Heart of Lymphoma: Primary Mediastinal Large B-Cell Lymphoma with Endomyocardial Involvement

Abstract

Primary mediastinal B-cell lymphoma (PMBCL) is an uncommon aggressive subset of diffuse large B-cell lymphomas. Although PMBCL frequently spreads locally from the thymus into the pleura or pericardium, it rarely invades directly through the heart. Herein, we report a case of a young Mexican female diagnosed with PMBCL with clear infiltration of lymphoma through the cardiac wall and into the right atrium and tricuspid valve leading to tricuspid regurgitation. This was demonstrated by cardiac MRI and transthoracic echocardiogram. In addition, cardiac MRI and CT scan of the chest revealed the large mediastinal mass completely surrounding and eroding into the superior vena cava (SVC) wall causing a collar of stokes. The cardiac and SVC infiltration created a significant therapeutic challenge as lymphomas are very responsive to chemotherapy, and treatment could potentially lead to vascular wall rupture and hemorrhage. Despite the lack of conclusive data on chemotherapy-induced hemodynamic compromise in such scenarios, her progressive severe SVC syndrome and respiratory distress necessitated urgent intervention. In addition to the unique presentation of this rare lymphoma, our case report highlights the safety of R-CHOP treatment.

Cardiac Magnetic Resonance Evaluation of Cardiac Masses in Patients with Suspicion of Cardiac Masses on Echo or Computed Tomography

“…Cardiac lymphomas usually present as large, bulky masses with vascular encasement without obliterating the lumen. [ 5 , 24 ] In our study, one patient was probably having the cardiac lymphoma based on CMR features. This was a 68-year-old man with a history of weight loss, night sweats, and shortness of breath.…”

Left ventricular rigid body rotation in a diffuse large B-cell lymphoma patient with cardiac involvement: A case from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study

“…Histology confirmed a CD20-positive primary mediastinal lymphoma, which is considered a subset of diffuse large B-cell lymphoma and is an uncommon clinical and pathological entity in the World Health Organization classification of lymphomas. 1,4 Chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine and methylprednisolone (R-CHOP) was started according to international recommendations. 9 R-CHOP includes rituximab, an anti-CD20 antibody, an essential part of the treatment.…”

“…Primary mediastinal B-cell lymphoma is an uncommon aggressive subset of diffuse large B-cell lymphoma. 1 It frequently spreads locally from the thymus into the pleura and the pericardium, but rarely invades directly through the heart. 1 It occasionally manifests as an intracardiac mass, often diagnosed on autopsy, and 90% are clinically silent.…”

“…1 It frequently spreads locally from the thymus into the pleura and the pericardium, but rarely invades directly through the heart. 1 It occasionally manifests as an intracardiac mass, often diagnosed on autopsy, and 90% are clinically silent. 2,3 This type of lymphoma is more prevalent in women, mainly in the third and fourth decades of life, and represents 2-3% of non-Hodgkin's lymphomas.…”

“…2,3 This type of lymphoma is more prevalent in women, mainly in the third and fourth decades of life, and represents 2-3% of non-Hodgkin's lymphomas. 1,4 It usually presents with systemic symptoms (weight loss, fever, night sweats), shortness of breath, chest discomfort, and palpable lymph nodes.…”