and the patient was diagnosed with primary central nervous system lymphoblastic B cell lymphoma.Then the patient received regular chemotherapy,including 6 cycles of chemotherapy containing high-dose methotrexate(HD-MTX).The patient remains alive 15 months after the diagnosis with no evidence of active disease,but suffered twice chronic subdural hematoma,which was treated by burr hole drainage.Conclusion:Lymphoblastic B cell lymphoma is a rare histologic subtype of pediatric PCNSL.Chemotherapy containing HD-MTX remains the most effective treatment.The patient should avoid head impact after surgical resection of the tumor to prevent chronic subdural hematoma.

Disseminated soft tissue diffuse large B-cell lymphoma involving multiple abdominal wall muscles:A case report

BACKGROUND Diffuse large B-cell lymphoma(DLBCL)is the most common subtype of non�Hodgkin lymphoma,and patients with DLBCL typically present rapidly growing masses.Lymphoma involving muscle is rare and accounts for only 5%;furthermore,multiple muscles and soft tissue involvement of DLBCL is unusual.Due to unusual clinical manifestation,accurate diagnosis could be delayed.CASE SUMMARY A 61-year-old man complained of swelling,pain and erythematous changes in the lower abdomen.Initially,soft tissue infection was suspected,however,skin lesion did not respond to antibiotics.18Fluoro-2-deoxy-D-glucose(18F-FDG)positron emission tomography-computed tomography demonstrated FDG uptake not only in the skin and subcutaneous tissue of the abdomen but also in the abdominal wall muscles,peritoneum,perineum,penis and testis.DLBCL was confirmed by biopsy of the abdominal wall muscle and subcutaneous tissue.After intensive treatment including chemotherapy with rituximab,cyclophosphamide,doxorubicin,vincristine and prednisolone,central nervous system prophylaxis(intrathecal injection of methotrexate,cytarabine and hydrocortisone)and orchiectomy,he underwent peripheral blood stem cell mobilization for an autologous hematopoietic stem cell transplantation.Despite intensive treatment,the disease progressed rapidly and the patient showed poor outcome(overall survival,9 mo;disease free survival,3 mo).CONCLUSION The first clinical manifestation of soft tissue DLBCL involving multiple muscles was similar to the infection of the soft tissue.

Presenting clinical features of patients with vitreoretinal lymphoma

AIM: To assess the presenting clinical features, time from presentation to diagnosis and association with central nervous system(CNS) lymphoma in patients with vitreoretinal lymphoma.METHODS: Retrospective case series of patients diagnosed with vitreoretinal lymphoma between 2009 and 2011 at a single center.RESULTS: Fifteen eyes in 9 patients were included. Common presenting ocular symptoms included blurred vision(78%) and worsening floaters(44%) with an average symptom duration prior to presentation of 88.4 d(range 7-365 d). Common ophthalmic exam findings were vitreous haze(89%) and subretinal lesions(56%). The average time from presentation to diagnosis was 56.3 d(range 16-180 d). All patients were diagnosedwith large B-cell lymphoma according to pathology results. Lymphoma was restricted to the eye in 33%, while 67% of patients had CNS involvement. Of the patients with secondary vitreoretinal lymphoma, 67% initially presented with CNS lymphoma while 33% initially presented with vitreoretinal lymphoma. Of the patients with CNS involvement, memory loss(67%) was the most common presenting symptom.CONCLUSION: Vitreoretinal lymphoma most commonly presents with symptoms of blurred vision and/or worsening floaters and vitreous haze on exam. The average time from presentation to diagnosis may be decreasing with increased awareness among clinicians.