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Table 1 Demographic data, clinical data, and characteristics of reported cases of Fibrin-Associated Diffuse Large B-Cell Lymphoma

From: Fibrin-associated large B-cell lymphoma: first case report within a cerebral artery aneurysm and literature review

SITE/REF.

AGE SEX

Immunosupp

CLINICAL FEATURES

HISTOLOGY

IIC/EBV/CLONALITY

STAGING THERAPY

FOLLOW-UP

Atrial myxoma Bagwan 2009 (ref [2])

81/M

negative

Multiple cerebral strokes

Foci of large lymphoid cells at myxoma surface

CD20+, CD79α+, CD10+, BCL6+, BCL2+, CD3-. Ki67:80% EBV: NV. Ig clonality NP.

NS Staging: neg; BM: neg. Surgery+ R-CHOP

NA

Atrial myxoma Dimitrova 2010 (ref [3])

51/M

negative

Acute obstructive left heart failure

Foci of large lymphoid cells at myxoma surface

CD20+, CD10+. Ki67 high EBV: NV. Ig clonality NP.

Imaging/BM Staging: neg. Surgery+ CHOP (VI)

NA

Atrial myxoma Loong 2010 (ref [4])

70/F

negative

Ischemic stroke

Foci of large lymphoid cells

CD20+, CD79α+, PAX5+, CD43+, MUM1+, CD10-, BCL6+, BCL2+, CD30+, CD138-, HHV8-, CD3-. Ki67 100%. LMP1+, EBNA2+, EBER+. Ig clonality +.

CT/BM Staging: neg. Surgery + R-CEOP (IV)

Died for CH complications (neutropenia+ pneumonia) at 5 mo. No autopsy

Atrial myxoma Svec 2012 (ref [5])

60/F

negative

Embolic brain stroke

Foci of large lymphoid cells

CD20+, CD79α+, PAX5+, CD10-MUM1+, CD23+, BCL2+, BCL6-, CD5-, CD3-, cyclin D1-, CD138-, CD38-; Ki67: 100%. LMP1+, EBER+, EBNA2+. FISH MYC, BCL2, BCL6 -. Ig clonality NP.

CT/PET/BM Staging: neg. Surgery+ R-CHOP (VI)

NED at 7 mo

Atrial myxoma Bartoloni 2013 (ref [6])

55/F

negative

Fatigue, fever

Foci of large lymphoid cells at myxoma surface

LCA+, CD20+, CD79α+, MUM1+, HHV8-, CD3-, CD5-. Ki67: 90%. LMP1+, EBNA2-, EBER+ Ig clonality NP.

CT/BM staging: neg. Surgery only

NED at 72 mo

Atrial myxoma Aguilar 2015 (ref [7])

52/M

negative

Dysarthria and hemiplegia

Foci of large lymphoid cells

CD20+, CD79α+, PAX5+, CD30+, MUM1+, ALK-1-, CD10-CD43-, cyclinD1-, CD3-, LMP1+, EBNA2+, EBER+. Ig clonality +.

CT/BM staging: neg. Surgery only

NED at 42 mo

Atrial myxoma Tapan 2015 (ref [8])

49/M

negative

Palpitations

Foci of large lymphoid cells

CD20+, CD79α+, CD30+, MUM1+, CD3-, CD5-, CD10-, CD138-, cyclin D1-, ALK1-, EMA-. Ki67 80%. EBNA2+, EBER+. Ig clonality NP.

NS Staging: neg; BM neg. Surgery + R-CHOP

NED at 12 mo

Atrial myxoma Boyer 2017 (ref [9])

54/F

negative

Syncope

Foci of large lymphoid cells

CD20+, PAX5+, CD79α+, BCL6+, CD30+, CD10-, CD138- CD3-, HHV8-, Ki67 80%. EBER+. Ig clonality NP.

NS Staging: neg. Surgery/ Other therapy: NA

NED at 130 mo

Atrial myxoma Boyer 2017 (ref [9])

55/F

negative

Syncope, cough, dyspnea

Foci of large lymphoid cells

CD20+, PAX5+, CD79α+, BCL6+, MUM1+, CD10-, CD45+, CD30+, HHV-8-, CD138-, CD3-. Ki67: > 95%. EBER+, LMP1+, EBNA2+. FISH for MYC-. Ig clonality NP.

NS Staging: neg. Surgery only

Died at 2 mo for cardiac cause. Autopsy: No lymphoma

Atrial myxoma Boyer 2017 (ref [9])

54/M

negative

Dyspnea, respiratory failure

Foci of large lymphoid cells

CD20+, PAX5+, BCL6+, MUM1+, CD10-, CD38+, CD45+, CD30+, CD3-. Ki67:90% EBER+, LMP1+, EBNA2+. FISH MYC, BCL6, BCL2 -. Ig clonality NP.

CT/BM Staging: neg. Surgery only

Recurrent FA-DLBCL at mitral valve after 25 mo. Died at 26 mo (embolic stroke). No autopsy.

Atrial myxoma Yan 2017 (ref [9])

54/M

negative

Congestive heart failure

Foci of large lymphoid cells within fibrin

CD20+, CD79α+, MUM1+, CD10-, BCL6+, CD30+. ALK-, BCL2+, CD3-, CD5-, Ki67 90% LMP1+, EBNA-2+, EBER+, FISH for MYC, Bcl6, BCL2-. Ig clonality NP.

CT/BM Staging: neg. Surgery only

NED at 7 MO

Atrial myxoma Yan 2017 (ref [10])

61/F

negative

Congestive heart failure

Foci of large lymphoid cells within fibrin

CD20+, CD79α+, MUM1+, CD10+, BCL6+, CD30+. ALK-, BCL2+, CD3-, CD5-, Ki67 95% LMP1+, EBNA-2+, EBER+, FISH for MYC, Bcl6, BCL2-. Ig clonality NP.

CT/BM Staging: neg. Surgery only

NED at 84 mo

Atrial myxoma Yan 2017 (ref [10])

46/F

negative

Congestive heart failure

Foci of large lymphoid cells within fibrin

CD20+, CD79α+, MUM1+, CD10-, BCL6+, CD30+. ALK-, BCL2+, CD3-, CD5-, Ki67 90% LMP1+, EBNA-2+, EBER+, FISH for MYC, Bcl6, BCL2-. Ig clonality NP.

CT/BM Staging: neg. Surgery only

NED at 3 mo

Atrial myxoma Yan 2017 (ref [10])

46/F

negative

Congestive heart failure

Foci of large lymphoid cells within fibrin

CD20+, CD79α+, MUM1+, CD10-, BCL6-, CD30+. ALK-, BCL2-, CD3-, CD5-, Ki67 85% LMP1+, EBNA-2+, EBER+, FISH for MYC, Bcl6, BCL2-. Ig clonality NP.

CT/BM Staging: neg. Surgery only

NED at 120 mo

Atrial thrombus Qigley 2003 (ref [11])

29/M

negative

Cerebral embolic stroke

Foci of large lymphoid cells at clot’s surface

CD45+, CD20+, CD79α+, CD43+, CD30+, CD3, LMP -, HHV8-, EBER+. Clonality:κ rearrangement +. IGH -, TCR-.

Imaging/BM Staging: neg. Surgery+ R-CHOP (VI)

NED at 24 mo

Atrial thrombus Gruver 2012 (ref [12])

56/M

negative

Short breath

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+. Ig clonality +.

NS Staging: neg. Surgery+ R-CHOP (VI)

NED at 8 mo

Myxomatous mitral valve Gruver 2012 (ref [12])

75/M

negative

Dyspnea, aortic insufficiency, mitral valve regurgitation

Foci of large lymphoid cells within fibrin on mitral valve

CD20+, CD79α+, PAX5+, CD30-, CD43-, CD45+, BCL6-, MUM1+, BCL2+, CD10-, CD3-, CD5-, HHV8-, MYC -. KI67100%. LMP1-, EBNA2-. EBER-. Ig clonality +.

NS Staging: neg. Surgery+R-CVP (I) + R-CHOP (VI)

NED at 39 mo

Prosthesis (knee) Cheuk 2005 (ref [13])

78/M

negative

Pain at knee prosthesis (implanted 22 yrs. before)

Foci of large lymphoid cells within fibrin and necrosis

CD20+, CD79α+, CD138+/−. CD2-, CD3-, CD5-, CD10-, BCL6-, HHV8-. Ki67:70%. LMP1+, EBER+. Ig clonality +.

NS Staging: neg. Surgery+RT

NED at 24 mo

Prosthesis (aortic valve) Bagwan 2009 (ref [2])

50/M

negative

Symptoms of aortic regurgitation. Aortic valve prosthesis (16 yrs. before)

Foci of large lymphoid cells within aortic valve leaflets

CD45+, CD20+, CD79α+, CD10+, BC6+/−, BCL2+/−, Ki67:80% LMP1-. Ig clonality: NP.

NS Staging: neg; BM: neg. Surgery+ R-CHOP

Died after 6mo for prosthesis rupture. Autopsy: no lymphoma

Prosthesis (aortic valve) Berrio 2010 (ref [14])

60/M

negative

Acute left heart failure. History of aortic valve prosthesis for stenosis

Foci of large lymphoid cells within valve vegetations

CD20+, CD43+, CD3-. Ki67:80–90% EBV: NV. Ig clonality: NP.

NS Staging: neg. Surgery only

Died for tricuspidal endocarditis, pneumonia 2 yrs. later. No autopsy.

Prosthesis (aortic graft) Miller 2010 (ref [15])

48/M

negative

Ischemic attack. Marfan sy. Asc.a. aneurysm graft+ aortic valve prosthesis (24 yrs. before)

Foci of large lymphoid cells within fibrin

CD20+, MUM1+, CD10-, BCL6- BCL2+, CD3-, HHV8-. EBER+. Ig clonality +.

CT/PET/BM Staging: neg. Surgery only

NED at 6 mo

Prosthesis (aortic valve) Miller 2010 (ref [15])

80/F

negative

Heart failure. Aortic valve prosthesis (8 yrs. before)

Foci of large lymphoid cells within fibrin

CD20+, MUM1+, CD10-, BCL6-BCL2-, CD3-, HHV8-, EBER+. Ig clonality +.

CT/PET/BM Staging: neg. Surgery only

Died (for breast cancer 18 mo after aortic valve surgery). No autopsy.

Prosthesis (aortic graft) Miller 2010 (ref [15])

79/F

negative

Short breath, thoracic pulsing sensation. Tube graft for asc. a. dissection (5 yrs. before)

Foci of large lymphoid cells within fibrin

CD20+, MUM1+, CD10-, BCL6+, BCL2+, CD3-, HHV8-, EBER+, Ig clonality +.

CT/PET/BM Staging: neg. Surgery only

Died for surgical complications. No autopsy

Prosthesis (aortic graft) Gruver 2012 (ref [12])

55/M

negative

Stroke. Aortic graft for aneurysm (4 yrs. before)

Foci of large lymphoid cells within thrombus

CD20+, CD79α+, PAX5+, CD30+, CD43+, CD45+, BCL6+, MUM1+, BCL2-, CD10-, CD3-, CD5-, HHV8-, MYC-; KI67 100%. LMP1+, EBNA2 + .EBER+. Ig clonality +.

NS Staging: neg. Surgery + R-CEOP (VIII)

NED at 16 mo

Prosthesis (vascular graft) Boyer 2017 (ref [9])

56/M

negative

IR aorta+ CIA aneurysms. TAA aneurysm graft + thrombectomy (1 yr. before). Asc a. dissection graft (9 yrs. before).

Foci of large lymphoid cells within thrombus of IR aorta and CIA aneurysms. In retrospect foci within thrombus of TAA aneurysm

CD20+, PAX5+, BCL6-, MUM1+, CD10-, CD138-, HHV8-, CD30+, KI67: 95%. EBER+, LMP1+, EBNA2+. FISH for MYC -. Ig clonality +.

CT/PET/BM Staging: neg. Surgery+ R-CHOP (VI) + IT MTX

AWSD at 24 mo. Surgical revision of aortic graft: persistent foci of EBV+ large B cell.

Prosthesis (vascular graft) Boyer 2017 (ref [9])

68/M

negative

Lower limbs ischemia. AA aneurysm repair with IR graft (7 yrs. before).

Foci of large lymphoid cells within thrombus

CD20+, PAX5+, BCL6+, CD10-MUM1+, CD30+, HHV8-, KI67 90%, EBER+, LMP1+, EBNA2+. FISH for MYC -. Ig clonality NP.

CT/PET Staging neg. 3 mo after: PET/CT/biopsy: foci of EBV+ cells near adrenal gland. R-COEP (II)

Died at 10 mo for embolic stroke. No progressive lymphoma. No autopsy

Prosthesis (vascular) Boyer 2017 (ref [9])

71/M

MG for THY treated with surgery+ steroids+ AZA

AF graft (6 yrs. before).

Foci of large lymphoid cells within thrombus associated with graft

CD20+, CD79α+, PAX5+, CD10-BCL6+, MUM1+, CD30+, CD45+, CD138-, HHV8-, KI67 > 95%, EBER+, LMP1+. Ig clonality +.

NS Staging: neg. Surgery only

NED at 10 mo

Pseudocyst (kidney) Lee 2009 (ref [16])

61/M

negative

Renal cyst (for 20 yrs)

Foci of large lymphoid cells within necrosis

CD22+, CD45+, CD79α+, MUM1+, PAX5+, CD3-, CD10-, CD20-, CD138-, BCL6-, ALK1-, HHV8-, κ-, λ-, EBER+. Ig clonality NP.

Staging NA. Surgery+ CHOP (VI)

NA

Pseudocyst (spleen) Loong 2010 (ref [4])

29/M

negative

Abdominal pain

Foci of large lymphoid cells within necrosis

CD20+, CD79α+, PAX5+, CD43+, MUM1+, CD10-, BCL6-CD138-, BCL2+, CD30-, HHV8-, CD3-. Ki67 90%. LMP1+, EBNA2+, EBER+. Ig clonality +.

PET/BM Staging: neg. Surgery (splenectomy) + R (IV)

NED at 6 mo

Pseudocyst (kidney) Valli 2011 (ref [17])

46/M

negative

Left-sided flank pain

Foci of large lymphoid cells within necrosis

CD20+, MUM1+, CD10-, BCL6-BCL2+, CD30-, HHV8-;Ki67:90%. EBER+. Ig clonality NP.

CT/PET/BM Staging: neg. Surgery+ R-CHOP (VI)

NED at 1 mo

Pseudocyst (adrenal gland) Boroumand 2012 (ref [18])

63/F

negative

Right abdominal pain

Foci of large lymphoid cells within fibrin

CD20+, CD79α+, PAX5+, MUM1+, BCL2+, CD3-, CD10-, CD30-, BCL6-, HHV8-. Ki67 > 90%. LMP1-; EBER+. Ig clonality NP.

NS Staging: neg. Surgery + R-CHOP (VI) + RT

NED at 40 mo

Pseudocyst (testis) Boroumand 2012 (ref [18])

27/M

negative

R. scrotal swelling. Herniorraphy followed by l. scrotal hematoma (removed 3 yrs. before)

Foci of large lymphoid cells within fibrin

CD20+, CD79α+, CD30+, MUM1+, BCL2+, CD3-, CD10- BCL6-, HHV8-. Ki67 > 90%. LMP1+, EBER+. Ig clonality NP.

NS Staging: neg. Surgery only

NED at 9 mo

Pseudocyst (spleen) Boyer 2017 (ref [9])

37/F

negative

Splenic mass (9 cm), incidentally found

Foci of large lymphoid cells within fibrin

CD20+, PAX5+, MUM1+, CD10-BCL6-, CD30-, CD45+, KI67 > 90% EBER+. Ig clonality NP.

CT/PET/BM Staging: neg. Surgery + R-CHOP (III)

NED at 32 mo

Pseudocyst (retrop.) Boyer 2017 (ref [9])

73/M

negative

Femoral a. aneurysm repair

Foci of large lymphoid cells within fibrin

CD20+, PAX5+, CD79α+, BCL6-, CD10-, MUM1+, CD30+, CD45+, HHV8-, KI67 > 95%, EBER+. Ig clonality NP.

CT/BM Staging: neg. Surgery+ R-CHOP (VI)

NED at 43 mo

Pseudocyst (adrenal gland) Boyer 2017 (ref [9])

70/M

negative

Adrenal mass (7 cm) causing bladder obstruction

Foci of large lymphoid cells within fibrin

CD20-, PAX5+, CD79α+, BCL6-, CD10-, MUM1+, CD45+, CD30+, CD138-, HHV8-, KI67 > 90%, LMP1-, EBNA2+, EBER+. FISH for MYC -. Ig clonality NP.

CT/PET Staging: neg. Surgery only

NED at 14 mo

Pseudocyst (retrop.) Boyer 2017 (ref [9])

44/M

negative

Right flank pain

Foci of large lymphoid cells within fibrin

CD20+, PAX5+, CD10-, BCL6-, MUM1+, CD45+, CD30-, KI67 40%, LMP1+, EBNA2+, EBER+, FISH for MYC -. Ig clonality +.

BM/imaging Staging: neg. 5-CHOP

NED at 84 mo

Pseudocyst (adrenal gland) Zanelli 2019 (ref [19])

71/F

negative

Lower limbs edema+ abdominal distension

Foci of large lymphoid cells within fibrin

CD20+, PAX5+, CD30+, MUM1+, CD10-, BCL6-, EBER+, Ki67 90%. Ig clonality NP.

CT Staging: neg. Surgery only

NED at 6 mo

Teratoma (ovary) Valli 2014 (ref [20])

56/F

negative

Abdominal pain+ swelling

Foci of large lymphoid cells

CD20+, MUM1+, CD45+, PAX5+, CD30-, BCL6-, CD10-, CD3-, CD2-, HHV8-, CD138-. Ki67: 80%. EBER+. Ig clonality +.

CT/PET Staging: neg. Surgery+ R-CHOP (VI)

NED at 8 mo

Hydrocele (testis) Loong 2010 (ref [4])

88/M

negative

Fever, scrotal pain, swelling

Foci of large lymphoid cells within necrosis

CD20+, CD79α+, PAX5+, MUM1+, CD10-, BCL6-, CD138-, BCL2+, CD30-, HHV8-, CD3+, CD2-, CD5-, CD7-. Ki67 70% LMP1+, EBNA2+, EBER+. Ig clonality -.

Staging NA. Surgery only (Orchidectomy)

NA

Hematoma (testis) Boyer 2017 (ref [9])

79/M

negative

Testicular trauma (5 yrs. before)

Foci of large lymphoid cells within hematoma

CD20+, PAX5+, CD79α+, CD10-CD138-, BCL6-, MUM1+, CD45+, CD30+, HHV8-, KI67 > 90%, EBER+, LMP1+, EBNA2+. Ig clonality +.

NS Staging: neg. Surgery only

NED. Died at 17 mo

Hematoma (thigh) Hayes 2014 (ref [21])

91/M

negative

Thigh hematoma. (6 yrs. before leg amputation for popl. a. aneurysm rupture at prior artery bypass graft site)

Foci of large lymphoid cells

CD45+, CD20+, MUM1+, CD30+, CD43+, BCL2+/−, MYC+, p53+/−, HHV8-, CD3-, CD5-, CD10-, BCL1-, BCL6-. Ki67: 90%. LMP1-, EBER+. Ig clonality NP.

NS Staging: neg. Surgery only

NED at 18 mo

Subdural hematoma Reyes 1990 (ref [22])

56/M

negative

Headaches, dizziness, unsteady gait

Foci of large lymphoid cells within fibrin, clots, necrosis

B-cell phenotype. EBV NV. Ig clonality NP.

CT/BM Staging: neg. Surgery only

NA

Subdural hematoma Sugita 2012 (ref [23])

77/M

negative

Dementia due to head trauma (20 yrs. before)

Foci of large lymphoid cells

CD20+, CD79α+, MUM1+, CD3-, BCL6-, CD10-. Ki67 high. EBNA2+, LMP1-, EBER+. Ig clonality - (rare neoplastic foci).

Imaging Staging: neg. Surgery only

NA

Subdural hematoma Kameda 2015 (ref [24])

96/M

negative

Gait disturbs+ anorexia. Trauma+ subdural hematoma (7 mo before).

Brain mass: DLBCL EBV+. Subdural hematoma: FA-DLBCL. No continuity among 2 lesions

CD20+, CD79α+, CD3-, CD4-, CD7-, CD8-, LMP1+, EBNA2+, EBER+. Ig clonality NP.

CT Staging: neg at presentation. Brain mass + subdural hematoma resection. IT MTX + cytarabine+ glucocorticoids

Died after 3 mo for lymphoma dissemination. No autopsy

Subdural hematoma Boyer 2017 (ref [9])

25/M

negative

SD hematoma since child. Hydrocephalus+ SD catheter. Steroid tp for pituitary overactivity

Foci of large lymphoid cells within hematoma

CD20+, PAX5+, MUM1+, CD10-BCL6-, CD30+, HHV8-, KI67 > 90%; EBER+, LMP1+. Ig clonality NP.

CT/PET/BM Staging: neg. Surgery only

NED at 7 mo

Arachnoid cyst Kirshenbaum 2017 (ref [25])

81/M

negative

Tremor, gait ataxia, memory disturbs

Foci of large lymphoid cells within fibrin

CD20+ CD30+, BCL2+, MUM1+, BCL6+/−, CD10-, TdT-, CD5-, cMYC+ (50%) Ki67: > 80%. EBER+. FISH MYC -. Ig clonality +.

CT/PET staging: neg. Surgery (cyst excision) + R-lenalidomide

NA

Cerebral artery aneurysm Present case

64/F

negative

Cerebral hemorrhage. Left middle cerebral artery aneurysm

Foci of large lymphoid cells within fibrin

CD20+/−, PAX5+, CD79α+, CD30+, MUM1+, CD10-, BCL6-, EBER+, Ki67 90%. Ig clonality NP.

CT/BM Staging: neg. Surgery only

NED at 5 mo

  1. Literature review of fibrin-associated diffuse large B-Cell Lymphoma
  2. A artery, AA abdominal aorta, AF, aortofemoral, Asc. A ascending aorta, AWSD alive with stable disease, AZA azathioprine, BM bone marrow, CEOP cyclophosphamide, etoposide, oncovin, prednisone, CHOP cyclophosphamide, doxorubicin, vincristine, prednisone, retro retroperitoneum, CIA common iliac arteries, CH chemotherapy, CVP cyclophosphamide, vincristine, prednisone, CT Computerized tomography, DEXA dexamethasone, F female, IT intrathecal, IR infrarenal, Ig immunoglobulin, IGH immunoglobulin heavy chain, mo months, M male, MTX methotrexate, MG myasthenia gravis, NA not available, NED not evidence of disease, Neg negative, NP not performed, NS not specified, PBL plasmablastic lymphoma, popl. A popliteal artery, R rituximab, Retrop retroperitoneum, RT radiotherapy, SD subdural, sy syndrome, TAA thoracoabdominal aorta, TCR T cell receptor, THY Thymoma, Tp therapy, yrs. years