Fibrin-associated EBV-positive Large B-Cell Lymphoma: An Indolent Neoplasm With Features Distinct From Diffuse Large B-Cell Lymphoma Associated With Chronic Inflammation

Incidental cases of localized fibrin-associated Epstein-Barr virus (EBV)+ large B-cell proliferations have been described at unusual anatomic sites and have been included in the category of diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) in the WHO Classification. We describe 12 cases and review the literature to define their clinicopathologic spectrum and compare features with typical cases of DLBCL-CI. Median age was 55.5 years with a M:F ratio of 3. In all 12 cases, the lymphoma was an incidental microscopic finding involving atrial myxomas (n=3), thrombi associated with endovascular grafts (n=3), chronic hematomas (n=2), and pseudocysts (n=4). All cases tested were nongerminal center B-cell origin, type III EBV latency, and were negative for MYC rearrangements and alternative lengthening of telomeres by FISH. Most showed high CD30, Ki67, and PD-L1, and low to moderate MYC and p53 expression. Among 11 patients with detailed follow-up, 6 were treated surgically, 3 with cardiac or vascular lesions had persistent/recurrent disease at intravascular sites, and 4 died of causes not directly attributable to lymphoma. Reports of previously published fibrin-associated cases showed similar features, whereas traditional DLBCL-CI cases with a mass lesion had significantly higher lymphoma-associated mortality. Fibrin-associated EBV+ large B-cell lymphoma is clinicopathologically distinct from DLBCL-CI, warranting separate classification. Most cases, particularly those associated with pseudocysts, behave indolently with the potential for cure by surgery alone and may represent a form of EBV+ lymphoproliferative disease rather than lymphoma. However, primary cardiac or vascular disease may have a higher risk of recurrence despite systemic chemotherapy..

Medienart:

Artikel

Erscheinungsjahr:

2017

Erschienen:

2017

Enthalten in:

Zur Gesamtaufnahme - volume:41

Enthalten in:

The American journal of surgical pathology - 41(2017), 3, Seite 299-312

Sprache:

Englisch

Beteiligte Personen:

Boyer, Daniel F [VerfasserIn]
McKelvie, Penelope A [Sonstige Person]
de Leval, Laurence [Sonstige Person]
Edlefsen, Kerstin L [Sonstige Person]
Ko, Young-Hyeh [Sonstige Person]
Aberman, Zachary A [Sonstige Person]
Kovach, Alexandra E [Sonstige Person]
Masih, Aneal [Sonstige Person]
Nishino, Ha T [Sonstige Person]
Weiss, Lawrence M [Sonstige Person]
Meeker, Alan K [Sonstige Person]
Nardi, Valentina [Sonstige Person]
Palisoc, Maryknoll [Sonstige Person]
Shao, Lina [Sonstige Person]
Pittaluga, Stefania [Sonstige Person]
Ferry, Judith A [Sonstige Person]
Harris, Nancy Lee [Sonstige Person]
Sohani, Aliyah R [Sonstige Person]

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BKL:

44.47

44.65

RVK:

RVK Klassifikation

doi:

10.1097/PAS.0000000000000775

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

OLC1992483019