Primary Cardiac Sarcoma: A Rare, Aggressive Malignancy with a High Propensity for Brain Metastases

Area of Science:

• Cardiovascular Pathology
• Oncology
• Surgical Oncology

Background:

• Primary cardiac sarcoma (PCS) presents a significant clinical challenge with a generally poor prognosis.
• Factors contributing to poor outcomes include late disease presentation, difficulties in surgical resection, frequent metastasis, and limited systemic therapy effectiveness.

Purpose of the Study:

• To analyze the clinical characteristics, treatment patterns, and survival outcomes of patients diagnosed with primary cardiac sarcoma.
• To identify prognostic factors, with a specific focus on the incidence and impact of brain metastases.

Main Methods:

• A retrospective review of medical records was conducted for patients diagnosed with PCS between 1992 and 2017 at the University of Michigan.
• Data collected included patient demographics, tumor histology, site of origin, metastatic patterns, treatment interventions (surgery and systemic therapy), and survival data.

Main Results:

• Thirty-nine patients with PCS were identified, with common histologies including angiosarcoma and undifferentiated pleomorphic sarcoma, predominantly originating in the atria.
• Metastases were present at diagnosis in 18 patients, with lung and brain being common sites. Surgical resection was performed in 25 patients, with R0 resection achieved in only 3.
• Median overall survival was 12.1 months. Resection was associated with improved survival (14.0 vs. 8.2 months, p=0.018). Brain metastases occurred in 31% of patients, significantly impacting survival (median OS 5.6 months post-diagnosis).

Conclusions:

• Primary cardiac sarcoma is associated with poor prognosis due to challenges in achieving complete resection and high rates of advanced-stage disease and metastasis.
• The high incidence of brain metastases, particularly in left-sided tumors, necessitates increased provider awareness.
• Consideration of routine brain imaging at diagnosis and during follow-up is recommended for patients with PCS.