CASE 15436 Published on 29.01.2018

Mediastinal non-Hodgkin lymphoma with cardiac involvement and cardiac chamber compression

Section

Chest imaging

Case Type

Clinical Cases

Authors

Vinicius Carraro Do Nascimento (MD), Elisa Chia (MBBS), Yuranga Weerakkody (MBChB, FRANZCR)

Royal Perth Hospital;
Level 3, North Block,
Wellington Street
6000 Perth, Australia;
Email:vinicn@icloud.com
Patient

88 years, female

Categories
Area of Interest Cardiac, Cardiovascular system ; Imaging Technique PET-CT, CT, Conventional radiography
Clinical History
An 85-year-old male patient presented to our institution with a 1 to 2 month history of vague chest discomfort and bilateral upper limb swelling. As part of his admission work up, he was initially evaluated with a chest radiograph followed by a contrast CT scan which showed the following findings.
Imaging Findings
Initial chest radiographs (Fig. 1a-b) show abnormal cardiomediastinal contours, with increased soft tissue density outlining both left hilar and right paraspinal regions.

Subsequent contrast-enhanced CT images (Fig. 2a-f, 3a-b and 4) show a rather homogeneous large mass-like conglomeration infiltrating through the anterior mediastinum with invasion into the right atrial walls and its cardiac chamber with resultant marked narrowing of the right atrial chamber and lower part of the superior vena cava. Only a filiform contrast tract is seen communicating with the right ventricle. There is partial encasement of great vessels especially in the region of the aortic arch.

Further work up with CT-PET (Fig. 5) to assess wider disease status within the rest of the body showed avid FDG enhancement throughout the anterior mediastinal mass-like conglomeration but no areas of distant uptake. The patient then underwent a CT-guided mediastinal biopsy (Fig. 6) giving a diagnosis of non-Hodgkin lymphoma.
Discussion
Diffuse large B-cell lymphoma is an aggressive form of non-Hodgkin lymphoma (NHL) and is also the most common type of lymphoma. NHL accounts for more than 80% of all cases of lymphoma with approximately half of affected patients having intra-thoracic disease. In comparison with Hodgkin lymphoma (HL), NHL is three times less likely to occur in the mediastinum. Mediastinal lymphoma can often be responsible for superior vena cava (SVC) syndrome.

Primary cardiac lymphoma is exceedingly rare with occurrence in around 1% of extranodal lymphoma cases [1]. Secondary involvement of the myocardium to some degree has been demonstrated in up to 30% of patients in patients with systemic lymphoma with disseminated disease at autopsy [2].

Clinical presentation of cardiac metastases is dependent on various factors including tumour location, size, growth rate, degree of invasion, and friability of the tumour. In cases of malignant lymphoma, a cardiac or cardiovascular manifestation as an initial sign of the disease is extremely rare, and symptoms can often be sub-clinical or non-specific [3-7].

Common presentations of cardiac lymphoma include dyspnoea, congestive heart failure, pericardial effusion, and haemopericardium. Cardiac arrhythmias and other non-specific electrocardiographic abnormalities can also be common, notably with ranging degrees of atrioventricular block [8].

Computed tomography with contrast can adequately demonstrate morphology, location, and extent of cardiac neoplasms with a larger field view. However, magnetic resonance imaging with contrast enhancement results in superior images identifying anatomy, blood flow, and cardiac function [9, 10]. Fluorodeoxyglucose (18F) positron emission tomography imaging has been reported to be a useful tool in revealing previously unsuspected cardiac involvement [11, 12].

Cardiac lymphoma has the tendency to extend along the epicardial surfaces of the heart, encasing adjacent structures including coronary arteries and the aortic root. Infiltration beyond the myocardium may occur, and if affecting the right atrium, may result in venous extension leading to superior or inferior vena caval thrombosis. Right-sided cardiac chamber involvement (as with our case) is commoner while left atrial and ventricular infiltration tends to be far less common [13-18].

Overall prognosis for primary and secondary cardiac lymphoma is generally poor.
The literature on management contains several treatment combinations including chemotherapy, chemotherapy plus radiation therapy and varying combinations of these options [19]. The most frequently used chemotherapy regime is cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). With the tissue necrosis that occurs with the administration of chemotherapy, cardiac rupture and embolic phenomena [20, 21] are important complications to consider.
Differential Diagnosis List
Mediastinal non-Hodgkin lymphoma with cardiac involvement and chamber compression
Thymic carcinoma with cardiac invasion
Mediastinal sarcoma
Final Diagnosis
Mediastinal non-Hodgkin lymphoma with cardiac involvement and chamber compression
Case information
URL: https://www.eurorad.org/case/15436
DOI: 10.1594/EURORAD/CASE.15436
ISSN: 1563-4086
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