Primary leptomeningeal lymphoma masquerading as infectious tubercular meningitis

Primary central nervous system lymphoma (PCNSL) is infrequent and often poses diagnostic conundrums due to its protean manifestations. We present the case of a South Asian young man presenting with raised intracranial pressure and a lymphocytic cerebrospinal fluid (CSF) with pronounced hypoglycorrhachia. Progression of the neuro-ophthalmic signs while on early stages of antitubercular treatment led to additional investigations that produced a final diagnosis of primary leptomeningeal lymphoma. Treatment with chemoimmunotherapy (methotrexate, cytarabine, thiotepa and rituximab (MATRix)) achieved full radiological remission followed by successful autologous transplant. This case highlights the difficulties and diagnostic dilemmas when PCNSL presents as a chronic meningeal infiltrative process. While contextually this CSF is most often indicative of central nervous system tuberculosis and justifies empirical treatment initiation alone, it is essential to include differential diagnoses in the investigation work-up, which also carry poor prognosis without timely treatment. High suspicion, multidisciplinary collaboration and appropriate CSF analysis were the key for a correct diagnosis.

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Distinguishing primary central nervous system lymphoma from other central nervous system diseases: a neurosurgical perspective on diagnostic dilemmas and approaches

Neurosurgical FOCUS ◽

10.3171/foc.2006.21.5.4 ◽

2006 ◽

Vol 21 (5) ◽

pp. 1-7 ◽

Cited By ~ 20

Author(s):

Matthew A. Hunt ◽

Kristoph Jahnke ◽

Tulio P. Murillo ◽

Edward A. Neuwelt

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Demyelinating Disease ◽

Imaging Techniques ◽

Tumor Resection ◽

Delayed Diagnosis ◽

Central Nervous System Lymphoma ◽

Final Diagnosis ◽

Diagnosis And Treatment ◽

Initial Symptoms

Object White matter diseases, including demyelinating or inflammatory disorders, may be indistinguishable clinically and radiologically from some central nervous system (CNS) tumors. In such situations, determination of the final diagnosis is difficult. An example is the differential diagnosis of non-acquired immunodeficiency syndrome–related primary central nervous system lymphoma (PCNSL) and multiple sclerosis (MS), a demyelinating disease. Unfortunately, delayed diagnosis and treatment of PCNSL can negatively affect prognosis. Methods The authors reviewed the cases of eight patients with PCNSL or MS. In each case, the initial diagnosis (PCNSL or MS) was equivocal. In these cases, conventional diagnostic approaches were not definitive, thus further delaying diagnosis. The initial symptoms, the selected diagnostic tests, and the presumptive as well as final diagnosis for each case are discussed. The final diagnosis was PCNSL in six cases and MS in two. The uncertainty about the clinical or initial pathological presentation required further diagnostic evaluation in all cases. Two important neurosurgical guidelines are the avoidance of corticosteroid agents and performance of biopsy sampling rather than volumetric tumor resection. High-volume lumbar puncture, slit-lamp examination/vitrectomy, new CNS imaging techniques, and repeated biopsy procedures also proved helpful. Conclusions In PCNSL, early definitive diagnosis and treatment are the keys to successful outcomes. Knowledge of strategies essential to early diagnosis lessens the need for brain biopsy sampling, but this procedure is still usually necessary. In such selected cases, biopsy sampling is appropriate even when pathological investigation shows MS rather than PCNSL. Complete resection is not indicated in PCNSL and can lead to additional sequelae.

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Primary Central Nervous System Lymphoma of the Posterior Fossa in Immunocompetent Patient: A Case Report and Review of Literature

10.5327/1516-3180.025 ◽

2021 ◽

Author(s):

Vitória de Ataide Caliari ◽

Herika Negri ◽

Claudio vidal ◽

Bruno lobo ◽

Dhyego lacerda ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Case Report ◽

Caudate Nucleus ◽

Posterior Fossa ◽

Correct Diagnosis ◽

Central Nervous System Lymphoma ◽

Clinical History ◽

Cerebellar Hemisphere ◽

Histopathological Analysis

Context: Primary central nervous system lymphomas (PCNSL) are a rare but very aggressive subtype of extranodal non-Hodgkin lymphomas. They represent only 4% of primary central nervous system lesions and are more common in patients with aggressive non-Hodgkin lymphomas, who are HIV positive. Moreover, PCNSL, usually presents as intraparenchymal supratentorial expansive lesions, while secondary CNS lymphomas tend to present as metastases in the leptomeninges. Although they are more common in immunocompromised patients, their incidence has increased with advancing age. Due to its uniqueness in findings, rarity, and severity of the case, we present an immunocompetent elderly patient with a primary lesion of the posterior fossa. Case report: A 85-year-old female was admitted to the emergency room with incoercible vomiting for 48 hours. Initial clinical examination showed dysmetry, and dysbasia. There was no clinical history compatible with immunosuppression. The initial magnetic resonance imaging revealed two non-enhancing contrast lesions in T1 and hyperintense in T2/Flair in the left caudate nucleus, and at the right cerebellar hemisphere near the fourth ventricle and a third parafalcine lesion with homogenous contrast-enhancing on T1 compatible with an incidental meningioma. PET scan, thyroid and breast ultrasonography, and abdominal MRI were done to rule out metastasis, and all results were negative. The histopathological analysis after a stereotactic biopsy performed on the caudate nucleus lesion confirmed the presence of primary central nervous system lymphoma. Conclusions: The pattern of PCNSL is changing due to aging. Knowing this is indispensable for the correct diagnosis and management.

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Primary Central Nervous System Lymphoma: Treatment and Nursing Management of Immunocompetent Patients

Clinical Journal of Oncology Nursing ◽

10.1188/21.cjon.439-448 ◽

2021 ◽

Vol 25 (4) ◽

pp. 439-448

Author(s):

Kurt David ◽

Mary Elizabeth Davis

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Lymphoma ◽

Nursing Management ◽

Lymphoma Treatment ◽

Immunocompetent Patients

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Safety and efficacy of primary central nervous system lymphoma treatment in elderly population

Neurological Sciences ◽

10.1007/s10072-015-2371-2 ◽

2015 ◽

Vol 37 (1) ◽

pp. 131-133

Author(s):

Paola Gaviani ◽

G. Simonetti ◽

A. Innocenti ◽

E. Lamperti ◽

A. Botturi ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Elderly Population ◽

Central Nervous System Lymphoma ◽

Safety And Efficacy ◽

Lymphoma Treatment

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Pathology of primary central nervous system lymphoma

Neurosurgical FOCUS ◽

10.3171/foc.2006.21.5.3 ◽

2006 ◽

Vol 21 (5) ◽

pp. 1-10 ◽

Cited By ~ 28

Author(s):

Deborah L. Commins

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Lymphoma ◽

Great Promise ◽

Histological Classification ◽

Relative Rarity ◽

Diagnostic Work ◽

Work Up ◽

Diagnostic Work Up

✓ The understanding of primary central nervous system lymphoma (PCNSL) has lagged behind that of the much more common systemic, nodal lymphomas. Reasons for this include the relative rarity of PCNSL and the fact that these lesions differ in some ways from their histologically similar systemic counterparts. The purpose of this paper is twofold: first, the author provides concise descriptions of the pathological features of both common and uncommon types of PCNSL while discussing issues such as the confusion surrounding histological classification of PCNSLs, problems related to intraoperative diagnosis, and the appropriate diagnostic work up. Second, the author discusses a small number of molecular studies to demonstrate the great promise they offer. Such studies have already clarified some issues, including the category of lymphocyte from which the majority of PCNSLs are derived. Hopefully in the future these studies will help guide treatment decisions.

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P14.49 Primary central nervous system lymphoma presenting as dementia: do not forget IL10 in CSF analysis

Neuro-Oncology ◽

10.1093/neuonc/noz126.284 ◽

2019 ◽

Vol 21 (Supplement_3) ◽

pp. iii78-iii78

Author(s):

A Blain ◽

A Brinet ◽

M Berthel ◽

A Camoyret ◽

C Gaultier ◽

...

Keyword(s):

Central Nervous System ◽

Flow Cytometry ◽

Nervous System ◽

Symptom Onset ◽

Clinical Presentation ◽

Diagnostic Delay ◽

Central Nervous System Lymphoma ◽

Treatment Modalities ◽

Progressive Dementia ◽

Csf Analysis

Abstract BACKGROUND Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that aﬀects the central nervous system without systemic involvement. Clinical presentation depends on its localization: Focal deficits usually lead to prompt neuroradiologic evaluation, whereas cognitive or behavioral changes can lead to diagnostic delay. Rapidly progressive dementia (RPD), defined as a 1- to 2-year course from symptom onset to cognitive and functional debilitation, is a clinical presentation of PCNSL and thus a potentially curable cause of dementia, leading to death if it remains untreated. Nevertheless, its diagnosis is frequently delayed as other origins (CJD, autoimmune, paraneoplastic, infectious, and neurodegenerative disorders) are more frequently considered. We aimed to describe diagnostic features and treatment modalities in a series of patients presenting with RPD diagnosed as PCNSL. METHODS Patients with RPD and final diagnosis of PCNSL were retrospectively identified from 2012 to 2019 from the database of our academic teaching hospital. Clinical presentation, neuroimaging, and cerebro-spinal fluid (CSF) analysis were assessed from their medical records. Interleukin (IL) 10 and 6 concentrations were measured by flow cytometry using the cytometric bead array (CBA) technique (BD Biosciences) with a detection limit set at 2.5 pg/ml. RESULTS 50 patients with diagnosis of PCNSL were identified, 7 (3 women, 4 men) presented with RPD.Median age was 69 years (range 59–84). Time from symptom onset to diagnostic confirmation of PCNSL ranged from 1–12 months (median 3). All patients presented with cognitive impairment (disorientation, memory disorders), accompanied by psycho-behavioral disorders in 6 of them. MRI disclosed enhancing lesions in all patients. Lumbar puncture was performed in 5 patients. All of them presented pleiocytosis, and lymphomatous meningitis was detected in 4/5 of them by cytology or flow cytometry (FCM). Elevated interleukin 10 (IL-10) was found in all patients with available CSF sample, 4 of them had an IL-10/IL-6 ratio >1. DISCUSSION AND CONCLUSION Patients presenting with rapidly progressive dementia should be evaluated for treatable forms of dementia. PCSNL has to be considered as soon as possible as diagnostic delay impacts its outcome. Imaging studies, usually contrast-enhanced MRI, is recommended, although nonspecific findings are not uncommon, especially in case of non-enhancing lesions. CSF sampling can help to prove diagnosis of PCNSL by cytology (generally associated with FCM), but it requires an experienced cytologist and immediate sample analysis.IL10 and IL10/6 ratio can be used as a more easily available marker to support suspicion of PNSCL, leading to further specific workup. ACKNOWLEDGMENTS NENO & LOC Network

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ML-09 DIAGNOSIS AND TREATMENT OF PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA IN HIV POSITIVE PATIENTS

Neuro-Oncology Advances ◽

10.1093/noajnl/vdz039.151 ◽

2019 ◽

Vol 1 (Supplement_2) ◽

pp. ii33-ii34

Author(s):

Koji Takano ◽

Keisuke Nishimoto ◽

Hiroki Yamazaki ◽

Koki Murakami ◽

Tetsuro Tachi ◽

...

Keyword(s):

Central Nervous System ◽

Cerebrospinal Fluid ◽

Nervous System ◽

Malignant Lymphoma ◽

Central Nervous System Lymphoma ◽

Brain Biopsy ◽

Final Diagnosis ◽

Diagnosis And Treatment ◽

Hiv Positive ◽

Whole Brain Irradiation

Abstract INTRODUCTION HIV infection is known to cause a variety of central nervous system complications, such as malignant lymphoma (ML), toxoplasma encephalopathy, cryptococcal encephalopathy, progressive multifocal leukoencephalopathy (PML), brain tuberculosis and HIV encephalopathy. In our hospital, we performed brain biopsy for HIV-positive patients with central nervous system lesions suspected malignant lymphoma, or cases difficult to diagnose with blood, cerebrospinal fluid, and imaging alone. In this study, we retrospectively examined HIV-positive patients who underwent brain biopsy at our hospital, and analyzed diagnosis and treatment of patients with ML. Methods HIV-positive patients who underwent brain biopsy in our hospital from January 2010 to April 2019 were examined in this study. We analyzed background factors, preoperative examination results, pathological diagnosis, treatment and prognosis. RESULTS There were 1,894 HIV-positive patients who were treated at our hospital during the period, of which 16 cases underwent a total of 18 brain biopsies. The final diagnosis was 7 ML (6 PCNSL, 1 brain metastasis), 3 toxoplasma encephalopathy, 1 PML, 1 brain tuberculosis, 1 immune reconstitution syndrome, 1 Penicillium marneffei infection, 1 hematoma and one case that could not be diagnosed. In patients with ML, preoperative sIL-2 receptor was higher and EBV positive cases in cerebrospinal fluid tended to be more common (p=0.051, p=0.086, respectively). Five of the six patients with PCNSL were treated at our hospital, four of which were treated with highly active antiretroviral treatment (HAART) and whole-brain irradiation and one with HAARA alone, resulting four with CR and one with SD. Four patients, except one who had sudden death of unknown cause, were still alive without recurrence (median observation period 44.5 months). CONCLUSION At the moment, it is difficult to diagnose ML without brain biopsy. HIV-positive status has been regarded as a poor prognosis factor in PCNSL patients, but the prognosis seems to have improved with HAART.

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Cardiovascular Outcomes in the Patients With Primary Central Nervous System Lymphoma: A Multi-Registry Based Cohort Study of 4,038 Cases

Frontiers in Oncology ◽

10.3389/fonc.2021.691038 ◽

2021 ◽

Vol 11 ◽

Author(s):

Zicong Qiu ◽

Yongshi Tang ◽

Yanting Jiang ◽

Miao Su ◽

Xuemin Wang ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Lymphoma ◽

Clinical Information ◽

Cardiovascular Death ◽

Cardiovascular Outcomes ◽

First Year ◽

Non Hodgkin Lymphoma ◽

Elder Patients ◽

Lymphoma Treatment

Primary central nervous system lymphoma (PCNSL) is a rare but highly aggressive non-Hodgkin lymphoma. Treatment-related cardiovascular lesion has become one of the most common complications in patients with tumor. However, very little is known about the cardiovascular death (CVD) of the patients with PCNSL. This study aims at identifying the cardiovascular outcomes of PCNSL patients and making comparison on CVD with extra central nervous system lymphoma (ECNSL). Clinical information of PCNSL and ECNSL was retrieved from the Surveillance, Epidemiology and End Results database. The risk factors of CVD in PCNSL patients and the comparison on the CVD hazard between PCNSL and ECNSL were assessed with the competing risks regression. A 1:2 propensity score matching was used to reduce the imbalanced baseline characteristics between PCNSL and ECNSL. Four thousand thirty-eight PCNSL subjects and 246,760 ECNSL subjects were enrolled in this retrospective study. CVD was the leading cause (41.2%) of non-cancer death in PCNSL patients and mostly occurred within the first year of diagnosis. Age over 60s and diagnosis in 2000–2008 were significantly associated with the elevated risk of CVD in PCNSL patients, while chemotherapy and radiotherapy play no role on the cardiovascular outcomes. Compared with ECNSL patients, the risk of CVD in PCNSL patients were 40% approximately lower. The risk of CVD in the patients with PCNSL still remains unclear currently. Clinicians ought to pay more attention on the risk of CVD in PCNSL patients, especially the elder patients within the first year of diagnosis.

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