Primary cardiac sarcomas: A multi‐national retrospective review

Abstract

BackgroundPrimary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.MethodsClinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).ResultsSixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age <65 (P = 0.01) was the only significant favorable prognostic factor. For first‐line palliative chemotherapy, the median PFS was 4.4 months (95% CI 2.9‐7.7 months). The best response for first‐line chemotherapy was 32% (CR = 1, PR = 9). No significant improvement in OS was identified in patients presenting throughout the 20‐year period of this review.ConclusionYounger age at diagnosis was associated with improved outcome although the prognosis of PCS remains poor. Given the lack of improvement in survival, further dedicated research is required.

Cardiac Angiosarcoma from a Saphenous Vein Coronary Artery Bypass Graft

“…Patients received various combinations of chemotherapy (primarily either doxorubicin/ifosfamide or gemcitabine/paclitaxel combinations) and radiation (dose range 43.5-55.8 Gy). Multi-modality therapy did not significantly impact prognosis in the multinational series [18]. In the present case, 60 Gy of adjuvant radiotherapy was given in standard fractionation, and chemotherapy was deferred to the time of progression, in part because of medical comorbidities and lack of known overall survival benefit in the adjuvant setting.…”

“…[6]. Surgical resection was statistically significant on univariate, but not multivariate, analysis of 62 patients from a multinational case series [18]. Highlighting the surgical complexity of these cases, only 8% of patients had R0 resections in this international cohort.…”

“…About a quarter of all tumors are malignant [16]. Sarcomas are the most common primary cardiac malignancy, with angiosarcoma accounting for 35%-39% of PCS [2,[17][18]. Ab cardiac angiosarcoma is characterized by aggressive clinical behavior: median overall survival between six and 17.5 months has been reported [1,18], and four-year overall survival in one multiinstitutional series was 8.4% for angiosarcomas vs. 28.8% for other PCS histologies [3].…”

Primary malignant schwannoma of the heart

“…Partial surgical resection, followed by chemotherapy and radiotherapy can be used as palliative treatment. Postoperative survival varies from 3 months to 1 year, with no significant differences between different tissue types …”

Clinical and Imaging Features of Primary Cardiac Angiosarcoma

“…Clinical manifestations of primary cardiac angiosarcomas are not specific and depend on their infiltration into the myocardium and adjacent structures as well as the extent of metastases [ 6 ]. Patients usually visit a hospital for treatment because of their complications including chest pain, arrhythmia, peripheral edema, dyspnea, orthopnea, congestive heart failure, and pericardial tamponade [ 7 ]. The prognosis of primary cardiac angiosarcoma is considerably poor owing to many reasons including late diagnosis, extensive metastases, incomplete resection, unsatisfactory effect of adjuvant therapy, and lack of standard treatment.…”