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Published on: August 8, 2022
Primary cardiac lymphoma presenting as an atypical type of hypertrophic cardiomyopathy
Dong-Hyeok Kim1, Yong-Hyun Kim, Woo-Hyuk Song
1Division of Cardiology, Department of Internal Medicine, Korea University Ansan Hospital, Ansan, Korea.
Insights
Primary cardiac lymphoma (PCL) can mimic hypertrophic cardiomyopathy, delaying diagnosis. Early detection via prompt thoracotomy is crucial for this rare cardiac malignancy.
Area of Science:
- Oncology
- Cardiology
- Hematology
Background:
- Primary cardiac lymphoma (PCL) is an exceptionally rare cardiac malignancy.
- Cardiac involvement is more common in disseminated lymphomas than primary tumors.
Observation:
- A 43-year-old man presented with PCL initially misdiagnosed as hypertrophic cardiomyopathy due to diffuse cardiac wall thickening.
- Diagnosis was delayed by 9 months until atypical lymphocytes (CD79a+, CD20+) were found in a growing mediastinal mass.
Findings:
- PCL can present as diffuse infiltrative cardiac disease without a distinct mass.
- Treatment with anthracycline-based chemotherapy and anti-CD20 immunotherapy led to significant reduction in cardiac and mediastinal masses.
Implications:
- Highlights the importance of considering PCL in unexplained cardiac thickening, even without a mass.
- Emphasizes the need for timely exploratory thoracotomy when PCL diagnosis remains elusive.
Abstract:
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.
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