Clinicopathological analysis and management of primary cardiac sarcomas: A retrospective study from two cancer centers in China.

Abstract

e23544 Background: Primary cardiac sarcomas (PCS) are rare malignant tumors associated with a poor prognosis. The clinical and pathological features of PCS remain elusive. Methods: We retrospectively collected records of patients with PCS at Peking University Cancer Hospital and Zhejiang Cancer Hospital from 2016 to 2023. Clinicopathological data, treatments, and outcomes were included and analyzed. Results: Between January 2016 and October 2023, a total of 26 patients with primary cardiac sarcoma were included, with the majority of patients being female (57.7%). The median age of the patients was 38.5 years (range: 15-82). Tumors were most frequently located in the right (42.3%) and left (26.9%) atria. The predominant histological subtype was angiosarcoma (46.1%), followed by myxoid fibrosarcoma (15.4%) and intimal sarcoma (11.5%). At baseline, the LDH level was elevated in 42.3% of patients. Metastases were observed in 21 (80.8%) patients, predominantly in lung (61.9%), bone (42.9%) and liver (28.6%). Nineteen patients (73.1%) underwent surgery for the primary tumor, with 8 (42.1%) achieving a complete (R0) resection and 11 (57.9%) achieving a microscopically (R1) or macroscopically (R2) incomplete resection. Sixteen patients (61.5%) received anthracycline-based chemotherapy as their first-line palliative treatment. The median overall survival (OS) for all patients was 19.2 months (95% CI: 16.7-31.2). Patients with normal baseline LDH levels had a significantly longer median OS (25.4 months) compared to patients with elevated LDH levels (11.6 months, P= 0.001). The median OS was 25.4 months for patients who underwent R0 resection, compared to 18.7 months for those who underwent R1/R2 resection, and 6.03 months for those who did not undergo surgery. Patients who received anthracycline-based chemotherapy had a moderately better survival than those who received paclitaxel-based chemotherapy (mOS 19.2 vs.14.8 months, P= 0.13). Conclusions: In patients with PCS, angiosarcoma is the predominant histological subtype, and the most common sites of metastasis are the lung, bone, and liver. Elevated LDH is associated with a poorer prognosis. Complete surgical resection of the cardiac sarcoma after diagnosis may benefit long-term survival. [Table: see text]