CardioNerds Cofounder Dr. Amit Goyal is joined by an esteemed group of UCLA cardiology fellows – Dr. Patrick Zakka (CardioNerds Academy Chief), Dr. Negeen Shehandeh (Chief Fellow), and Dr. Adrian Castillo – to discuss a case of primary cardiac angiosarcoma. An expert commentary is provided by Dr. Eric Yang, beloved educator, associate clinical professor of medicine, assistant fellowship program director, and founder of the Cardio-Oncology program at UCLA.
Case synopsis: A female in her 40s presents to the ED for fatigue that had been ongoing for approximately 1 month. She also developed night sweats and diffuse joint pains, for which she has been taking NSAIDs. She was seen by her PCP and after bloodwork was done, was told she had iron deficiency so was on iron replacement therapy. Vital signs were within normal limits. She was in no acute distress. Her pulmonary and cardiac exams were unremarkable. Her lab studies showed a Hb of 6.6 (MCV 59) and platelet count of 686k. CXR was without significant abnormality, and EKG showed normal sinus rhythm. She was admitted to medicine and received IV iron (had not consented to receiving RBC transfusion). GI was consulted for anemia work-up. Meanwhile, she developed a new-onset atrial fibrillation with rapid ventricular response seen on telemetry, for which Cardiology was consulted. A TTE was ordered in part of her evaluation, and surprisingly noted a moderate pericardial effusion circumferential to the heart. Within the pericardial space, posterior to the heart and abutting the RA/RV was a large mass measuring approximately 5.5×5.9 cm. After further imaging work-up with CMR and PET-CT, the mass was surgically resected, and patient established care with outpatient oncology for chemotherapy.
The pericardium is composed of an outer fibrous sac, and an inner serous sac with visceral and parietal layers.
Pericardial masses can be primary (benign or malignant) or metastatic. There are other miscellaneous pericardial masses.
Imaging modalities for the pericardium include echocardiography, cardiac CT and cardiac MRI. There is also role for PET-CT in pericardial imaging for further characterization of pericardial masses.
Cardiac angiosarcomas are extremely rare but are the most common cardiac primary malignant tumors.
Evidence-based management if lacking because of paucity of clinical data given the rarity of cardiac angiosarcomas. Surgery is the mainstay of therapy. Radiotherapy and chemotherapy are often used as well.
Notes – primary cardiac angiosarcoma
Pericardial Anatomy
The pericardium is a fibroelastic sac composed of two layers.
Outer layer: fibrous pericardium (<2 mm thick)
Inner layer: serous pericardium, two-layered sac.
Visceral pericardium: adherent to underlying myocardium
Parietal pericardium: lines fibrous sac.
Between the serous layers, there is the pericardial cavity which normally contains up to 50 cc pericardial fluid.
Pericardial Masses
Benign
Lipoma: slow-growing, collection of adipose cells, thought to arise in AV groove
Teratoma: benign germ cell tumors, often right sided. Can cause compressive symptoms of RA, SVC, PA, aortic root.
Fibroma: solid mass of connective tissue
Hemangioma: vascular mass, often arising from visceral pericardium
Malignant
Sarcoma: various types including angiosarcoma and liposarcoma.
Lymphoma: usually non-Hodgkin B-cell lymphoma, often in immunocompromised patients
Mesothelioma: no apparent association with asbestos. Pericardial effusions with nodules/plaques are seen.
Metastatic
Often associated with hemorrhagic pericardial effusions
Breast cancer, lung cancer, melanoma and renal cell carcinoma are most common
Pericardial Imaging
Echocardiography
Advantages:
widely available
low cost
safe
can be performed in multiple settings (e.g., HD unstable)
Has been shown to be an effective additional imaging modality in patients in whom cardiac mass is suspected to be malignant, and helps provide further confirmation and screening for metastatic disease.
Angiosarcoma of the Pericardium
Very rare, but most common cardiac primary malignant tumor.
Typically right-sided and secondarily involves the pericardium.
Primary pericardial angiosarcoma usually occurs in middle-aged, more frequently in males.
Often metastatic at time of diagnosis.
Clinical presentation
Variety of symptoms, and often undetected early on.
Symptoms include dyspnea, chest pain, cough, fatigue/malaise, and signs of caval obstruction.
Clinical picture rapidly deteriorates as it can eventually result in intractable heart failure and death due to multi-organ failure.
Lab/Imaging Tests
Tumor marker CA125 elevated.
Pericardiocentesis usually reveals bloody fluid (containing RBCs, WBCs). Cytology often misses malignant cells.
EKG can show non-specific ST-T wave abnormalities and low QRS voltage.
CXR may show an enlarged cardiac silhouette.
Transthoracic echocardiography can show pericardial effusion but may fail to show echogenic mass if no good acoustic windows. Large masses in the pericardium may be seen in some patients.
CT can show location, size, and extent of mass.
CMR can further show tumor necrosis or hemorrhage. Can help characterize and stage tumors.
PET/CT can help detect metastasis from pericardial tumors.
Definitive diagnosis = biopsy (mediastinoscopy, exploratory pericardiotomy or thoracotomy). Extensive excision is usually recommended. Angiosarcomas histologically are characterized by presence of anastomosing vascular channels that are lined by atypical/malignant endothelial cells showing frequent mitoses.
Because of rarity there is little clinical evidence-based data for management.
Usually responds poorly to chemotherapy and radiation.
Surgery is challenging because these tumors are diagnosed late and there is already metastatic disease.
Orthotopic cardiac transplantation is sometimes done and has prolonged life, though incidence of metastatic disease limits utility. Could be helpful in patients who have unresectable but locally aggressive tumors without metastasis.
Palliative treatment options are usually resorted to because the disease often presents so late. Length of survival after diagnosis ranges between 6-11 months.
Ultimately, surgical resection with negative margins is associated with best outcome; there is some benefit to then adding chemotherapy and radiotherapy.
Yin H, Mao W, Tan H, et al. Role of 18F-FDG PET/CT imaging in cardiac and pericardial masses. J Nucl Cardiol. 2022; 29(3):1293-1303. https://pubmed.ncbi.nlm.nih.gov/33462788/
