A Case of Central Nervous System Lymphoma Manifesting as Multiple Patchy White Matter Lesions with a Past History of Tonsil Lymphoma

Background: Venous thromboembolism (VTE) is a frequent, potentially lethal complication in individuals with cancer. Patients with brain tumors are at particularly high risk for VTE. Primary central nervous system lymphoma (PCNSL) is a rare subtype of diffuse large B cell lymphoma, involving the craniospinal axis. The incidence of VTE in patients with PCNSL was reported as very high, occurring mostly in the early period of therapy. Objectives: We aimed to evaluate the efficacy and safety of prophylactic low-molecular-weight heparin (LMWH) throughout the treatment of PCNSL. Patients: All patients >18 years of age diagnosed and treated for PCNSL at our institution in 2005–2017 were included. Results: There were 44 patients; mean age at diagnosis was 61.5 years. Three patients (6.8%) had a personal history of thrombosis, 11 (25%) had a history of diabetes or smoking, and 32 (72%) had an Eastern Cooperative Oncology Group performance status of 0–1 at diagnosis. During treatment with LMWH, no VTE events were recorded; 2 (4.5%) patients experienced a minor bleeding event and 1 (2.3%) a major bleeding event. Conclusions: Among our 44 patients with PCNSL treated with prophylactic LMWH, no VTE events were recorded, and only 1 (asymptomatic) intracranial bleed was recorded. Within the limitations of a retrospective nonrandomized study, our findings suggest that VTE prophylaxis may be beneficial for individuals with PCNSL.

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Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre

Journal of Child Neurology ◽

10.1177/0883073820931253 ◽

2020 ◽

Vol 35 (11) ◽

pp. 753-762

Author(s):

Ronald R. Seese ◽

Daniel Glaser ◽

Andre Furtado ◽

Kavita Thakkar ◽

Kathryn S. Torok

Keyword(s):

Central Nervous System ◽

Nervous System ◽

White Matter ◽

Neurological Symptoms ◽

Localized Scleroderma ◽

Imaging Findings ◽

History Of ◽

Parry Romberg Syndrome ◽

Clinical Associations

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Methods: Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. Results: From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions: Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings.

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Kaposi's Sarcoma After Liver Transplantation from a Donor with a History of Ventriculoperitoneal Shunt and Craniotomy for Primary Central Nervous System Lymphoma: Report of a Case

Surgery Today ◽

10.1007/s00595-007-3565-x ◽

2007 ◽

Vol 38 (1) ◽

pp. 90-94 ◽

Cited By ~ 1

Author(s):

Burak Isik ◽

Sezai Yilmaz ◽

Vedat Kirimlioglu ◽

Hale Kirimlioglu ◽

Mehmet Yilmaz ◽

...

Keyword(s):

Central Nervous System ◽

Liver Transplantation ◽

Nervous System ◽

Ventriculoperitoneal Shunt ◽

Kaposi's Sarcoma ◽

Central Nervous System Lymphoma ◽

Kaposi’S Sarcoma ◽

History Of

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Primary peripheral T-cell central nervous system lymphoma

Surgical Neurology International ◽

10.25259/sni_224_2021 ◽

2021 ◽

Vol 12 ◽

pp. 465

Author(s):

Cylaina E. Bird ◽

Jeffrey I. Traylor ◽

Jenna Thomas ◽

James P. Caruso ◽

Benjamin Kafka ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

T Cell ◽

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Central Nervous System Lymphoma ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

History Of ◽

Sjögren‘S Syndrome

Background: Primary peripheral T-cell central nervous system lymphoma (PCNSL) is a rare, aggressive tumor that arises in the craniospinal axis and has an increased risk in individuals who are immunocompromised. This lesion often mimics other benign and malignant processes on radiographic imaging, leading to misdiagnosis and delays in treatment. We present a case of a patient with a history of Sjögren’s syndrome and progressive neurologic symptoms who underwent craniotomy for diagnosis. Case Description: A 61-year-old woman with a history of Sjögren’s syndrome, progressive aphasia, left facial droop, and right-sided paresthesias for 4 months presented for evaluation and management. An enhancing, infiltrative lesion in the left frontal lobe with underlying vasogenic edema was appreciated and suggestive of a primary or metastatic neoplasm. The patient underwent an open biopsy for further evaluation of the lesion. Extensive histopathologic evaluation revealed a diagnosis of T-cell PCNSL. The patient was started on induction methotrexate and temozolomide followed by consolidative radiotherapy. Conclusion: Autoimmune conditions are a risk factor for T-cell PCNSL development. T-cell PCNSL has radiographic and gross histologic features that are consistent with a broad differential, including gliomas and inflammatory processes. Prompt diagnosis and extensive histopathological evaluation is essential to ensure appropriate treatment.

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Low Molecular Weight Heparin (LMWH) for Venous Thromboembolism (VTE) Prophylaxis in Patients with Primary Central Nervous System Lymphoma (PCNSL)

Blood ◽

10.1182/blood-2018-99-119154 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 2993-2993

Author(s):

Deborah Rund ◽

Stav Gazal ◽

Yosef Kalish ◽

Chen Makranz ◽

Neta Goldschmidt

Keyword(s):

Molecular Weight ◽

Central Nervous System ◽

Nervous System ◽

Venous Thromboembolism ◽

Low Molecular Weight Heparin ◽

Medical Center ◽

Bleeding Event ◽

Central Nervous System Lymphoma ◽

Low Molecular Weight ◽

History Of

Abstract Introduction: Venous thromboembolism (VTE) is a frequent, potentially lethal, complication in patients with cancer. Patients with brain tumors are at a particularly high risk for VTE. Primary central nervous system lymphoma (PCNSL) is a rare subtype of diffuse large B-cell lymphoma, involving the cranio-spinal axis. The incidence of VTE in patients with PCNSL is as high as 30-60% in various series, occurring mostly in the early period of therapy. Due to this high incidence, the policy in our medical center since the year 2005, is to treat with prophylactic low molecular weight heparin (LMWH) from the time of PCNSL diagnosis until the end of treatment. We aimed to evaluate the incidence of VTE in patients with PCNSL treated with prophylactic LMWH. Material and methods: All patients ≥18 years who were diagnosed and treated for PCNSL in Hadassah-Hebrew University Medical Center between the years 2005-2017 were included in the study. We retrospectively reviewed their medical records for demographic details and initial disease characteristics (age at diagnosis, sex, performance status, laboratory results such as LDH, cerebrospinal fluid content and location of the growth), for details of risk factors for VTE such as diabetes, smoking or heart failure, and for personal or familial history of thrombosis. Therapeutic details including chemotherapy protocol, response to treatment and supportive care were compiled. Specifically we noted if prophylactic LMWH was given, if any complications developed due to the LMWH treatment and whether a VTE event occurred. Results: Forty four patients were included in the study. Mean age at diagnosis was 60.2 years and there were 27 (61%) females. Three (6.8%) patients had a personal history of thrombosis and 13 (29%) had a history of diabetes or smoking. Thirty two (72%) had an ECOG performance study of 0-1 at diagnosis and seven (16%) had leptomeningeal involvement. Forty one (93%) of patients were treated with a systemic high dose methotrexate (HDMTX) based protocol (mean of 7.6 courses of HDMTX per patient) and thirty two (73%) patients were treated with systemic rituximab. All 44 patients were treated with prophylactic LMWH, mostly at a dose of 40 mg per day (41 patients, 93%). Of the 44 patients, five (11%) discontinued treatment; 2 due to side effects (abnormal liver function tests and subdural hematoma (SDH)) and 3 for an unknown reason. Three (7%) patients had a minor bleeding event (gum, conjunctival, Ommaya reservoir catheter tract). One patient (2.3%) had a major bleeding event (SDH) while on LMWH treatment which was found on routine MRI imaging of the brain as he was asymptomatic. No VTE events (0%) were recorded in patients treated with LMWH. Two patients had a VTE, however both patients were off LMWH treatment at the time of VTE (one stopped LMWH, the other was diagnosed with VTE concurrently with the diagnosis of PCNSL). Conclusions: In our group of 44 PCNSL patients, prophylactic use of LMWH was highly effective, with no VTE events. Two cases of VTE occurred in our patient group, both occurred while the patients were off LMWH treatment. Only one, asymptomatic, intracranial bleed was recorded, indicating the relative safety of this treatment in PCNSL patients. Further prospective studies should be done to support the routine use of this prophylactic strategy. Disclosures No relevant conflicts of interest to declare.

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Fatal bromethalin intoxication in 3 cats and 2 dogs with minimal or no histologic central nervous system spongiform change

Journal of Veterinary Diagnostic Investigation ◽

10.1177/1040638718775463 ◽

2018 ◽

Vol 30 (4) ◽

pp. 642-645 ◽

Cited By ~ 2

Author(s):

Megan C. Romano ◽

Alan T. Loynachan ◽

Dave C. Bolin ◽

Uneeda K. Bryant ◽

Laura Kennedy ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

White Matter ◽

Clinical Signs ◽

Experimental Models ◽

Presumptive Diagnosis ◽

Spongiform Change ◽

History Of ◽

The Central Nervous System ◽

Cns Lesions

Use of the neurotoxic rodenticide bromethalin has steadily increased since 2011, resulting in an increased incidence of bromethalin intoxications in pets. Presumptive diagnosis of bromethalin toxicosis relies on history of possible rodenticide exposure coupled with compatible neurologic signs or sudden death, and postmortem examination findings that eliminate other causes of death. Diagnosis is confirmed by detecting the metabolite desmethylbromethalin (DMB) in tissues. In experimental models, spongiform change in white matter of the central nervous system (CNS) is the hallmark histologic feature of bromethalin poisoning. We describe fatal bromethalin intoxication in 3 cats and 2 dogs with equivocal or no CNS white matter spongiform change, illustrating that the lesions described in models can be absent in clinical cases of bromethalin intoxication. Cases with history and clinical signs compatible with bromethalin intoxication warrant tissue analysis for DMB even when CNS lesions are not evident.

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Utility of Flow Cytometry of Cerebrospinal Fluid as a Screening Tool in the Diagnosis of Central Nervous System Lymphoma

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2012-0313-oa ◽

2013 ◽

Vol 137 (11) ◽

pp. 1610-1618 ◽

Cited By ~ 11

Author(s):

Meredith Pittman ◽

Susan Treese ◽

Ling Chen ◽

John L. Frater ◽

TuDung T. Nguyen ◽

...

Keyword(s):

Central Nervous System ◽

Flow Cytometry ◽

Cerebrospinal Fluid ◽

Nervous System ◽

Predictive Value ◽

Central Nervous System Lymphoma ◽

Brain Biopsy ◽

Predictive Values ◽

History Of

Context.—Experiences at our institution show that flow cytometry analysis (FCA) has become routine clinical practice in the workup of patients with altered mental status, even if risk factors are low. Objective.—To assess diagnostic accuracy of combined FCA and cytology in the diagnosis of central nervous system lymphoma in an unselected patient population with neurologic symptoms, including patients with no history of lymphoma or suspicious radiology. Design.—Between 2001 and 2011, cerebrospinal fluid was submitted from 373 patients for lymphoma screening by FCA. The medical records were reviewed for patient symptomatology, history of malignancy, brain imaging, FCA results, cytology results, brain biopsy, and clinical follow-up. Results.—A lymphoid malignancy was detected by FCA in 4% of cases. A positive diagnosis was more likely in patients with either a history of hematologic malignancy and/or a suspicious radiology result (P = .009). All patients with no history of lymphoma and no suspicious radiology (n = 102) had negative cytology, and none had a correspondingly positive FCA result. The positive and negative predictive values of combined cytology and FCA in the patients with history of lymphoma and/or abnormal imaging results were 92% and 89%, respectively, when compared with open brain tissue biopsy, and 89% and 86%, respectively, when compared with clinical follow-up. When low-risk patients were included, the positive predictive value remained at 92%, but the negative predictive value dropped to 52% with the open brain biopsy as the reference, and values did not change significantly for the group with clinical follow-up. Conclusions.—Concurrent FCA and cytology are most useful in the appropriate clinical setting, and we propose a triage algorithm for how FCA on cerebrospinal fluid is best used.

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