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Cardiac Angiosarcoma: A Rare Cause of Recurrent Pericardial Effusion
Cardiovascular Disease SESSION TITLE: Cardiovascular Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM
Cardiac Angiosarcoma: A Rare Cause of Recurrent Pericardial Effusion Sidra Khalid* Richard Meredith and Allan Klein Fairview Hospital, Cleveland Clinic, Cleveland, OH INTRODUCTION: Cardiac angiosarcoma is a rare entity. Early diagnosis is delayed due to generalized symptoms. Diagnosis is made when specific symptoms related to tumor growth are investigated.
DISCUSSION: Over the years, the diagnosis of cardiac sarcomas has improved due to a combination of physical examination, ekg, cardiac catheterization, echocardiography, CT scan and MRI. It mostly involves the right atrium, presenting as pericardial effusion and/or cardiac tamponade in about 56% of cases. Complete or partial surgical resection is done for palliation or complete cure. Angiosarcomas are mostly resistant to chemotherapy and radiotherapy. However, recently paclitaxel and pegylatedliposomal doxorubicin have shown efficacy in treating angiosarcoma. CONCLUSIONS: Overall, even with the new imaging techniques for diagnosis and management, cardiac sarcomas still carry a poor prognosis with a 5-year survival rate of 14%. Reference #1: Blackmon, Shandra H., and Michael J. Reardon. “Surgical Treatment of Primary Cardiac Sarcomas.” Texas Heart Institute Journal 36.5 (2009): 451-452. Reference #2: Loukas, Marios. “Primary Cardiac Angiosarcoma - A Review”. Medical Science Monitor 20 (2014): 103-109. Reference #3: Skubitz, Keith M., and Philip A. Haddad. “Paclitaxel And Pegylated-Liposomal Doxorubicin Are Both Active In Angiosarcoma”. Cancer 104.2 (2005): 361-366. DISCLOSURE: The following authors have nothing to disclose: Sidra Khalid, Richard Meredith, Allan Klein No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2017.08.113
Copyright ª 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
chestjournal.org
83A
CARDIOVASCULAR DISEASE
CASE PRESENTATION: A 20 y/o female presented with pleuritic chest pain, treated as pericarditis. After one month, she developed fatigue and dyspnea. CT chest was remarkable for bilateral pleural effusions and pericardial effusion. She underwent pericardiocentesis. Pericardial bacterial and fungal evaluation was unrevealing. ANA, anti dsDNA, antihistone, complement levels, RF, HIV, acute hepatitis panel, RPR, coccidiomycosis and CMV titers, toxoplasma Ab, ANCA levels, coxsackie B, and IgM EBV were negative. She had a positive IgG EBV and her symptoms were attributed to viral perimyocarditis. She continued to have right-sided chest pain and was admitted to the hospital after three months. Her vital signs and physical examination were unremarkable. Cardiac MRI revealed a large mass, 4.1 x 6.3 x 4.8 cm, anterior to the right atrium and extending to the AV groove and proximal SVC, along with a moderate pericardial effusion. On perfusion images, there was gadolinium uptake at the anterior most part of the tumor with three hypoperfused areas portraying vascularity. Left heart catheterization showed extensive vascularization in the angiographic location of the right atrium where the tumor was located. She underwent tumor resection. The mass was extensive, beyond the right atrial free wall, on the right ventricle surface, pericardial metastasis and underneath an innominate vein on top of the aorta. Pathology displayed an aggressive high-grade angiosarcoma with a sheet-like growth pattern. Tumor cells had CD31 membranous and ERG nuclear staining. She was stable after surgery and further managed with paclitaxel.
Cardiac Angiosarcoma: A Rare Cause of Recurrent Pericardial Effusion Sidra Khalid* Richard Meredith and Allan Klein Fairview Hospital, Cleveland Clinic, Cleveland, OH INTRODUCTION: Cardiac angiosarcoma is a rare entity. Early diagnosis is delayed due to generalized symptoms. Diagnosis is made when specific symptoms related to tumor growth are investigated.
DISCUSSION: Over the years, the diagnosis of cardiac sarcomas has improved due to a combination of physical examination, ekg, cardiac catheterization, echocardiography, CT scan and MRI. It mostly involves the right atrium, presenting as pericardial effusion and/or cardiac tamponade in about 56% of cases. Complete or partial surgical resection is done for palliation or complete cure. Angiosarcomas are mostly resistant to chemotherapy and radiotherapy. However, recently paclitaxel and pegylatedliposomal doxorubicin have shown efficacy in treating angiosarcoma. CONCLUSIONS: Overall, even with the new imaging techniques for diagnosis and management, cardiac sarcomas still carry a poor prognosis with a 5-year survival rate of 14%. Reference #1: Blackmon, Shandra H., and Michael J. Reardon. “Surgical Treatment of Primary Cardiac Sarcomas.” Texas Heart Institute Journal 36.5 (2009): 451-452. Reference #2: Loukas, Marios. “Primary Cardiac Angiosarcoma - A Review”. Medical Science Monitor 20 (2014): 103-109. Reference #3: Skubitz, Keith M., and Philip A. Haddad. “Paclitaxel And Pegylated-Liposomal Doxorubicin Are Both Active In Angiosarcoma”. Cancer 104.2 (2005): 361-366. DISCLOSURE: The following authors have nothing to disclose: Sidra Khalid, Richard Meredith, Allan Klein No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2017.08.113
Copyright ª 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
chestjournal.org
83A
CARDIOVASCULAR DISEASE
CASE PRESENTATION: A 20 y/o female presented with pleuritic chest pain, treated as pericarditis. After one month, she developed fatigue and dyspnea. CT chest was remarkable for bilateral pleural effusions and pericardial effusion. She underwent pericardiocentesis. Pericardial bacterial and fungal evaluation was unrevealing. ANA, anti dsDNA, antihistone, complement levels, RF, HIV, acute hepatitis panel, RPR, coccidiomycosis and CMV titers, toxoplasma Ab, ANCA levels, coxsackie B, and IgM EBV were negative. She had a positive IgG EBV and her symptoms were attributed to viral perimyocarditis. She continued to have right-sided chest pain and was admitted to the hospital after three months. Her vital signs and physical examination were unremarkable. Cardiac MRI revealed a large mass, 4.1 x 6.3 x 4.8 cm, anterior to the right atrium and extending to the AV groove and proximal SVC, along with a moderate pericardial effusion. On perfusion images, there was gadolinium uptake at the anterior most part of the tumor with three hypoperfused areas portraying vascularity. Left heart catheterization showed extensive vascularization in the angiographic location of the right atrium where the tumor was located. She underwent tumor resection. The mass was extensive, beyond the right atrial free wall, on the right ventricle surface, pericardial metastasis and underneath an innominate vein on top of the aorta. Pathology displayed an aggressive high-grade angiosarcoma with a sheet-like growth pattern. Tumor cells had CD31 membranous and ERG nuclear staining. She was stable after surgery and further managed with paclitaxel.