Primary Cardiac Lymphoma Presenting as an Atypical Type of Hypertrophic Cardiomyopathy

• Published in: Echocardiography (Mount Kisco, N.Y.) Vol. 31; no. 4; pp. E115 - E119
• Main Authors: Kim, Dong‐Hyeok, Kim, Yong‐Hyun, Song, Woo‐Hyuk, Ahn, Jeong‐Cheon
• Format: Journal Article
• Language: English
• Published: United States 01-04-2014
• Subjects: Adult; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biopsy, Needle; cardiac tumor; Cardiomyopathy, Hypertrophic - diagnosis; Delayed Diagnosis; Diagnosis, Differential; Dyspnea - diagnosis; Dyspnea - etiology; Echocardiography; Emergency Service, Hospital; Heart Neoplasms - diagnosis; Heart Neoplasms - drug therapy; Humans; hypertrophic cardiomyopathy; Immunohistochemistry; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - drug therapy; Male; mediastinal mass; Radiographic Image Enhancement; Rare Diseases; Risk Assessment; Tomography, X-Ray Computed - methods; Treatment Outcome; cardiac tumor; hypertrophic cardiomyopathy; mediastinal mass
• ISSN: 0742-2822; 1540-8175
• DOI: 10.1111/echo.12477

Primary cardiac lymphoma (PCL) is a very rare malignancy although cardiac involvement with the disseminated disease is not uncommon. We present a case of a 43‐year‐old man with PCL that initially presented as marked thickening of all cardiac walls and was mistakenly diagnosed as an atypical type of hypertrophic cardiomyopathy. The diagnosis of PCL was made with a delay of 9 months after the initial presentation, when atypical lymphocytes staining positive for CD79a and CD20 were demonstrated in the rapidly growing mediastinal and neck mass. Anthracycline‐based chemotherapy and anti‐CD20 immunotherapy resulted in a remarkable reduction in cardiac wall thickness and mediastinal mass. The first lesson to be learnt from this case is that PCL can present as a diffuse infiltrative disease without a mass. The second lesson is that prompt exploratory thoracotomy should not be delayed when the diagnosis is elusive.
Mini‐
Primary cardiac lymphoma (PCL) is a very rare malignancy, although cardiac involvement with the disseminated disease is not uncommon. We present a case of PCL that initially was mistakenly diagnosed as an atypical hypertrophic cardiomyopathy. In this case, the atypical radiological findings, and negative results of the pericardial fluid cytology and endomyocardial biopsy assessment, delayed the diagnosis of PCL. This case shows that PCL can present as a diffuse infiltrative disease, without the presence of a mass, and the importance of a prompt exploratory thoracotomy when diagnosis is elusive.