Epstein-Barr Virus-Associated Diffuse Large B-Cell Lymphoma (DLBCL) Arising on Cardiac Prostheses
DV Miller, DJ Firchau, RF McClure, PJ Kurtin, AL Feldman. Intermountain Central Lab, Salt Lake City, UT; Hennepin County Medical Examiner, Minneapolis, MN; Mayo Clinic, Rochester, MN
Background: Primary cardiac lymphoma is extremely rare and lymphoma arising in association with prosthetic valves has been described in only 3 single case reports. We describe three patients with diffuse large B-cell lymphoma (DLBCL) involving prosthetic heart valves and a synthetic tube graft.
Design: Patients with primary DLBCL involving prosthetic heart valves or synthetic grafts either treated at our institution or reviewed as a pathology consultation case were identified through a laboratory information system database query. Medical records were reviewed to systematically abstract patient demographics, presenting signs and symptoms, intraoperative findings, prosthetic valve or graft characteristics, postoperative lymphoma staging, and patient outcomes. Histologic slides were reviewed and immunohistochemistry was performed for all cases using formalin-fixed paraffin-embedded sections, including stains for: CD3, CD10, CD20, BCL-2, BCL-6, MUM1, and HHV-8. In-situ hybridization for EBV EBER mRNA was also performed on paraffin sections.
Results: Three patients were identified with DLBCL involving a bioprosthetic aortic valve, a mechanical aortic valve, and a composite ascending aortic graft. All specimens showed shallow layering of acellular fibrinous debris over the prosthetic or synthetic materials, with neoplastic lymphocytes present at the luminal surface. There were frequent mitoses and abundant karyorrhectic debris. All demonstrated a non-germinal center B-cell phenotype. All three cases were positive for Epstein-Barr virus, but there was no staining for HHV8. There was no other evidence of distant disease at the time of diagnosis and no recurrence or dissemination occurred after surgical removal of the prosthesis, though follow-up was limited.
Conclusions: Based on 2008 WHO diagnostic criteria, we believe these cases should be classified as DLBCL associated with chronic inflammation (DLBCL-CI). However, unlike the characteristically poor prognosis reported in this entity, we hypothesize that the disease resectability in these cardiac sites, in many cases, may allow for a better prognosis than DLBCL-CI at other less resectable sites.
Wednesday, March 24, 2010 1:00 PM
Poster Session VI # 32, Wednesday Afternoon