scholarly journals NIMG-62. ATYPICAL IMAGING CHARACTERISTICS OF PRIMARY CNS LYMPHOMA AT INITIAL PRESENTATION LEADS TO FREQUENT MISDIAGNOSIS AND DELAYS IN DIAGNOSIS

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi175-vi175
Author(s):  
Marissa Barbaro ◽  
Peter Pan ◽  
Kiran Thakur ◽  
Mary Welch
Keyword(s):  
Medical Center ◽  
Primary Cns Lymphoma ◽  
Brain Biopsy ◽  
Cns Lymphoma ◽  
Imaging Features ◽  
Columbia University ◽  
Imaging Characteristics ◽  
Increased Risk ◽  
Mri Scans ◽  
Delays In Diagnosis

Abstract BACKGROUND Primary CNS lymphoma (PCNSL) has protean appearances on magnetic resonance imaging (MRI) that may lead to delays in diagnosis. METHODS We retrospectively reviewed histologically-confirmed PCNSL at Columbia University Irving Medical Center (CUIMC, 2010–2019), and characterize imaging features on pre-treatment MRI scans. RESULTS 64 patients were analyzed. 61 of 64 (95%) presented with enhancement. 35 of 64 (55%) were multiply enhancing, and 26 of 64 (41%) were singly enhancing (of which 2 were dural-based). 3 of 64 (5%) were non-enhancing. 42 of 59 (71%) had diffusion restriction. 36 of 49 (73%) lacked susceptibility. 40 of 64 (63%) were periventricular. 28 of 64 (43%) had callosal involvement. In 14 of 54 (26%), lymphoma was either not included in the differential or specifically noted less likely in radiographic report – this radiographically misdiagnosed group was significantly more likely to be either non-enhancing or non-periventricular (p=0.026). Furthermore, radiographic misdiagnosis was associated with an increased risk of a more than 14-day delay from the initial MRI to the initial invasive study, either lumbar puncture or brain biopsy (p=0.04). Presentation with a single enhancing lesion, on the other hand, was associated with significantly faster time to diagnosis – median 4.6 days (IQR 3) vs 21.6 days (IQR 4.5) from initial MRI (p=0.04). CONCLUSION In PCNSL, imaging characteristics influence outcomes. While a classic single enhancing lesion is associated with rapid diagnosis, non-enhancing and non-periventricular disease are the most likely to be misdiagnosed and require a heightened index of suspicion to avoid delays to diagnosis.

scholarly journals PATH-60. PRIMARY CNS LYMPHOMA PRECEDED BY ACUTE DEMYELINATION

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi156-vi157
Author(s):  
Justin Low ◽  
Anne Buckley ◽  
Dina Randazzo
Keyword(s):  
Oral Prednisone ◽  
Primary Cns Lymphoma ◽  
Brain Biopsy ◽  
B Cell Lymphoma ◽  
High Grade Glioma ◽  
Acute Disseminated Encephalomyelitis ◽  
Cns Lymphoma ◽  
Central Demyelination ◽  
Initial Biopsy ◽  
Radiographic Improvement

Abstract Primary CNS lymphoma commonly presents with nonspecific encephalopathy or focal neurologic deficits. Magnetic resonance imaging typically shows a homogeneously enhancing mass with surrounding edema. The imaging differential diagnosis is broad, and includes high grade glioma and neuroinflammatory conditions. Definitive diagnosis therefore requires biopsy. Here we present a case of primary CNS lymphoma that was diagnosed as an acute demyelinating process on initial biopsy. A 68 year old female presented with gait instability and vertigo. MRI showed right cerebellar and right trigonal enhancing lesions. Biopsy revealed an acute demyelinating inflammatory process and she was diagnosed with acute disseminated encephalomyelitis. She was treated with intravenous methylprednisolone followed by oral prednisone with resulting clinical and radiographic improvement. She was re-admitted to hospital 4 months later with encephalopathy. Imaging showed a new enhancing mass in the pericallosal frontal lobes. Repeat brain biopsy showed diffuse large B-cell lymphoma. This case illustrates a highly unusual situation of biopsy-proven central demyelination preceding a primary CNS lymphoma diagnosis. It raises a number of etiopathological questions concerning the coexistence and potential causal relationships between demyelination and lymphoma. Additionally, it highlights the need for repeat biopsy if clinical and radiographic suspicion for lymphoma persists despite an alternative initial biopsy result.

Primary intraocular lymphoma (PIOL): An International Primary CNS Lymphoma Collaborative group report

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 1534-1534
Author(s):  
S. Grimm ◽  
J. Pulido ◽  
K. Jahnke ◽  
D. Schiff ◽  
A. Hall ◽  
...  
Keyword(s):  
Performance Status ◽  
Primary Cns Lymphoma ◽  
Local Therapy ◽  
Initial Therapy ◽  
Collaborative Group ◽  
Intraocular Lymphoma ◽  
Cns Lymphoma ◽  
Treatment Type ◽  
Increased Risk ◽  
Sites Of Relapse

1534 Background: PIOL is a hemopoietic tumor that arises in the retina, vitreous or optic nerve head, and carries a high risk of ocular and CNS relapse. The natural history and optimal management are unknown. Methods: A retrospective series of 81 patients with PIOL was assembled from 15 centers in 7 countries. Only patients with isolated ocular lymphoma were included; none had brain, spinal cord, or systemic lymphoma at diagnosis. Results: The median age at diagnosis was 65 (24–85). 58% were women. The median ECOG performance status was 0, and only three had a score > 1. The median latency from symptom onset to diagnosis was 6 months (0– 36). Slit lamp exam was positive in 51, negative in 6, and not reported in 24. Vitrectomy was positive in 72 and negative in 2. 6 had a positive choroidal or retinal biopsy and 1 had no ocular surgery. CSF cytology was positive in 10 (17%), negative in 48, and unknown in 23. 21 received local therapy at diagnosis: 6 intra-ocular methotrexate (400 ug), 14 ocular radiation (median 3600 cGy), and 1 both modalities. 52 received more extensive therapy including systemic chemotherapy alone in 20 and a combination of chemotherapy and radiotherapy in 32. 5 received no treatment and details are unknown in 3. 47 patients (58%) relapsed a median of 19 months (0.5–180) after initial therapy. Sites of relapse included brain 47%, eyes 30%, brain and eyes 15%, and systemic 8%. Patients treated with ocular therapy alone did not have an increased risk of failing in the brain (p = 0.6). Progression free survival (PFS) and overall survival (OS) were 29.6 and 57 months respectively and were unaffected by the choice of therapy. CNS disease was the cause of death in 19/33 (58%). Conclusions: In this series, treatment type did not affect sites of relapse, PFS or OS in patients with PIOL. To minimize toxicity, the best initial therapy should be limited to intraocular chemotherapy or focal radiotherapy. Prospective clinical trials are needed to improve our understanding and treatment of this disease. No significant financial relationships to disclose.

Primary CNS Lymphoma

2006 ◽  
Vol 24 (8) ◽  
pp. 1281-1288 ◽  
Author(s):  
Tracy Batchelor ◽  
Jay S. Loeffler
Keyword(s):  
Tumor Response ◽  
Negative Impact ◽  
Systemic Disease ◽  
Primary Cns Lymphoma ◽  
Response Rates ◽  
Therapeutic Benefit ◽  
Tumor Control ◽  
Cns Lymphoma ◽  
Imaging Features ◽  
Whole Brain Radiation

Primary CNS lymphoma (PCNSL), an uncommon form of extranodal non-Hodgkin's lymphoma (NHL), has increased in incidence during the last three decades and occurs in both immunocompromised and immunocompetent hosts. PCNSL in immunocompetent patients is associated with unique diagnostic, prognostic, and therapeutic issues, and the management of this malignancy is different from that of other forms of extranodal NHL. Characteristic imaging features should be suggestive of the diagnosis, avoidance of corticosteroids, if possible, and early neurosurgical consultation for stereotactic biopsy. Because PCNSL may involve the brain, CSF, and eyes, diagnostic evaluation should include assessment of all of these regions as well as screening for possible occult systemic disease. Resection provides no therapeutic benefit and should be reserved for the rare patient with neurologic deterioration due to brain herniation. Whole-brain radiation therapy (WBRT) alone is insufficient for durable tumor control and is associated with a high risk of neurotoxicity in patients older than age 60. Neurotoxicity typically is associated with significant cognitive, motor, and autonomic dysfunction, and has a negative impact on quality of life. Chemotherapy and WBRT together improve tumor response rates and survival compared with WBRT alone. Methotrexate-based multiagent chemotherapy without WBRT is associated with similar tumor response rates and survival compared with regimens that include WBRT, although controlled trials have not been performed. The risk of neurotoxicity is lower in patients treated with chemotherapy alone.

Progressive leukoencephalopathy caused by primary CNS lymphoma

2010 ◽  
Vol 63 (4) ◽  
pp. 359-361 ◽  
Author(s):  
Benjamin Matošević ◽  
Michael Knoflach ◽  
Martin Furtner ◽  
Thaddäus Gotwald ◽  
Hans Maier ◽  
...  
Keyword(s):  
Small Vessel Disease ◽  
State Of The Art ◽  
Correct Diagnosis ◽  
Primary Cns Lymphoma ◽  
Brain Biopsy ◽  
Cns Lymphoma ◽  
Clinical Routine ◽  
Non Invasive ◽  
Vessel Disease ◽  
Inflammatory Tissue

Prominent leukoaraiosis is common in the clinical routine setting. In addition to microatheroma and hypertensive small vessel disease (lipohyalinosis), a large number of rare but clinically relevant differential diagnoses have to be considered. A man in his 60s presented with left pontine infarction and subsequent rapidly deteriorating leukoaraiosis associated with dementia. Standard non-invasive examination did not enable the correct diagnosis to be obtained. A brain biopsy sample revealed a combination of diffuse infiltrating and intravascular large B cell central nervous system (CNS) lymphoma, which has not previously been described in literature. Despite immediate treatment with state of the art chemotherapy, the patient died 3 months after the onset of symptoms. Diffuse infiltrating and intravascular primary CNS lymphoma is a rare cause of rapidly progressive leukoencephalopathy and stroke mediated by neoplastic microvessel occlusion and inflammatory tissue damage. This report intends to increase awareness among neurologists and other stroke physicians about this disease in order to accelerate diagnosis and initiation of treatment.

NIMG-68. SARCOPENIA AS MEASURED BY TEMPORALIS MUSCLE WIDTH IS A PREDICTOR OF SURVIVAL IN PRIMARY CNS LYMPHOMA

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi145-vi145
Author(s):  
Alipi Bonm ◽  
Anthony Menghini ◽  
Jerome Graber
Keyword(s):  
Clinical Trials ◽  
Standard Deviation ◽  
Intraclass Correlation ◽  
Primary Cns Lymphoma ◽  
Muscle Thickness ◽  
Important Variable ◽  
Cns Lymphoma ◽  
Routine Practice ◽  
Temporalis Muscle ◽  
Increased Risk

Abstract Sarcopenia refers to a loss of skeletal muscle mass, which has been associated with increased risk of injury and decreased ability to perform activities of daily living. In multiple cancers including systemic lymphomas, sarcopenia has been strongly associated with survival and may be an important way to risk stratify patients in clinical trials as well as routine practice. Temporalis muscle width has been reported as an indicator of sarcopenia and independent predictor of outcomes in multiple settings including glioblastoma, brain metastases and subarachnoid hemorrhage. We evaluated temporalis width in primary CNS lymphoma (PCNSL) patients at presentation and outcomes. Using an institutional database of immunocompetent PCNSL patients treated at the University of Washington, two independent readers reviewed the initial MRIs for 104 patients who presented from 2011-2021 and measured the width of the temporalis muscle on axial T1 images. Median duration of follow up was 42.2 months (range 0.59-125.9 months). Median age at diagnosis was 65 (range 19-90 years), and patients were 42.8% male, 57.2% female. Interrater variability was acceptable with an average intraclass correlation coefficient of 0.934. Temporalis measurements were normally distributed, with mean 0.79 cm and standard deviation 0.18 cm. We divided patients into two groups, those with temporalis width less than or greater than 1 standard deviation below the mean (absolute value 0.60 cm). Temporalis width was strongly associated with survival among all patients (χ2=15.5, p< 0.001) as well as patients 65 years or older (χ2=4.5, p=0.03). We conclude that sarcopenia as measured by temporalis muscle thickness is associated with survival in PCNSL and may be an important variable to consider in clinical trials and routine practice.

scholarly journals Inflammatory Demyelinating Brain Lesions Heralding Primary CNS Lymphoma

2012 ◽  
Vol 39 (1) ◽  
pp. 6-10 ◽  
Author(s):  
Leila Husseini ◽  
Andreas Saleh ◽  
Guido Reifenberger ◽  
Hans-Peter Hartung ◽  
Bernd C. Kieseier
Keyword(s):  
White Matter ◽  
Demyelinating Disease ◽  
Primary Cns Lymphoma ◽  
Central Nervous System Lymphoma ◽  
White Matter Lesions ◽  
Brain Biopsy ◽  
Cns Lymphoma ◽  
Cerebral Lesions ◽  
Diagnostic Features ◽  
Relapsing Remitting

Brain biopsy plays a crucial role in the exploration of suspect white matter lesions in the differential diagnosis of primary central nervous system lymphoma (PCNSL) and inflammatory demyelination. We present the case of a previously healthy, immunocompetent woman, aged fifty-nine, who developed a histologically confirmed demyelinating white matter lesion months prior to the manifestation of a PCNSL. Similar cases of “sentinel lesions” preceding a PCNSL have been reported. In a literature review, we compared the diagnostic features that may be useful to differentiate a PCNSL from inflammatory demyelinating disease in older age. We conclude that the occurrence of large, contrast-enhancing cerebral lesions in older patients with a relapsing-remitting disease course and steroid-resistant vision disorders should lead to the consideration of a PCNSL.

Rituximab, Methotrexate, Procarbazine and Lomustine (R-MPL) for the Treatment of Primary CNS Lymphoma

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 48-49
Author(s):  
Eyal Lebel ◽  
Neta Goldschmidt ◽  
Tali Siegal ◽  
Alexander Lossos ◽  
Moshe Gatt ◽  
...  
Keyword(s):  
Medical Center ◽  
Performance Status ◽  
Primary Cns Lymphoma ◽  
Response To Treatment ◽  
Cns Lymphoma ◽  
High Dose ◽  
Cell Transplant ◽  
Rare Type ◽  
End Of Treatment ◽  
Grade 3

Introduction: Primary central nervous lymphoma (PCNSL) is a rare type of non-Hodgkin lymphoma that may involve the brain, spinal cord, leptomeninges and vitreous. The median age at diagnosis is in the fifth decade and most patients (pts) present with significant neurologic deficit and a low performance status. The optimal treatment of PCNSL is controversial. High dose methotrexate (HDMTX) is a standard treatment of PCNSL and is more effective when given in combination with other CNS-penetrating medications, which, however, add to the toxicity of the treatment. We aimed to evaluate the effectiveness and the safety of the combination of rituximab, HDMTX, procarbazine and lomustine (R-MPL) in pts with PCNSL. Patients and methods: We retrospectively reviewed the files of PCNSL pts treated in Hadassah Medical Center, Jerusalem, Israel, between the years 2006-2019. The medical records were reviewed for demographic details and initial disease characteristics (age, sex, performance status, laboratory results, cerebrospinal fluid (CSF) content and tumor location), and for therapeutic details including chemotherapy protocol, toxicity, response to treatment and survival. We excluded pts who could not receive HDMTX. The R-MPL is a 42-day cycle protocol, consisting IV Rituximab 375 mg/m2 on days 1, 15 and 28, IV HDMTX 5 g/m2 (3.5 g/m2 for pts > 60 Years (y)) on days 2, 15 and 29, PO procarbazine 100 mg/m2 (60 mg/m2 for pts > 60 y) on days 3-9, PO lomustine 60 mg/m2 (40 mg/m2 for pts > 60 y) on day 2. Six to nine intrathecal (IT) injections of MTX / cytarabine were included for pts with positive CSF cytology, tumor adjacent to ventricles, or per physician's decision. Rituximab was given for no more than 8 doses in total. A total of 3-4 courses of R-MPL were given. Responsive pts could proceed to autologous stem cell transplant (ASCT) with TECAM conditioning or 2 cycles of intermediate dose cytarabine (IDAC, 1.5 g/m2), 2 doses in each cycle. Those who achieved less than CR or had significant toxicity to R-MPL received additional ifosfamide/etoposide or high dose cytarabine or temazolamide or topotecan. Radiation was given only for salvage. Results: Fifty-two pts were included in the study. Median age was 62 years (range 28-94). Three (6%) had leptomeningeal involvement, thirteen (25%) had vitreoretinal involvement, 30 (58%) had involvement of the deep brain. Mean ECOG, IELSG and MSKCC scores were 1.98, 2.53 and 1.94 respectively. The median number of HDMTX doses was 8 (range 3-16). Forty-five (87%) pts completed at least one 42-day cycle of R-MPL. Among this group, 29 (64%) pts received at least 3 IT injections, 12 (27%) underwent ASCT, 10 (22%) received IDAC, and 7 (16%) received other chemotherapies. The median follow-up was 27 months (m) (range 0.4-140m). The overall response rate (ORR) was 79% (41/52), and the complete response (CR) rate was 52% (27/52). The median progression free survival (PFS) was 15.5m and the median overall survival (OS) was 27m. Among the 45 pts who received at least one R-MPL cycle, the ORR was 87% (39/45), the CR rate was 60% (27/45), the median PFS was 84m and the median OS was not reached (figure 1). Known prognostic factors correlated with OS: age (p<0.01), ECOG performance status (p<0.01), CSF protein (p=0.01) and IELSG/MSKCC scores (p=0.05/0.04). Patients who received at least 3 IT injections had a trend to longer PFS (32.4m Vs 19.2m, p=0.09), and a significant OS benefit (48.6m Vs 20.8m, p<0.01). In a multivariable model, the effect of IT injections was independent of age, sex and IELSG score, and attenuated by number of HDMTX doses. ASCT/IDAC did not improve PFS/OS, however numbers were small. Achieving CR at the end of treatment strongly correlated with PFS/OS, but timing of best response (after one 42-day cycle Vs later) did not. Grade 3-4 adverse events included infections (21%) and kidney injury (13%). Two pts died during therapy, both in the 1st cycle due to disease progression. Ten pts (19%) discontinued therapy due to toxicity. No treatment related mortality (TRM) was documented. Conclusions: The R-MPL protocol achieved a favorable ORR/PFS/OS as described, with reasonable grade 3-4 toxicity and no TRM. The advantage of ASCT/IDAC could not be assessed due to small number of pts. IT injections (at least 3) and achievement of CR at the end of treatment correlated with survival. The R-MPL protocol was reported in previous trials only among the elderly, and should further be evaluated in all age groups. Disclosures Goldschmidt: Abbvie Inc: Consultancy, Research Funding.

scholarly journals Risk of progression of interstitial pneumonia with autoimmune features to a systemic autoimmune rheumatic disease

Rheumatology ◽  
2019 ◽  
Vol 59 (6) ◽  
pp. 1233-1240 ◽  
Author(s):  
Michail K Alevizos ◽  
Jon T Giles ◽  
Nina M Patel ◽  
Elana J Bernstein
Keyword(s):  
Rheumatic Disease ◽  
Interstitial Pneumonia ◽  
Smoking Status ◽  
Medical Center ◽  
Columbia University ◽  
Autoimmune Rheumatic Disease ◽  
Increased Risk ◽  
Systemic Autoimmune Rheumatic Disease ◽  
Risk Of Progression

Abstract Objective The aim of this study was to determine the risk of developing a systemic autoimmune rheumatic disease (ARD) after an initial diagnosis of interstitial pneumonia with autoimmune features (IPAF). Methods We performed a retrospective cohort study of patients with interstitial lung disease (ILD) who were evaluated at Columbia University Irving Medical Center from 2009 to 2017. We divided patients with idiopathic ILD into two groups: those who met IPAF criteria and those who did not meet IPAF criteria at initial ILD diagnosis. We examined the association between IPAF and diagnosis of ARD during the follow-up period using a multivariable-adjusted logistic regression model. Results Of the 697 patients with ILD who were screened, 174 met inclusion criteria (50 met IPAF criteria and 124 did not). During a median follow-up period of 5.2 years, 16% (8/50) of subjects with IPAF were diagnosed with an ARD compared with 1.6% (2/124) of subjects without IPAF (P = 0.001). Adjusting for age, sex, smoking status and use of immunosuppressive therapy, the odds of progressing to an ARD were 14 times higher in subjects with IPAF than in those without IPAF (odds ratio 14.18, 95% CI 1.44–138.95, P = 0.02). Conclusion The presence of IPAF confers an increased risk of developing an ARD. Patients with IPAF should therefore be followed closely for the development of an ARD.

scholarly journals OS1.3 Tumor cell-free DNA detection in CSF for primary CNS lymphoma diagnosis

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii5-iii6
Author(s):  
V Rimelen ◽  
G Ahle ◽  
E Pencreach ◽  
N Zinniger ◽  
A Debliquis ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Primary Cns Lymphoma ◽  
Brain Biopsy ◽  
B Cell Lymphoma ◽  
Cns Lymphoma ◽  
Circulating Dna ◽  
Analytical Sensitivity ◽  
Digital Droplet Pcr ◽  
Droplet Pcr ◽  
Pcr Targeting

Abstract BACKGROUND Primary CNS lymphoma (PCNSL) is a rare disease accounting for around 3% of the primary brain tumors. In the vast majority of cases, PCNSL are classified as diffuse large B-cell lymphoma according to the WHO classification. The diagnosis is based on cranial MRI, in combination with a brain biopsy. In case of classical MRI findings, the identification of lymphoma cells in the cerebrospinal (CSF) or vitreous fluid by cytology and flow cytometry might obviate brain biopsy. The presence of the somatic mutation p.Leu265Pro (L265P) in MYD88 is detectable in 50 to 80% of PCNSL, and might also be helpful to confirm the diagnosis. The aim of our study was to evaluate the contribution of a highly sensitive digital droplet PCR, targeting the mutation L265P MYD88, for the detection of tumoral circulating DNA from CSF supernatant. MATERIAL AND METHODS We identified 9 PCNSL expressing the L265P mutation at diagnosis or relapse. The mutation was found by an allele specific PCR technique either on biopsy or in CSF cells. Circulating DNA was isolated from CSF supernatant with the « QiaAmp® circulating nucleic acid» kit. The quantity of DNA collected was estimated by quantitative PCR for a reference gene (albumine) with 7900HT (Life technologies™) device. Subsequently, the L265P MYD88 mutation was quantified by digital droplet PCR Biorad™: The droplets generated were amplified by PCR, detected with the QX200 Reader, and analyzed with the QuantaSoft™ software. RESULTS The circulating DNA concentration was low, varying between 0 and 2.2 ng/mL of CSF. However, the mutation was detected in the circulating DNA from CSF supernatant in 6 out of 9 cases (66%). The fractional abundance varied from 2.6 to 85%. In 3 cases, the mutation was detected even though cytology and flow cytometry did not reveal leptomeningeal disease. For 3 other cases, the mutation was not detected: The genome copy number was below 1 copy/µL, indicating a low analytical sensitivity for theses samples. CONCLUSION This study shows that circulating DNA is present in low concentration in CSF and can be amplified by a sensitive digital PCR for the L265P MYD88 mutation. The detection of circulating PCNSL DNA in CSF is possible and might be used to improve the non-invasive diagnosis of PCNSL. It might also help to select patients for targeted therapies.

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