Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.
Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.
Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).
Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.
