Abstract
A 41-year-old woman presented acute cerebral infarction. Transesophageal echocardiography revealed multiple masses only on both surfaces of the aortic valve cusps. There was no primary lesion outside the heart according to various examinations. After treatment for cerebral infarction, we replaced the aortic valve instead of preservation because the intraoperative histological examination reported that malignancy was highly suspected. Contrary to the rapid frozen section diagnosis, histological and immunohistochemical examinations failed to exhibit malignancy. The tumors were composed of atypical large lymphoid cells and they were assessed to be related to T-/natural killer-cells. Furthermore, Epstein–Barr virus related markers were also positive. Her three-year postoperative course was uneventful without chemotherapy. We report an extremely rare case of Epstein–Barr virus-associated T-/natural killer-cell lymphoproliferative disease which formed multiple small tumors on both surfaces of the aortic valve.
Highlights
Epstein–Barr virus (EBV) is quite the common virus
EBV has been involved in the occurrence of a wide range of B-cell lymphoproliferative disorders (LPD) and T/ NK-cell lymphomas
We report an extremely rare case in which EBV-associated cytotoxic T/NK-cell LPD (EBV–T/ NK LPD) formed aortic valve tumors
Summary
Epstein–Barr virus (EBV) is quite the common virus. EBV has been involved in the occurrence of a wide range of B-cell lymphoproliferative disorders (LPD) and T/ NK-cell lymphomas. Since tumor malignancy was strongly suspected, we removed the cusps and replaced the valve with a Carpentier-Edwards PERIMOUNT Magna Ease pericardial aortic bioprosthesis with ThermaFix process, 21 mm (Edwards Lifesciences Corp, Irvine, California), instead of preservation. She chose bioprosthetic valve because she had desire to bear children. EBER-1 in situ hybridization (Fig. 2e) and LMP-1 were positive, while CD20cy, CD4, CD5, ALK, and CD56 were negative These results meant the tumor cells were infected by EBV. CD8-positive cytotoxic T-cells and NK-cells contain cytotoxic granules in their cytoplasm, showing immunohistochemically positive reaction for granzyme B (Fig. 2f) and T-cell intracytoplasmic antigen (TIA-1) (Fig. 2g) We diagnosed this tumor to have originated due to EBV-T/NK LPD. She has had no signs of recurrence three years after the cardiac operation
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