1113 Primary and secondary malignant cardiac tumors - a 22 year case review
Authors: Garcia Bras P , Moura Branco L , Castelo A , Vaz Ferreira V , Branco Mano T , Galrinho A , Timoteo A T , Abreu J , Pinto E , Coelho P , Bravio I , Ferreira R C 
Published: January 2020

Abstract

Background
Primary cardiac tumors are extremely rare and are usually diagnosed late due to the non-specific symptomatology. Surgery is the main treatment option and despite chemotherapy, the prognosis remains poor. Cardiac invasion by metastatic tumors, while more common, also entails an unsatisfactory outcome.

Purpose
To review patients (P) with malignant cardiac tumours that were diagnosed by transthoracic echocardiography (TTE) or by transesophageal echocardiogram (TEE) in a tertiary center between 1997 and 2019.

Methods
Retrospective analysis of clinical data from the digital files, echocardiographic assessment of tumor location and morphology, histology results and survival outcomes.

Results
A total of 33 malignant cardiac tumors were diagnosed: 12 primary tumors (A) and 21 metastatic tumors (B).

A
Regarding primary cardiac tumors, the most common types were angiosarcomas (6 cases), 2 undifferentiated pleomorphic sarcomas, 1 right ventricle (RV) sarcoma, 1 primary cardiac lymphoma, 1 myxofibrosarcoma and 1 fibrosarcoma.
The mean age of the P at time of diagnosis was 43 ± 15 years, 50% female gender. The most frequent presentation was heart failure symptoms (50% of P) followed by arrhythmias (20%). One patient had a rare presentation with pruritus and polyarthralgias.
On TTE, the most prevalent tumor location was in the right-heart chambers (70%) - mostly the right atrium (50%), with mean dimensions of 40 ± 18 mm x 27 ± 11 mm.
85% of patients had preserved systolic left ventricular function and there was moderate or severe pericardial effusion in 38%.
The most frequent distant metastatic involvement of primary tumors at diagnosis was pulmonary (33%) and hepatic (33%).
50% of P were submitted to tumor resection and 40% were submitted to chemotherapy.
In the case of angiosarcomas, the most common immunohistochemical markers were vimentin, CD31 and CD34.
The authors found a mortality rate of 81% in P with primary cardiac tumors, with a median time of follow-up of 6 months (minimum of 20 days and maximum of 18 years). In the latter case, the P was submitted to heart transplantation after diagnosis of a sarcoma of the RV and is still alive and well.

B
Regarding secondary cardiac invasion, there was a diagnosis of the following primary tumor sites: 6 thymomas, 4 cases of lymphoma, 3 lung carcinomas, 3 hepatocellular carcinomas, 2 bladder carcinomas, 1 parathyroid carcinoma, 1 soft tissue sarcoma and 1 melanoma.
The mean age of P with metastastic involvement of the heart was 57 ± 22 years, 65% male. On TTE/TEE the authors also found a right-sided chambers predominance (60%), with pericardial metastasis in 35%.
As expected, the mortality rate was also extremely high (90%), with a median time of follow up of 1.5 months (minimum of one week, maximum of 44 months)

Conclusion
Cardiac malignant tumors generally present in a late stage with a dismal prognosis. When possible, heart transplantation can be an option with a good outcome.