Abstract
BackgroundPrimary cardiac lymphoma (PCL) is a rare neoplasm, defined as lymphoma with the main bulk localized in the heart; diffuse large B cell lymphoma (DLBCL) is the most common type. It usually involves the pericardium and the right heart and has a poor prognosis with a median survival of less than 1 year.Case presentationA 62-year-old female presented to the emergency department for palpitations and recent-onset dyspnea. The echocardiogram showed a round-shaped mass (33 x 32 mm) in the left atrium, a mild circumferential pericardial effusion, and a hyperechogenic mass at the level of the atrio-ventricular junction. A thoracic CT scan demonstrated the presence of a bulky mediastinal mass, strictly attached to the left heart and infiltrating its posterior wall, the left pulmonary veins, and the inferior lobar bronchus. The patient underwent CT-guided biopsy, and the diagnosis of double-expressor DLBCL was made. Given the potential risk of heart rupture during chemotherapy, the first cycle of R-CHOP was performed in an in-hospital setting, with initial benefit. After completing 6 cycles of R-CHOP, imaging studies showed rapid progression of the disease; the patient was started on the salvation protocol R-DHAOX but died of septic shock 10 months later.ConclusionsPCL is rare and accounts for less than 2% of primary cardiac tumors; double-expressor DLBCL carries a poor prognosis.As in most cases, the diagnosis was made after the onset of nonspecific symptoms (dyspnea) but—despite the strong predilection for right heart involvement reported in literature—our patient had a predominant left atrial infiltration.
Highlights
ConclusionsPrimary cardiac lymphoma (PCL) is rare and accounts for less than 2% of primary cardiac tumors; double-expressor diffuse large B cell lymphoma (DLBCL) carries a poor prognosis
Primary cardiac lymphoma (PCL) is a rare neoplasm, defined as lymphoma with the main bulk localized in the heart; diffuse large B cell lymphoma (DLBCL) is the most common type
Primary cardiac lymphoma (PCL) is a rare entity accounting for less than 2% of primary cardiac tumors: it lacks a univocal definition, and the evidence in literature derives almost exclusively from case reports [1–13]
Summary
PCL is a rare and not well-defined malignancy that accounts for less than 2% of primary cardiac tumors; double-expressor DLBCL carries a poor prognosis. The overall prognosis is poor, with a median survival of less than 1 year after diagnosis. Nonspecific signs and symptoms, the rapid evolution of cardiac involvement, and late diagnosis are major factors affecting the outcome. In our patient, the diagnosis was made after the onset of nonspecific symptoms (dyspnea); despite the strong propensity for right heart involvement reported in the literature, our patient had a rather unusual presentation with a predominant left atrial infiltration. Individualized strategies are often necessary for treating these patients due to the lack of consistent evidence in the literature and based on the specific characteristics and localization of the tumor
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