A Rare Case of Composite Dural Extranodal Marginal Zone Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

Bustoros, Mark; Liechty, Benjamin; Zagzag, David; Liu, Cynthia; Shepherd, Timothy M.; Gruber, Deborah; Raphael, Bruce; Placantonakis, Dimitris G.

doi:10.3389/fneur.2018.00267

Cited by 20 publications

(29 citation statements)

References 63 publications

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“…DLBCL was the most frequently encountered histological subtype in our cohort, and it was followed by MZL. Emerging reports may suggest that dural DLBCL represents a considerably large number of PDLs, whereas most retrospective studies have postulated that the majority of PDL cases are MZL . Immunohistochemistry for MYC, BCL2, and BCL6 expression may be helpful in further characterizing histological subtypes.…”

Section: Discussionmentioning

confidence: 99%

Primary dural lymphomas: Clinical presentation, management, and outcome

Karschnia

Batchelor

Jordan

et al. 2020

Cancer

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BACKGROUND: Clinical experience is limited for primary central nervous system (CNS) lymphoma that arises from the dura mater, which is denoted with the term primary dural lymphoma (PDL). This study was aimed at determining the relative incidence, presentation, and outcomes of PDL. METHODS: The institutional databases of the Divisions of Neuro-Oncology at the Massachusetts General Hospital and the Yale School of Medicine were retrospectively searched for patients with primary CNS lymphoma. Patients with pathologically confirmed dural lymphoma and no evidence of primary cerebral or systemic involvement were identified. Clinical data, diagnostic findings, treatments, and outcomes were recorded. RESULTS: A total of 20 patients with PDL were identified, and they represented 6.3% of the individuals with primary CNS lymphomas (20 of 316). Histopathological examination of PDL revealed the following underlying subtypes: diffuse large B-cell lymphoma (10 of 20 patients), marginal zone lymphoma (6 of 20), follicular lymphoma (2 of 20), undefined B-cell non-Hodgkin lymphoma (1 of 20), and T-cell non-Hodgkin lymphoma (1 of 20). On imaging, all tumors appeared as extraaxial masses with avid contrast enhancement and mostly mimicked meningioma. The median apparent diffusion coefficient value was 667 ± 26 mm 2 /s. Cerebrospinal fluid analyses and symptoms were nonspecific, and the diagnosis rested on tissue analysis. Therapeutic approaches included surgery, radiotherapy, and chemotherapy. The median overall survival was not reached after 5 years. Three patients were deceased at database closure because of tumor progression. The extent of tumor resection correlated positively with overall survival (P = .044). CONCLUSIONS: PDL is a rare variant of primary CNS lymphoma that can be radiographically mistaken for meningioma. The outcome is excellent with multimodality treatment, and aggressive surgery may convey a survival advantage in select cases.

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Section: Discussionmentioning

confidence: 99%

Primary dural lymphomas: Clinical presentation, management, and outcome

Karschnia

Batchelor

Jordan

et al. 2020

Cancer

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“…Initially reported by Kumar et al [6] in 1997, 15 years after the first MALT lymphomas description, there are nearly 100 reported cases in literature of MALT arising at the dura [4]. Appearing more frequently in the fourth and fifth decades (sooner than most indolent lymphoproliferative neoplasms) [7], with a male to female ratio of 1:5, it is usually characterized as a slowly growing dural mass with no infiltrative aspect or systemic involvement.…”

Section: Discussionmentioning

confidence: 99%

“…[12]. Bustoros et al [4] reviewed 104 patients in a literature search. Most of them (64%) were treated with two modalities including resection, RT or chemotherapy, and the majority achieved complete remission on follow-up (only 12 relapses).…”

Section: Discussionmentioning

confidence: 99%

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Marginal zone lymphoma of the dura — two case reports with long-term follow-up

Rosas

Favareto

Vieira

et al. 2021

Rep Pract Oncol Radiother.

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“…In general, marginal zone lymphoma is also indolent [5,8,9] and rarely spreads to the CNS [10]. To date, reports of CNS invasion by marginal zone lymphomas describe focal intracranial lesions, for instance mucosa-associated lymphoid tissue (MALT) subtypes mimicking meningioma [11][12][13] and dural MZL [14].…”

Section: Discussionmentioning

confidence: 99%

Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report

et al. 2020

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Background: Hematologic malignancies can spread to the central nervous system (CNS), either as focal lesions or as leptomeningeal disease. Marginal zone lymphoma (MZL) is a low-grade non-Hodgkin lymphoma and generally presents as an indolent disease. This case report illustrates an unexpected diagnosis of leptomeningeal metastasis in an MZL, presenting as a delirium without B symptoms, pronounced hematologic progression or abnormalities on cerebral imaging. Case presentation: An 80-year-old patient with a medical history of monoclonal B-cell lymphocytosis (MBL) with a clone indicative for an MZL, presented to the emergency and the geriatric departments with a recent cognitive deterioration and behavioral changes. MMSE score was 18/30. After excluding the most common etiologies through classical work-up including a normal head magnetic resonance imaging, a lumbar puncture was performed. In the cerebrospinal fluid an elevated protein level and increased lymphocyte count were identified, whereas beta-amyloid and tau protein levels were normal. Immunophenotyping of the lymphocytes confirmed CNS invasion by the MZL clone. Staging revealed mild splenomegaly. Prednisolone, intrathecal and systemic chemotherapy were initiated, leading to quick cognitive improvement with a final MMSE score of 28/30. Conclusions: To the best of our knowledge a delirium in an older patient due to leptomeningeal disease in MZL has never been described. To date, rare reports of CNS invasion by MZL describe focal intracranial lesions. After exclusion of common etiologies, physicians should remain vigilant when confronted with a patient with history of MBL presenting neurological symptoms. This case illustrates the importance of low threshold for lumbar punctures in this population, also for those patients with normal imaging studies.

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A Rare Case of Composite Dural Extranodal Marginal Zone Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

Cited by 20 publications

References 63 publications

Primary dural lymphomas: Clinical presentation, management, and outcome

Primary dural lymphomas: Clinical presentation, management, and outcome

Marginal zone lymphoma of the dura — two case reports with long-term follow-up

Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report

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