Extranodal NK/T-cell lymphoma presenting with primary cardiac involvement

Abstract

Primary cardiac lymphoma is extremely uncommon. We report a case of a 54 year old Caucasian male with a history of non-small cell lung cancer treated by surgical resection who presented with chest pain and dyspnea on exertion. Computerized tomography (CT) imaging confirmed a 7.8×3.8 cm right atrial soft tissue mass infiltrating the lateral wall of the right atrium, and a 5 cm pericardiophrenic mass. Echocardiography confirmed a moderate pericardial effusion without tamponade physiology. Percutaneous biopsy of the pericardiophrenic mass revealed pathologic features diagnostic of NK/T-cell lymphoma. He received CHOP chemotherapy with some improvement in symptoms, but experienced radiographic progression after 2 cycles. He received palliative involved field radiotherapy but developed new sites of progressive disease within the abdomen and died shortly after completing radiotherapy. NK/T-cell lymphomas are aggressive tumors that may present with unusual extranodal disease sites. Prompt diagnosis with consideration for referral to a specialty center with experience in treatment of these rare tumors may offer the greatest potential for improving treatment outcomes.

Keywords: NK/T-cell lymphoma; Non-Hodgkin's lymphoma; cardiac.; oncological hematology.

Figure 1

Computerized tomography imaging confirms a large left pericardiac mass (top right arrow) and infiltrative mass arising from the right atrium (left arrow).

Figure 2

Extranodal NK/T-cell lymphoma, nasal type. Hematoxylin and eosin stained sections of the mediastinal mass reveals a dense infiltrate of primarily small lymphocytes with irregular nuclear contours (A) and immunohistochemistry shows the neoplastic cell co-express CD3ε (B) and CD56 (C). Almost all of the neoplastic cells contained EBV-encoded RNA (EBER) when assessed by in situ hybridization.