Primary Cardiac Tumors: Surgical Treatment Improves Survival

Primary cardiac tumors are extremely rare, comprising only 0.008% of cancer diagnoses in the United States, and patients with primary cardiac malignancy carry a notoriously poor prognosis.¹ Due to the rarity of the malignancy, cardiac surgeons are challenged with determining when to attempt resection in patients with a primary cardiac tumor. Additionally, controversy has arisen, in the absence of clear data, regarding the role of adjuvant therapy in treatment. Moreover, it is difficult to accrue patients to studies at a single institution due to low volume and changing standards of care over time. For these reasons, we undertook a study to examine long-term outcomes of patients with primary cardiac malignancies using the National Cancer Database (NCDB). 

Primary cardiac tumors represent less than 1% of heart cancer diagnoses, as the vast majority of heart cancer is composed of metastases to the heart from a separate primary source.² Unfortunately, the majority of patients present with advanced cancer that is not diagnosed until heart failure and obstructive symptoms manifest due to tumor bulk. Complications of primary cardiac tumors include valve regurgitation and thrombus formation with heightened embolization risk. When assessing operative candidacy, surgeons should consider the patient’s age, comorbidities, and whether distant metastases are present. At UPMC, surgeons work collaboratively with a multidisciplinary team including colleagues in the Division of Cardiology to reach a consensus on the best treatment approach for patients with primary cardiac tumors.

Our goal in examining the patient cohort available in the NCDB, which is jointly sponsored by the American College of Surgeons and the American Cancer Society, was to analyze how surgical resection and other treatments affected survival and to better understand the typical profile of patients with a primary cardiac tumor. After excluding patients with heart metastases, benign tumors, and cardiac lymphomas, the cohort consisted of 747 patients with a median age of 53 years who were treated from 2004 to 2016. Most of their tumors were classified as sarcomas (89%). Surgery was performed in 59% of the patients, either alone (187 patients) or as part of a multimodality treatment plan (255 patients). The study confirmed the dismal prognoses associated with primary malignancy of the heart.

The overall survival was 81.2% after 30 days, 45.3% after one year, and just 11.5% after five years. Survival was correlated with tumor stage, and patients with stage I primary cardiac tumors had the best survival. Encouragingly, patients who underwent surgery had significantly better (p<0.001) estimated survival than those who underwent only radiation therapy and/or chemotherapy or who received no treatment. Due to the need to maintain vital heart structure, residual tumor often remains following resection, and an R0 resection was accomplished in only 26.0% of patients, with improved survival in this group. In the subpopulation of patients with stage III disease, patients who underwent surgical resection and then received adjuvant chemotherapy had significantly improved survival as compared with patients who only underwent resection.

Although our study of primary cardiac tumors using the NCDB did not identify treatment center as a factor influencing long-term survival, treatment at UPMC, a high-quality tertiary care center, has significant advantages. A multidisciplinary team of oncologists, cardio-oncologists, and cardiothoracic surgeons allows for a complete evaluation of a patient’s cardiac tumor. Experienced operating room staff and skilled critical care personnel are invaluable assets when caring for these patients who have complicated needs and high perioperative morbidity. Following assessment by our multidisciplinary team, including evaluation of the final tumor margins by our expert pathologists, recommendations on the potential benefits of adjuvant therapy are made by knowledgeable oncologists. This large study, based on a national multi-institutional database, performed by our group in the UPMC Department of Cardiothoracic Surgery provides good evidence that carefully selected patients with primary cardiac tumors fare better with early surgical intervention.

To learn more about the UPMC Heart and Vascular Institute or to refer a patient, call 412-770-4949. 

References

1. Oliveira GH, Al-Kindi SG, Hoimes C, Park SJ. Characteristics and Survival of Malignant Cardiac Tumors: A 40-Year Analysis of >500 Patients. Circulation. 2015;132(25):2395-2402.

2. Tyebally S, Chen D, Bhattacharyya S, et al. Cardiac Tumors. JACC: CardioOncology. 2020;2(2):293.

3. Sultan I, Bianco V, Habertheuer A, et al. Long-Term Outcomes of Primary Cardiac Malignancies: Multi-Institutional Results From the National Cancer Database. J Am Coll Cardiol. 2020;75(18):2338-2347.