Neoplastic pericardial disease, neoplastic pericardial involvement or neoplastic pericarditis refers to a pericardial infiltration by tumour cells usually associated with a variably sized pericardial effusion and is a form of non-infectious pericarditis. It needs to be differentiated from other causes of pericardial effusions.
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Epidemiology
Neoplastic pericardial involvement is one possible cause of pericardial effusion or pericarditis with an estimated frequency of 5-10% alongside other causes as idiopathic pericarditis, radiation or chemotherapy-induced pericarditis and others 1-3. Primary pericardial tumours are rare. Secondary involvement of other neoplasms as lung cancer, breast cancer, lymphomas or leukaemias are more common 1.
Associations
Pericardial involvement in neoplastic disease is associated with a variably sized pericardial effusion in about 12-25%.
Clinical presentation
Clinical features of pericardial involvement in neoplastic disease include dyspnoea and reduced exercise intolerance, pericarditic chest pain, fatigue and possible pericardial friction rub. Often there will be a known underlying malignancy. However, malignant pericardial effusion might also occur as an initial presentation of malignant disease 1-3.
Possible changes in electrocardiogram include widespread ST-segment elevation and PR-segment depression as seen in pericarditis.
Complications
Complications of pericardial involvement in neoplastic disease include the following 1:
Pathology
Pericardial involvement in neoplastic disease is characterised by tumour infiltration of the pericardium and/or pericardial effusion often associated with high monocyte count.
Aetiology
Causes pericardial involvement in neoplastic disease includes the following 1,4,5:
- primary tumours of the pericardium or the heart (rare)
- secondary pericardial involvement (direct invasion, metastases, venous extension)
Radiographic features
Imaging forms an integral part of the diagnosis of pericardial involvement in neoplastic disease. One of the main imaging features is a variable-sized pericardial effusion which will be apparent on different imaging modalities. Other imaging features include variable pericardial thickening or direct tumour growth.
Echocardiography
Echocardiography is a first-line imaging modality in the diagnosis and monitoring of pericardial effusions and pericarditis. It can also identify complications as cardiac tamponade or pericardial constriction and can be used to guide pericardiocentesis 1,4,5.
CT
CT is a standard imaging modality in the staging of malignancy. In addition, it can depict pericardial effusion which might be heterogeneous, pericarditis as well as direct tumour growth or deposits 1,4,5.
MRI
MRI cannot only detect and visualise pericardial effusion and pericarditis but can also characterise any underlying tumour or neoplastic involvement. Besides, cardiac MRI can detect haemorrhagic effusion. Imaging Finding includes the following 4,5:
- pericardial adhesions
- pericardial thickening and nodular changes
- pericardial oedema and enhancement
- pericardial (pseudo)mass or tumour deposits
Signal characteristics
- T1: variable, hyperintense if blood is present
- T2: heterogeneous high signal intensity
- STIR/PDFS: high signal intensity
- T1C+(Gd): variable enhancement
Nuclear medicine
Demonstration of FDG-uptake in the pericardium by PET or PET-CT in patients with cancer or lymphoma indicates pericardial involvement and can aid in the diagnosis as well as in monitoring therapy.
Radiology report
The radiological report should contain a description of the following:
- pericardial effusion, pericardial thickening, pericardial enhancement
- presence of pericardial haemorrhage
- location and size of tumour deposits
- cardiac volumes and measurements
- ventricular wall motion abnormalities
- myocardial involvement or signs of myocarditis
Treatment and prognosis
Pericardial involvement of neoplastic disease features a high rate of recurrence of 40-70%. Management involves the following 1,4:
- systemic tumour therapy as a baseline
- pericardiocentesis for securing/establishing the diagnosis and symptom relief
- extended pericardial drainage
- intrapericardial administration of cytostatic and/or sclerosing agents to prevent recurrences
Radiation therapy can control pericardial effusion in lymphomas and leukaemias 1. Percutaneous balloon pericardiotomy or pericardial window creation can be considered in large pericardial effusions to relieve symptoms.
Complications
Complications of intrapericardial treatment include the following 1:
- chest pain
- fever
- atrial arrhythmias
Complications of radiation therapy include 1:
- myocarditis
- radiation-induced pericarditis
Differential diagnosis
Pericardial involvement in neoplastic disease can mimic the appearance of other types of pericardial effusions 1,4:
- radiotherapy-induced pericarditis
- pericarditis due to chemotherapeutic toxicity
- pericarditis due to opportunistic infections
- tuberculous pericarditis
- purulent pericarditis