Primary Malignancies of the Heart and Pericardium

Abstract

Primary malignancies of the heart and pericardium are rare. All the available data come from autopsy studies, case reports, and, in recent years, from large, specialized, single‐center studies. Nevertheless, if primary malignancy is present, it may have a devastating implication for patients. Malignancies may affect heart function, also causing left‐sided or right‐sided heart failure. In addition, they can be responsible for embolic events or arrhythmias. Today, with the widespread use of noninvasive imaging modalities, heart tumors become evident, even as an incidental finding. A multimodality imaging approach is usually required to establish the final diagnosis. Despite the increased awareness and improved diagnostic techniques, clinical manifestations of primary malignancy of the heart and pericardium are so variable that their occurrence may still come as a surprise during surgery or autopsy. No randomized clinical trials have been carried out to determine the optimal therapy for these primary malignancies. Surgery is performed for small tumors. Chemotherapy and radiation therapy can be of help. Partial resection of large neoplasms is performed to relieve mechanical effects, such as cardiac compression or hemodynamic obstruction. Most patients present with marginally resectable or technically nonresectable disease at the time of diagnosis. It seems that orthotopic cardiac transplantation with subsequent immunosuppressive therapy may represent an option for very carefully selected patients. Early diagnosis and radical exeresis are of great importance for long‐term survival of a primary cardiac malignancy. This can rarely be accomplished, and overall results are very disappointing.

Characteristics and Survival of Malignant Cardiac Tumors

“…In the largest previous series of 143 cases of malignant cardiac tumors, angiosarcomas were most common at 23.1%, followed by leiomyosarcoma at 20.3% and rhabdomyosarcoma at 4.2%. 2 Our data show similar distributions of angiosarcoma (25.8%) and rhabdomyosarcoma (2.6%) but a much lower prevalence of leiomyosarcoma (3.7%). The predominance of angiosarcoma was also reported previously by researchers in Italy (28.6%), 18 the Mayo Clinic (41%), 19 and the British Columbia Registry.…”

“…10 Of these, malignant cardiac tumors were even less common, representing 5.1% to 28.7% of all heart tumors in small series. 2 Our study shows that clinically apparent PMCTs have an estimated prevalence of 34 cases per 100 million persons, >100 times lower than previous estimates. This discrepancy may be explained partially by the possibility that many of the autopsy-discovered tumors may have been incidentalomas rather than clinically significant tumors.…”

“…Although non-Hodgkin lymphoma has a strong tendency to involve the myocardium, with up to 20% of patients with non-Hodgkin lymphoma having evidence of myocardial involvement at autopsy, 26 immunosuppressed patients (transplant recipients, those with HIV, etc) typically present with primary cardiac lymphoma without extracardiac involvement. 2 In fact, 41% of all patients with primary cardiac lymphomas are immunocompromised and have universally poor survival. 25 Previous reports show that diffuse large B-cell lymphomas have a predilection for the right side of the heart (92% had involvement of right atrium or right ventricle) and usually present with dyspnea, constitutional symptoms, pain and arrhythmias.…”

“…[1][2][3] Because most practitioners may see only a handful of such cases in their lifetime, the accumulated experience on this subject has been collated and summarized in multiple extensive literature reviews. 1,4 Nevertheless, the core knowledge of PMCTs has continued to come from single-center studies consisting of surgical case series and autopsy reports.…”

“…1,4 Nevertheless, the core knowledge of PMCTs has continued to come from single-center studies consisting of surgical case series and autopsy reports. 2,[4][5][6][7] Because of the relatively small numbers and significant referral bias of these reports, the incidence of PMCTs remains unclear, their histology incompletely defined, and treatment ineffectual, and prognosis is thought to be universally poor.…”