fibrin and Lymphoma--B-Cell

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.

Topics: Adult; Aged; Biomarkers, Tumor; Case-Control Studies; Chronic Disease; Epstein-Barr Virus Infections; Female; Fibrin; Follow-Up Studies; Humans; Immunohistochemistry; Inflammation; Lymphoma, B-Cell; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Prognosis

An immunocompetent 29-year-old male presented with an embolic stroke from an unusual primary cardiac lymphoma. The cardiac lesion consisted of a polypoid, left atrial, mural fibrin thrombus with anaplastic tumor cells lining the surface of the clot. Histologic, immunophenotypic, and molecular characterizations were consistent with a diagnosis of CD30+ large B-cell lymphoma with anaplastic cytology. While tumor emboli from invasive primary cardiac lymphomas have been reported, this noninvasive fibrin thrombus-associated lymphoma appears to be unique and previously unreported.

Topics: Adult; Diagnosis, Differential; Fibrin; Heart Atria; Heart Neoplasms; Humans; Immunophenotyping; Intracranial Embolism; Ki-1 Antigen; Lymphoma, B-Cell; Lymphoma, Large B-Cell, Diffuse; Male; Myxoma; Platelet Count; Stroke; Ultrasonography

Malignant angioendotheliomatosis is a rare intravascular (angiotropic) lymphoma. Patients most often present with cutaneous or central nervous system findings. We describe three patients with malignant angioendotheliomatosis involving the skin. The initial lesions in each were tender, indurated nodules on the lower extremities, resembling inflammatory panniculitis. Skin biopsies and immunohistochemical studies from all patients confirmed intravascular B-cell lymphoma. Two patients had visceral involvement, and molecular genetics studies showed clonal immunoglobulin gene rearrangement in one. Electron microscopy in this case showed increased fibrin and atypical lymphocytes within blood vessels. Malignant angioendotheliomatosis is a monoclonal intravascular lymphoma, usually of B-cell phenotype. Occlusion of small blood vessels with lymphoid cells, fibrin, and degenerating cellular debris causes the cutaneous lesions. An excisional biopsy through the depth of subcutaneous tissue may be necessary to confirm the diagnosis of malignant angioendotheliomatosis.

Topics: Aged; Aged, 80 and over; Blood Vessels; Female; Fibrin; Gene Rearrangement; Genes, Immunoglobulin; Humans; Lymphocytes; Lymphoma, B-Cell; Lymphoma, Non-Hodgkin; Male; Microscopy, Electron; Middle Aged; Molecular Biology; Phenotype; Skin Neoplasms