Primary cardiac sarcoma: a case report of a therapeutic challenge

Abstract

Background Primary cardiac sarcomas are very rare and the prognosis is poor both because the diagnosis is typically made at an advanced stage of the disease and because data are insufficient to identify a standard treatment. Surgical resection is the cornerstone of therapy with the need to develop new therapeutic strategies. Case summary We present a case of a young man admitted to the emergency department due to worsening dyspnoea. A left-sided sarcoma was diagnosed and treated with surgery, chemo- and radiation therapy, and subsequently with heart transplant for local recurrence of the disease. Endomyocardial biopsy made during the routine follow-up period was complicated by pericardial tamponade and cardiogenic shock and the patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of left ventricular function (left ventricular ejection fraction of 55%). After 1 year a kidney transplant was performed. After 42 months from diagnosis, the patient is in good general condition. Discussion Primary cardiac sarcomas are treated with surgery to reach R0 (free resection margins) and with chemo- and radiation therapy with adjuvant purposes. Auto-transplantation is also performed, while conventional heart transplant must be customized on an individual basis, after excluding metastases. A multidisciplinary assessment should be performed and the single patient treated with a personalized approach, in relation to his performance status, location of the mass, and stage of the disease.

Clinical presentation, diagnostic evaluation, and management of undifferentiated/unclassified cardiac sarcoma: A case report and literature review

“…Symptoms of cardiac tumors typically depend on the location of the mass. Although many cardiac tumors are asymptomatic, they are often diagnosed at an advanced stage of the disease [16] . Dyspnea is the most common symptom, followed by chest pain, congestive heart failure, and palpitations [17] .…”

Primary cardiac sarcoma presenting with easy bruising: a case report

“… 1–3 Angiosarcomas are the most common type of sarcomas, occurring in 43% of all sarcoma cases. 2 , 3 Sarcomas are typically limited to a single cardiac chamber, most commonly to the left or right atria. 2 In our patient, the tumour was present in the right ventricle and atrium and invaded the interventricular septum.…”

“… 1 The median age at presentation is 40–50 years. 3 , 4 Currently, we do not have complete understanding of the behaviour of right heart sarcomas in the elderly. Symptoms of cardiac tumours are mainly due to the site of the mass.…”

“…Many cardiac tumours remain asymptomatic, and the diagnosis is made at an advanced stage of the disease. 3 Cardiac sarcomas have a propensity towards early metastatic dissemination to the lung, bone, soft tissue, and brain. While the right heart sarcomas have a more indolent presentation, they tend to metastasize, usually to the lungs.…”

Primary Cardiac Sarcoma: Clinical Characteristics and Prognostic Factors over the Past 2 Decades

“…Primary cardiac sarcomas (PCS) are the most common malignancies affecting the heart; however, overall, only 15 percent of cardiac neoplasms are malignant [ 1 ]. PCS remains a rare entity in the literature that has mainly been reported by means of case reports [ 2 , 3 , 4 , 5 , 6 ]. Almost exclusively, PCS does not arise from benign neoplasms but are believed to arise de novo [ 7 ].…”