Abstract
BackgroundFibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is a rare Epstein-Barr virus (EBV) positive lymphoproliferative disorder included in the current World Health Organization (WHO) classification. It arises within fibrinous material in the context of hematomas, pseudocysts, cardiac myxoma or in relation with prosthetic devices. In these clinical settings the diagnosis requires an high index of suspicion, because it does not form a mass itself, being composed of small foci of neoplastic cells. Despite overlapping features with diffuse large B-cell lymphoma associated with chronic inflammation, it deserves a separate classification, being not mass-forming and often following an indolent course.Case presentationA 64-year-old immunocompetent woman required medical care for cerebral hemorrhage. Computed Tomography (CT) angiography identified an aneurysm in the left middle cerebral artery. A FA-DLBCL was incidentally identified within thrombotic material in the context of the arterial aneurysm. After surgical removal, it followed a benign course with no further treatment.ConclusionsThe current case represents the first report of FA-DLBCL identified in a cerebral artery aneurysm, expanding the clinicopathologic spectrum of this rare entity. A complete literature review is additionally made.
Highlights
Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is a rare Epstein-Barr virus (EBV) positive lymphoproliferative disorder included in the current World Health Organization (WHO) classification
The current case represents the first report of FA-DLBCL identified in a cerebral artery aneurysm, expanding the clinicopathologic spectrum of this rare entity
In the current WHO classification, diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) is defined as an EBV-driven neoplasm, occurring in longstanding chronic inflammation in restricted spaces [1]
Summary
Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is a rare Epstein-Barr virus (EBV) positive lymphoproliferative disorder included in the current World Health Organization (WHO) classification. Our case represents the first report in a patient with a brain hemorrhage and incidentally identified within thrombotic material in a cerebral artery aneurysm. Foci of large lymphoid cells at CD20+, CD79α+, CD10+, BCL6+, BCL2+, myxoma surface Foci of large lymphoid cells within fibrin thrombus CD20+, CD79α+, PAX5+, CD30+, CD43-, CD45+, BCL6+, MUM1+, BCL2+, CD10-, CD3-, CD5-, HHV8-, MYC + 30%; KI67 > 90% LMP1+, EBNA2 + .EBER+.
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