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AN UNRESECTABLE PRIMARY CARDIAC SARCOMA PRESENTING AS HEART FAILURE
A598 JACC March 17, 2015 Volume 65, Issue 10S
FIT Clinical Decision Making An Unresectable Primary Cardiac Sarcoma Presenting as Heart Failure Poster Contributions Poster Hall B1 Saturday, March 14, 2015, 10:00 a.m.-10:45 a.m. Session Title: FIT Clinical Decision Making: Heart Failure and Cardiomyopathies Abstract Category: Heart Failure and Cardiomyopathies Presentation Number: 1109-139 Authors: Candice Baldeo, Robert Ali, Sana Chaudhry, Fawad Khawaja, UF Health, Jacksonville, FL, USA
Background: Resection remains the primary mode of treatment for primary cardiac sarcomas (PCS). PCS usually has a high recurrence rate of up to 50%, even after resection. Early diagnosis and initiation of treatment - resection and/or chemotherapy with radiation may decrease recurrence and have mortality benefit.
Case: A 64 year old female with hypertension and hyperlipidemia presented with progressive dyspnea on exertion, bilateral lower extremity edema, and chronic dry cough for three months. She had no previous smoking history. Examination noted bilateral pitting pedal edema and right basilar crackles. Chest xray showed moderate cardiomegaly and interstitial pulmonary edema. Electrocardiogram was low voltage with prolonged QTc. Computerised tomography(CT) revealed 9.9 x 11.5 x 14.2 cm heterogeneous mass associated with the anterior pericardium, displacing the heart superiorly and posteriorly. There was also internal necrosis and calcification suggestive of a sarcoma. Transthoracic echocardiogram showed preserved ejection fraction but revealed a large extracardiac mediastinal mass compressing the anterior right ventricle. Cardiac MRI showed a cystic-solid mass arising in the anterior pericardium. CT guided core needle biopsy resulted in spindle and epithelioid sarcoma. Left heart catheterization was normal. Decision Making: The decision was made for complete resection of the mass. At surgery an anterior mediastinal mass was wedged underneath the pericardium. Attempts at debulking revealed that the mass was involving the myocardium, making it unresectable. Pathology showed high-grade sarcoma with features favoring malignant perivascular epithelioid cell tumor. She refused chemotherapy or radiotherapy and opted for hospice care.
Conclusion: PCS remains an uncommon entity, as a result there is limited data on optimal treatment options. Reports have emphasized the benefit of complete surgical therapy followed by adjuvant chemotherapy and radiation. Given that our patient’s cardiac sarcoma was deemed unresectable, it postulates the role of (1) improved imaging techniques to better define the mass and (2) neoadjuvant chemotherapy to improve surgical outcome.
FIT Clinical Decision Making An Unresectable Primary Cardiac Sarcoma Presenting as Heart Failure Poster Contributions Poster Hall B1 Saturday, March 14, 2015, 10:00 a.m.-10:45 a.m. Session Title: FIT Clinical Decision Making: Heart Failure and Cardiomyopathies Abstract Category: Heart Failure and Cardiomyopathies Presentation Number: 1109-139 Authors: Candice Baldeo, Robert Ali, Sana Chaudhry, Fawad Khawaja, UF Health, Jacksonville, FL, USA
Background: Resection remains the primary mode of treatment for primary cardiac sarcomas (PCS). PCS usually has a high recurrence rate of up to 50%, even after resection. Early diagnosis and initiation of treatment - resection and/or chemotherapy with radiation may decrease recurrence and have mortality benefit.
Case: A 64 year old female with hypertension and hyperlipidemia presented with progressive dyspnea on exertion, bilateral lower extremity edema, and chronic dry cough for three months. She had no previous smoking history. Examination noted bilateral pitting pedal edema and right basilar crackles. Chest xray showed moderate cardiomegaly and interstitial pulmonary edema. Electrocardiogram was low voltage with prolonged QTc. Computerised tomography(CT) revealed 9.9 x 11.5 x 14.2 cm heterogeneous mass associated with the anterior pericardium, displacing the heart superiorly and posteriorly. There was also internal necrosis and calcification suggestive of a sarcoma. Transthoracic echocardiogram showed preserved ejection fraction but revealed a large extracardiac mediastinal mass compressing the anterior right ventricle. Cardiac MRI showed a cystic-solid mass arising in the anterior pericardium. CT guided core needle biopsy resulted in spindle and epithelioid sarcoma. Left heart catheterization was normal. Decision Making: The decision was made for complete resection of the mass. At surgery an anterior mediastinal mass was wedged underneath the pericardium. Attempts at debulking revealed that the mass was involving the myocardium, making it unresectable. Pathology showed high-grade sarcoma with features favoring malignant perivascular epithelioid cell tumor. She refused chemotherapy or radiotherapy and opted for hospice care.
Conclusion: PCS remains an uncommon entity, as a result there is limited data on optimal treatment options. Reports have emphasized the benefit of complete surgical therapy followed by adjuvant chemotherapy and radiation. Given that our patient’s cardiac sarcoma was deemed unresectable, it postulates the role of (1) improved imaging techniques to better define the mass and (2) neoadjuvant chemotherapy to improve surgical outcome.