Primary central nervous system Epstein–Barr virus-positive diffuse large B-cell lymphoma of the elderly: a clinicopathologic study of five cases

Jamal, Siraj M. El; Sy, Li; Bajaj, Renu; Wang, Zixuan; Kenyon, Lawrence C.; Glass, Jon; Pang, Changlee S.; Bhagavathi, Sharathkumar; Peiper, Stephen C.; Gong, Jerald Z.

doi:10.1007/s10014-013-0173-x

Cited by 17 publications

(20 citation statements)

References 27 publications

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“…The proportion of positivity for most biomarkers in this study was similar to that in previous studies [11,[29][30][31]. The positivity rate of Epstein-Barr virus was also similar to previously reported Asian data (9 % in Korea and 6.1-11.4 % in Japan) [32][33][34], and slightly higher than those of western countries (4 % in PCNSL of the elderly) [35]. Of all these biomarkers, no one was significantly associated with the intraocular involvement of PCNSL except Ki-67.…”

Section: Discussionsupporting

confidence: 93%

Risk factors for intraocular involvement in patients with primary central nervous system lymphoma

Cho

2014

J Neurooncol

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To determine the risk factors for intraocular involvement in patients with primary central nervous system lymphoma (PCNSL), a retrospective chart review was performed on 136 patients who were pathologically diagnosed with PCNSL. The patients were investigated for demographics, clinical manifestation, and the profile of immunohistochemical tumor biomarkers, as well as for the presence of intraocular involvement of lymphoma at diagnosis or during follow-up. The mean age of the entire cohort was 58.6 ± 12.4 years, and the mean follow-up period was 31.1 ± 30.8 months. Twenty-nine (21 %) patients had an intraocular involvement, among which 20 (69 %) patients presented with intraocular involvement at diagnosis of PCNSL and 9 (31 %) patients developed intraocular involvement after a mean period of 32.4 ± 33.6 months. Of the patients with intraocular involvement, 8 (28 %) had no visual symptom at the diagnosis of ocular invasion. Between those with and without intraocular involvement, no significant differences were found with respect to the age, sex, and follow-up period as well as cerebrospinal fluid spread and bone marrow involvement. Among the immunohistochemical biomarkers, the Ki-67 proliferation index was significantly higher in patients with intraocular involvement than in patients without (P = 0.021), but the other investigated biomarkers did not show a significant difference between the two groups. A Ki-67 level ≥80 % was a risk factor for the intraocular involvement in patients with PCNSL (odds ratio, 2.63). Median overall survival was 39.0 months in the entire cohort and was not significantly different between those with and without intraocular involvement (P = 0.959).

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Section: Discussionsupporting

confidence: 93%

Risk factors for intraocular involvement in patients with primary central nervous system lymphoma

Cho

2014

J Neurooncol

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“…However, in a review of the reported cases, EBV‐positive diffuse large B‐cell lymphomas of the elderly did occur in the CNS of immunocompetent patients, arising in elderly patients aged > 50 years without predisposing immunodeficiencies, suggesting that this disease in the CNS also has a relationship with an immunological deterioration derived from the aging process. Therefore, taking into consideration additional common clinicopathological features, several investigators have recently considered that EBV presence in primary CNS B‐cell lymphomas in immunocompetent elderly patients should be categorized with primary CNS EBV‐positive diffuse large B‐cell lymphomas of the elderly (Table ) …”

Section: Primary Cns Ebv‐positive Diffuse Large B‐cell Lymphomas Of Tmentioning

confidence: 99%

“…Recent advances in investigation of primary CNS lymphomas and related diseases have provided clinicians and pathologists with new information about these diseases . Although primary diffuse large B‐cell lymphomas are designated as primary CNS lymphomas according to the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue, a variety of other CNS lymphomas, such as primary CNS Burkitt lymphoma, primary CNS B‐cell lymphomas that have features intermediate between diffuse large B‐cell lymphomas and Burkitt lymphoma, and primary CNS EBV‐positive diffuse large B‐cell lymphoma of the elderly, have been reported in recent years. In addition, there has been controversy regarding cases of primary CNS lymphoid granulomatosis …”

Section: Introductionmentioning

confidence: 99%

Primary central nervous system lymphomas and related diseases: Pathological characteristics and discussion of the differential diagnosis

et al. 2015

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Although primary diffuse large B-cell lymphomas of the CNS are designated as primary CNS lymphomas according to the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue in 2008, a variety of other lymphomas (Burkitt lymphomas, EBV-positive diffuse large B-cell lymphoma of the elderly) and related diseases (lymphomatoid granulomatosis) that are also found in the CNS have been spotlighted in recent years. The histopathology of primary CNS Burkitt lymphomas mimics that of primary diffuse large B-cell lymphomas of the CNS after steroid administration. Therefore, for correct diagnosis of the involved lymphoma, comprehensive fluorescent in situ hybridization analysis for c-MYC and BCL2 is recommended in all primary CNS lymphoma cases with aggressive clinical course, multifocal involvement of the CNS, and a high proliferation index. The pathological characteristics of primary CNS EBV-positive diffuse large B-cell lymphoma of the elderly have similarities with those of the latency phenotype III, EBV lymphoproliferative disorders that arise in the setting of immunodeficiency. These age-related lymphomas usually occur in elderly immunocompetent patients, and the incidence of this disease was estimated to range from 4.0% to 13.6% of all primary CNS lymphomas. Shorter overall survival has been reported for patients with this disease. Lymphomatoid granulomatosis (LYG) is a systemic, EBV-driven, angiocentric and angiodestructive lymphoproliferative disorder. Primary LYG that shows distinct clinicopathological features compared with systemic LYG was recently reported. Finally, this review focuses on the relationship between primary CNS lymphomas and demyelinating diseases, and the concomitant use of intraoperative cytology and frozen sections that are helpful in rapid intraoperative diagnosis.

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“…Risk factors in immunocompetent patients are unknown, but long term immunosuppressive therapy or an underlying disease with associated immunosuppression are significant risk factors 7 . Lymphomagenesis is thought to be promoted by sustained antigenic stimulation by autoimmune diseases (such as SLE, Sjögren´s syndrome or rheumatoid arthritis) and/or by direct transforming properties of infectious agents 2,8,9 . CNS lymphomas developing in immunocompromised individuals are typically B-cell neoplasms, mostly DLBCL, and are EBV-positive (>95% vs 0%-20% in immunocompetent patients) 3,9 .…”

Section: Discussionmentioning

confidence: 99%

“…Lymphomagenesis is thought to be promoted by sustained antigenic stimulation by autoimmune diseases (such as SLE, Sjögren´s syndrome or rheumatoid arthritis) and/or by direct transforming properties of infectious agents 2,8,9 . CNS lymphomas developing in immunocompromised individuals are typically B-cell neoplasms, mostly DLBCL, and are EBV-positive (>95% vs 0%-20% in immunocompetent patients) 3,9 . As in most CNS lymphomas developing in immunocompromised individuals, this patient was diagnosed with a DLBCL, and was EBV-positive.…”

Section: Discussionmentioning

confidence: 99%

Multiple central nervous system lesions on chronic corticosteroid therapy – a diagnostic challenge

Caçola¹,

Augusto²,

Bernardes³

et al. 2015

Gal. Clin.

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Diagnostic challenges often arise when multiple central nervous system (CNS) lesions not accessible to biopsy are present on Magnetic Resonance Imaging (MRI). The differential diagnosis is broad, particularly in the context of exposure to steroids and/or in the setting of immunosuppression. Benign lesions to be considered are demyelinating diseases, sarcoidosis and other granulomatous diseases, and infectious diseases such as toxoplasmosis in immunocompromised patients and tuberculosis. Malignant lesions include CNS lymphoma, glioblastoma multiforme and brain metastases. The authors describe the clinical case of a 75-year-old woman with systemic lupus erythematosus (SLE), chronically medicated with Hydroxychloroquine 400 mg, Deflazacort 9 mg and Azathioprine 25 mg daily, who presented to the hospital with a 5-month history of progressive neurological deficits. It was particularly challenging, because the patient was immunocompromised, had multiple CNS lesions that were not accessible to biopsy and chronic therapy with corticosteroids altered the radiological pattern on brain MRI, masquerading the diagnosis. By writing up this case and review of the literature, further evidence is provided for loss of distinctive histological and radiographic findings of CNS lesions on steroid therapy, with a potential delay in diagnosis and subsequent worse prognosis. Key Words: Primary central nervous system neoplasms; central nervous system infections; Epstein-Barr virus infections; corticoids. ResumoDesafios diagnósticos surgem frequentemente quando lesões múltiplas do sistema nervoso central (SNC) não acessíveis a biópsia estão presentes na Ressonância Magnética (RM). O diagnóstico diferencial é vasto, particularmente no contexto de exposição a corticóides e/ou estado de imunodepressão. As lesões benignas a considerar são as doenças desmielinizantes, sarcoidose e outras doenças granulomatosas, e doenças infecciosas como a toxoplasmose nos pacientes imunodeprimidos e tuberculose. As lesões malignas incluem o linfoma do SNC, glioblastoma multiforme e metástases cerebrais. Os autores descrevem o caso clínico de uma mulher de 75 anos com lúpus eritematoso sistémico, medicada cronicamente com Hidroxicloroquina 400 mg, Deflazacorte 9 mg e Azatioprina 25 mg diariamente, que foi admitida no hospital com um quadro com cinco meses de evolução de défices neurológicos progressivos. Este foi particularmente desafiante, uma vez que a doente era imunodeprimida, tinha lesões múltiplas do SNC que não eram acessíveis a biópsia e a corticoterapia crónica alterou o padrão radiológico na RM cerebral, mascarando o diagnóstico. O relato deste caso e revisão da literatura fornece mais evidência sobre a perda das características histológicas e radioló-gicas das lesões do SNC sob corticoterapia, com um potencial atraso no diagnóstico e subsequente pior prognóstico. Palavras-chave: Neoplasias primárias do sistema nervoso central; infecções do sistema nervoso central; infecções pelo vírus Epstein-Barr; corticóides.

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Primary central nervous system Epstein–Barr virus-positive diffuse large B-cell lymphoma of the elderly: a clinicopathologic study of five cases

Cited by 17 publications

References 27 publications

Risk factors for intraocular involvement in patients with primary central nervous system lymphoma

Risk factors for intraocular involvement in patients with primary central nervous system lymphoma

Primary central nervous system lymphomas and related diseases: Pathological characteristics and discussion of the differential diagnosis

Multiple central nervous system lesions on chronic corticosteroid therapy – a diagnostic challenge

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