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 5.5x5.9 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.
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Case Media - primary cardiac angiosarcoma
Episode Schematics & Teaching
Pearls – primary cardiac angiosarcoma
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.
