Primary Soft Tissue Sarcoma of the Heart: An Emerging Chapter in Cardio-Oncology

Abstract

Primary malignant cardiac tumors are rare, with a prevalence of about 0.01% among all cancer histotypes. At least 60% of them are primary soft tissue sarcomas of the heart (pSTS-h) that represent almost 1% of all STSs. The cardiac site of origin is the best way to classify pSTS-h as it is directly linked to the surgical approach for cancer removal. Indeed, histological differentiation should integrate the classification to provide insights into prognosis and survival expectancy of the patients. The prognosis of pSTS-h is severe and mostly influenced by the primary localization of the tumor, the difficulty in achieving complete surgical and pharmacological eradication, and the aggressive biological features of malignant cells. This review aims to provide a detailed literature overview of the most relevant issues on primary soft tissue sarcoma of the heart and highlight potential diagnostic and therapeutic future perspectives.

Minimally-Invasive Approach in the Setting of a Malignant Primary Cardiac Tumor

“…Characteristically, the cytology reveals heterogeneous neoplasms with various plump spindle lesions and giant features, which make the definite diagnosis difficult. 3 Such cells often display frequent mitotic activity and nuclear polymorphism, while others undergo late programmed cell death like apoptosis ( Fig. 3 ).…”

Cardiac sarcoma presenting as tamponade

“…which is further directly linked to the surgical approach used for resection. 13 In early stage, surgical removal is suitable although feasible in only in less than half of patients as most have evidence of metastasis at the time of presentation or most tumors recur rapidly, limiting survival to approximately one year. 14 , 15 Cytoreductive chemotherapy with or without radiotherapy are other therapeutic options.…”

“…Nevertheless, the impact of chemotherapies on the natural history of cardiac sarcomas is limited, and hence the importance of a multidisciplinary evaluation to define the best therapeutic approach based on the experience of the group. 13 …”

The 2021 WHO Classification of Tumors of the Heart

“…Furthermore, molecular insights into the pathogenesis also afford the opportunity for refinement of classification and nomenclature in these rare neoplasms. 42 In addition to malignancies, better understanding of benign lesions such as PFEs is also critically important given their incidence and the potential they have for causing devastating sequelae (e.g., strokes, myocardial infarctions). Fortunately, the need for fresh tissues for such investigative work is being reduced as our technologies are increasingly allowing for interrogation of formalin-fixed, paraffin-embedded specimens.…”