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Primary cardiac lymphoma
Shu-Ching Hsueh1, Ming-Teng Chung, Richard Fang
1Division of Hematology/Oncology, Department of Internal Medicine, Cheng-Hsin Rehabilitation and Medical Center, Taipei, Taiwan, ROC. ch1835@chgh.org.tw
Insights
Primary cardiac lymphoma (PCL) is rare, often presenting in the right atrium with varied symptoms. Early diagnosis via imaging and pathology is crucial, with chemotherapy as the primary treatment, though large tumors may have a poorer prognosis.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- Primary cardiac lymphoma (PCL) is an exceptionally rare malignancy.
- PCL predominantly affects the right atrium, presenting with diverse clinical manifestations.
- Early detection is challenging due to nonspecific symptoms and late diagnosis, often leading to a poor prognosis.
Observation:
- A 58-year-old male presented with a large (8 x 5 cm) right atrial tumor.
- Comprehensive staging revealed no distant metastases.
- Pathological examination confirmed diffuse large B-cell lymphoma.
Findings:
- Chemotherapy regimens (COP and CHOPBE) led to complete resolution of the intracardiac tumor.
- Despite treatment, the patient survived for 12 months, with radiotherapy for pericardial lesions.
- The large tumor size and extensive cardiac involvement likely influenced treatment response.
Implications:
- This case highlights the importance of considering PCL in the differential diagnosis of cardiac masses.
- Advanced imaging and biopsy are essential for definitive diagnosis.
- While chemotherapy can be effective, extensive disease may limit long-term outcomes, underscoring the need for prompt diagnosis and treatment strategies.
Abstract:
Primary cardiac lymphoma (PCL) has rarely been reported in Chinese populations. PCL mostly occurs in the right atrium. The clinical manifestations may be variable and are attributed to its location, the presence of congestive heart failure, pericardial effusion, arrhythmia, and cardiomegaly. The prognosis is usually poor because it is usually found too late and therefore, clinicians should be aware of PCL. Imaging examinations are the best methods for initial diagnosis and include echocardiography, computed tomography (CT) scan, magnetic resonance imaging (MRI), and radioisotope scan. However, the final diagnosis is made by pathology, such as cytologic examination of the effusive fluid and tissue biopsy. Because the tumors are difficult to resect, the main treatment for the disease is chemotherapy, which can be successful. Here, we report a 58-year-old man who had a tumor measuring 8 x 5 cm in the right atrium. By clinical staging, including chest X-ray, echocardiography, CT scan of the abdomen, MRI of the heart, whole body tumor Gallium scan, and gastrointestinal series, no metastatic lesion or involvement was found in other parts of the body. Pathologic findings including cytology of pericardial effusion and heart tumor biopsy revealed the case as a diffuse large B-cell lymphoma. After chemotherapy with COP (cyclophosphamide + vincristine + prednisone) and CHOPBE (COP + doxorubicin + bleomycin + etoposide) regimens, the intracardiac tumor had disappeared, but the patient survived for 12 months in total, despite additional radiotherapy over the pericardial lesions. It was presumed that because the tumor was very large and involved all 3 layers of the heart, it did not respond as well to the therapy as expected.
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