Central MessageFibrin-associated large B-cell lymphoma around the vascular prosthesis mimics recurrent AAA after aortic replacement. Differentiation based on image finding is difficult.See Commentary on page XXX. Fibrin-associated large B-cell lymphoma around the vascular prosthesis mimics recurrent AAA after aortic replacement. Differentiation based on image finding is difficult. See Commentary on page XXX. Fibrin-associated large B-cell lymphoma (FA-LBCL) is a rare subtype of extranodal B-cell lymphoma that arises within the fibrinous material in cysts and pseudocysts, hematomas, cardiac myxomas, and prosthetic devices and is thought to occur secondary to chronic Epstein–Barr virus (EBV) infection in the setting of a chronic inflammatory state. EBV-infected B cells expressing latent membrane protein-1 are attacked by cytotoxic B cells under normal conditions, but under immunosuppressive conditions resulting from chronic inflammation caused by foreign materials, latent membrane protein-1 causes expression of Bcl-2, inhibiting EBV-infected cell apoptosis and providing favorable environments for the development of lymphoma. It does not form a mass; hence, it is often diagnosed incidentally on histologic evaluation of surgical specimens or at postmortem. We report a case of FA-LBCL around vascular prosthesis, which led to the enlargement of an abdominal aortic aneurysmal sac. A 67-year-old man presented to our hospital for examination of an enlarged abdominal aortic aneurysm (AAA) 6 years after endovascular aortic repair with Powerlink (Endologix). Contrast-enhanced computed tomography (CT) showed a 79-mm infrarenal AAA (Figure 1, A). Endoleaks were not detected on 4-dimensional CT. Therefore, a type V endoleak was considered to be present. Aortic replacement with a Y-shaped vascular prosthesis (Gelsoft Plus; Terumo Aortic) was performed. However, the aneurysmal sac covering the vascular prosthesis became enlarged subsequently, which caused the vascular prosthesis to be compressed forward, resulting in the narrowing of its lumen 3 years after aortic replacement (Figure 1, B, and Video 1). He was suggested to undergo surgical aortic repair but refused additional treatment. Therefore, CT-guided drainage within the aneurysmal sac was planned to confirm the diagnosis. Although we believed that there was no blood flow within the aneurysmal sac because of no-contrast effect on CT, we planned on performing emergency surgery if the AAA ruptured. CT-guided needle biopsy showed approximately 80 mL of dark-red drainage fluid, and cytology by cell block method revealed large, atypical lymphocytes (Figure 1, C). Immunostaining demonstrated that the sample was positive for CD20 (Figure 1, D), CD79a, MUM1, and BCL2 and negative for CD3 (Figure 1, E), CD5, CD10, BCL6, and human herpesvirus-8. The lymphoma cells were also positive for EBV. Encoded RNA in situ hybridization (Figure 1, F) contributed to the diagnosis of FA-LBCL. Fluorodeoxyglucose-positron emission tomography/CT showed mild uptake around the vascular prosthesis, indicating an inflammatory state. The patient declined redo aortic replacement and underwent 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. The aneurysmal sac continues to enlarge 2 years after diagnosis. FA-LBCL is uncommon, and only 10 cases of FA-LBCL associated with vascular prostheses have been reported in the literature1Yamamoto Y. Bhunchet E. Uchiyama H. Oonuki M. Fibrin-associated diffuse large B-cell lymphoma arising in an abdominal aortic graft: a case report and literature review.Ann Vasc Surg. 2022; 2: 100050Google Scholar, 2Miller D.V. Firchau D.J. McClure R.F. Kurtin P.J. Feldman A.L. Epstein–Barr virus-associated diffuse large B-cell lymphoma arising on cardiac prostheses.Am J Surg Pathol. 2010; 34: 377-384Crossref PubMed Scopus (50) Google Scholar, 3Maedeh M. Francisco T. Sunny K. Craig O. Jessica D. Guang F. Recurrent fibrin associated diffuse large B-cell lymphoma: a case report.Hum Pathol Case Rep. 2021; 25: 200538Crossref Scopus (0) Google Scholar, 4Zamora G. Altes P. Pons L. Llagostera S. Fibrin associated Epstein–Barr virus positive large B cell lymphoma as a complication of a repaired thoraco-abdominal aortic aneurysm.EJVES Vasc Forum. 2020; 47: 64-68Abstract Full Text Full Text PDF Scopus (0) Google Scholar (Table 1). In general, FA-LBCL does not present with specific symptoms, and favorable clinical outcomes have been reported with surgical resection alone. However, FA-LBCL arising in vascular prostheses causes thromboembolic events such as cerebral infarction2Miller D.V. Firchau D.J. McClure R.F. Kurtin P.J. Feldman A.L. Epstein–Barr virus-associated diffuse large B-cell lymphoma arising on cardiac prostheses.Am J Surg Pathol. 2010; 34: 377-384Crossref PubMed Scopus (50) Google Scholar or lower-limb ischemia,3Maedeh M. Francisco T. Sunny K. Craig O. Jessica D. Guang F. Recurrent fibrin associated diffuse large B-cell lymphoma: a case report.Hum Pathol Case Rep. 2021; 25: 200538Crossref Scopus (0) Google Scholar as well as anastomotic pseudoaneurysms or multiple visceral aneurysms, which lead to aneurysm rupture and death.4Zamora G. Altes P. Pons L. Llagostera S. Fibrin associated Epstein–Barr virus positive large B cell lymphoma as a complication of a repaired thoraco-abdominal aortic aneurysm.EJVES Vasc Forum. 2020; 47: 64-68Abstract Full Text Full Text PDF Scopus (0) Google Scholar The optimal treatment for FA-LBCL within vascular prostheses has not been established; 2 of 5 cases with surgical resection resulted in recurrent disease and 1 of 3 cases treated with surgical resection followed by adjuvant chemotherapy revealed persistent disease. Two patients who did not undergo replacement of vascular prosthesis died from aneurysm rupture4Zamora G. Altes P. Pons L. Llagostera S. Fibrin associated Epstein–Barr virus positive large B cell lymphoma as a complication of a repaired thoraco-abdominal aortic aneurysm.EJVES Vasc Forum. 2020; 47: 64-68Abstract Full Text Full Text PDF Scopus (0) Google Scholar or thromboembolism. In our case, FA-LBCL was found around the vascular prosthesis, and it could not be confirmed with any degree of certainty that the aneurysmal sac enlargement after endovascular aortic repair was caused by FA-LBCL because histopathologic examination was not performed. It mimicked perigraft seroma or recurrent AAA after aortic replacement, and differentiation based on image findings alone was difficult because of the high intraluminal pressure of the aneurysmal sac that was expected from the enlargement of the aneurysmal sac and narrowing of the vascular prosthesis. The tumor has been growing after completion of a course of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy, indicating that the lesion may be immunologically isolated and might be rendering chemotherapy ineffective.3Maedeh M. Francisco T. Sunny K. Craig O. Jessica D. Guang F. Recurrent fibrin associated diffuse large B-cell lymphoma: a case report.Hum Pathol Case Rep. 2021; 25: 200538Crossref Scopus (0) Google Scholar We believe that the optimal management plan would be tumor resection and a new aortic prosthesis replacement. In patients with apparently idiopathic persistent enlargement of the aortic aneurysmal sac after aortic replacement, the possibility of perigraft FA-LBCL should be considered and appropriately investigated. Further evaluation is required to determine the overall clinical behavior and optimal treatment.Table 1Cases of FA-LBCL associated with vascular prostheses reported in the literatureCase no.DateAuthorsAge, ySexPresentationDuration from the last surgeryInterventionOutcome (duration from the intervention)12010Miller et al2Miller D.V. Firchau D.J. McClure R.F. Kurtin P.J. Feldman A.L. Epstein–Barr virus-associated diffuse large B-cell lymphoma arising on cardiac prostheses.Am J Surg Pathol. 2010; 34: 377-384Crossref PubMed Scopus (50) Google Scholar48MIschemic stroke due to ascending aortic graft thrombosis24 yRedo ascending aortic graft replacementRecurrence (3 y)22010Miller et al2Miller D.V. Firchau D.J. McClure R.F. Kurtin P.J. Feldman A.L. Epstein–Barr virus-associated diffuse large B-cell lymphoma arising on cardiac prostheses.Am J Surg Pathol. 2010; 34: 377-384Crossref PubMed Scopus (50) Google Scholar79FAnastomotic pseudoaneurysm after ascending aortic graft replacement5 yRedo ascending aortic graft replacementDied from surgical complications (N/A)32012Gruver5Gruver A.M. Huba M.A. Dogan A. Hsi E.D. Fibrin-associated large B-cell lymphoma: part of the spectrum of cardiac lymphomas.Am J Surg Pathol. 2012; 36: 1527-1537Crossref PubMed Scopus (31) Google Scholar55MIschemic stroke due to ascending aortic graft thrombosis4 yRedo ascending aortic graft replacement followed by 8 cycles of R-CEOPNo recurrence (16 mo)42017Bell6Bell D. Marshman D. Diffuse large B cell lymphoma in a prosthetic aortic graft.Heart Lung Circ. 2017; 26: 4-6Abstract Full Text Full Text PDF Scopus (6) Google Scholar70MIschemic stroke due to ascending aortic graft thrombosis10 yRedo ascending aortic graft replacement followed by chemotherapyNo recurrence (N/A)52017Boyer7Boyer D.F. McKelvie P.A. de Leval L. Edlefsen K.L. Ko Y.H. Aberman Z.A. et al.Fibrin-associated EBV-positive large B-Cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-Cell lymphoma associated with chronic inflammation.Am J Surg Pathol. 2017; 41: 299-312Crossref PubMed Scopus (50) Google Scholar56MAneurysm after abdominal aortic graft replacement1 yRedo abdominal aortic graft replacement followed by 6 cycles of R-CHOP with methotrexateRecurrence (24 mo)62017Boyer7Boyer D.F. McKelvie P.A. de Leval L. Edlefsen K.L. Ko Y.H. Aberman Z.A. et al.Fibrin-associated EBV-positive large B-Cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-Cell lymphoma associated with chronic inflammation.Am J Surg Pathol. 2017; 41: 299-312Crossref PubMed Scopus (50) Google Scholar68MLower-limb ischemia due to endovascular graft thrombosis after EVAR7 yArtery thrombectomy followed by 2 cycles of R-CHOPDied from ischemic stroke (10 mo)72017Boyer7Boyer D.F. McKelvie P.A. de Leval L. Edlefsen K.L. Ko Y.H. Aberman Z.A. et al.Fibrin-associated EBV-positive large B-Cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-Cell lymphoma associated with chronic inflammation.Am J Surg Pathol. 2017; 41: 299-312Crossref PubMed Scopus (50) Google Scholar71MHematoma around graft after aortobifemoral bypass6 yRedo aortobifemoral bypassNo recurrence (10 mo)82020Zamora et al4Zamora G. Altes P. Pons L. Llagostera S. Fibrin associated Epstein–Barr virus positive large B cell lymphoma as a complication of a repaired thoraco-abdominal aortic aneurysm.EJVES Vasc Forum. 2020; 47: 64-68Abstract Full Text Full Text PDF Scopus (0) Google Scholar62MAnastomotic pseudoaneurysm and multiple visceral aneurysms after thoracic aortic graft replacement15 moTEVAR for anastomotic pseudoaneurysm and coil embolization for visceral aneurysmsDied from multiple visceral aneurysm ruptures (3 mo)92021Maedeh et al3Maedeh M. Francisco T. Sunny K. Craig O. Jessica D. Guang F. Recurrent fibrin associated diffuse large B-cell lymphoma: a case report.Hum Pathol Case Rep. 2021; 25: 200538Crossref Scopus (0) Google Scholar48MAneurysmal sac enlargement after EVAR/lower-limb ischemia due to abdominal aortic graft thrombosis6 y/10 moAbdominal aortic graft replacement/artery thrombectomy and bypass followed by R-CHOPRecurrence after aortic replacement (10 mo)/persistent (N/A)102022Yamamoto et al1Yamamoto Y. Bhunchet E. Uchiyama H. Oonuki M. Fibrin-associated diffuse large B-cell lymphoma arising in an abdominal aortic graft: a case report and literature review.Ann Vasc Surg. 2022; 2: 100050Google Scholar72MAbdominal aortic graft thrombosis3 yRedo abdominal aortic graft replacementDied from intracranial hemorrhage with no evidence of disease (3 mo)M, Male; F, female; N/A, not available; R-CEOP, rituximab, cyclophosphamide, etoposide, vincristine, and prednisone; EVAR, endovascular aortic repair; TEVAR, thoracic endovascular aortic repair; FA-LBCL, fibrin-associated large B-cell lymphoma. Open table in a new tab M, Male; F, female; N/A, not available; R-CEOP, rituximab, cyclophosphamide, etoposide, vincristine, and prednisone; EVAR, endovascular aortic repair; TEVAR, thoracic endovascular aortic repair; FA-LBCL, fibrin-associated large B-cell lymphoma. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiI0ZDk4NGQ1ZDI5OTk5OGE1OTZhOWFmZjc1YjU2NDczMiIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4ODc5MzYyfQ.rz7qK-x3eYNtyp-FYnJKEFvS0lp6SANRkV99chjVkYHBiAgpesllP_SaZkg9GvYwkQgwhM-nnHkDszEr16sjcwgB8-hFiZvXe2g_Zmhce_gxxmaVsFxiIsUTBJzav7CU3HLK9EaSSXWkQYG9YQCDo_wUt7bIautnyf-61wNvQrBu_CKm2gWEsuZNv72E6jYLgb7QQ5BvA8xKyV-Yq4DDLAlKsLOo0KbvfWUdkA0hy-ZdFv-GZ8gEbpMSIkPl1lCsmTf5qLcOoTMbIl6DM2yoBAf634eMruaXD7h83510t_Or6um5dQtamOgqG2vh0h4eCxQQYn7kPVRFR8F0XPcnAA Download .mp4 (2.38 MB) Help with .mp4 files Video 1Enhanced CT scan 3 years after aortic replacement showing the aortic aneurysmal sac covering the vascular prosthesis has become enlarged, which causes the vascular prosthesis to be compressed forward and its lumen to be narrowed. There is no contrast effect within the aortic aneurysmal sac. Video available at: https://www.jtcvs.org/article/S2666-2507(23)00048-2/fulltext. Download .jpg (.08 MB) Help with files Video 1Enhanced CT scan 3 years after aortic replacement showing the aortic aneurysmal sac covering the vascular prosthesis has become enlarged, which causes the vascular prosthesis to be compressed forward and its lumen to be narrowed. There is no contrast effect within the aortic aneurysmal sac. Video available at: https://www.jtcvs.org/article/S2666-2507(23)00048-2/fulltext.