Cardiac tumors are a rare, but potentially curably form of heart disease A high index of clinical suspicion is necessary for diagnosis as these tumors have protean manifestations that mimic a variety of other cardiac and noncardiac diseases Presently, M-mode and two-dimensional echocardiography are utilized as safe, reliable, and noninvasive imaging modalities Seventy-five per cent of these tumors are benign, with myxoma accounting for 50% and rhabodomyoma comprising 20% of lesions Various histologic types of sarcoma are the predominant malignant cardiac neoplasms With strict attention to avoiding perioperative tumor embolization, surgical resection of these lesions can be accomplished with minimal morbidity and mortality Sixteen consecutive primary tumors of the heart have been surgically treated at Duke University Medical Center since 1966 with no perioperative deaths and no late recurrences

Primary cardiac tumors

Primary cardiac sarcomas.

Forty-five cases of cardiac angiosarcomas were reviewed, and the data were compared with those of a 1968 review of 41 other cases which revealed these tumors to be typically located within the right atrium as large symptomatic masses and to be rapidly fatal, with the diagnoses usually determined only at autopsy. The relationship of these tumors to Kaposi's sarcoma was also examined. The findings paralleled those of the previous review. Additionally, the following points emerged: With the aid of newer imaging techniques, localization, biopsy diagnosis and resection of the atrial tumors are now being achieved more often, with some improvement in survival. Like angiosarcomas of other organs, atrial angiosarcomas exhibit highly variable histologic patterns, which often overlap those of Kaposi's sarcoma, and may also present metastatic patterns simulating widespread Kaposi's sarcoma or malignant melanoma. In reported cases of classical, endemic, or epidemic Kaposi's sarcoma, cardiac lesions are uncommon and typically are small, asymptomatic, restricted to the epicardium/or pericardium and, thus, are clearly different, both clinically and pathologically, from the atrial tumor group. The justification for designating cases of the latter group as "primary cardiac Kaposi's sarcoma" is open to debate. A case report illustrates many of the above points.

https://acsjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%2819860215%2957%3A4%3C852%3A%3AAID-CNCR2820570428%3E3.0.CO%3B2-S

Cardiac angiosarcomas: A review and a case report

The aim of the study was to create an anatomical correct 3D rapid prototyping model (RPT) for patients with complex heart disease and altered geometry of the atria or ventricles to facilitate planning and execution of the surgical procedure. Based on computer tomography (CT) and magnetic resonance imaging (MRI) images, regions of interest were segmented using the Mimics 9.0 software (Materialise, Leuven, Belgium). The segmented regions were the target volume and structures at risk. After generating an STL-file (StereoLithography file) out of the patient's data set, the 3D printer Ztrade mark 510 (4D Concepts, Gross-Gerau, Germany) created a 3D plaster model. The patient individual 3D printed RPT-models were used to plan the resection of a left ventricular aneurysm and right ventricular tumor. The surgeon was able to identify risk structures, assess the ideal resection lines and determine the residual shape after a reconstructive procedure (LV remodelling, infiltrating tumor resection). Using a 3D-print of the LV-aneurysm, reshaping of the left ventricle ensuring sufficient LV volume was easily accomplished. The use of the 3D rapid prototyping model (RPT-model) during resection of ventricular aneurysm and malignant cardiac tumors may facilitate the surgical procedure due to better planning and improved orientation.

3D-Imaging of cardiac structures using 3D heart models for planning in heart surgery: a preliminary study

Aims

Primary cardiac sarcomas are rare and aggressive tumours. The aims of this study were to precisely classify cardiac sarcomas according to their pathology, and to determine their clinicopathological features and prognosis.



Methods and results

Twenty-four primary cardiac sarcomas were studied. Clinical features and follow-up of all patients were collected. Histological diagnoses were obtained by combining both morphological features as described in soft tissue counterparts and the immunoprofile of the tumours. The 24 cases were classified as undifferentiated sarcoma (nine cases), angiosarcoma (six cases all located in the right atrium), leiomyosarcoma (six cases), malignant fibrous histiocytoma (one pleomorphic and one giant cell type) and synovial sarcoma (one case). Patients included 14 men and 10 women, with a mean age of 46 years. Clinical manifestations were protean, often delaying the diagnosis. Dyspnoea resulting from obstruction of the cardiac chambers was the most common symptom. Echocardiography and magnetic resonance imaging were useful respectively to detect and to evaluate tumour extension. Complete macroscopic resection was possible in only 33% of patients. The most common cause of death was local recurrence of the tumours (50%), even in the cases of complete macroscopic resection. Whatever the treatment, the prognosis was poor with a mean survival of 16.5 months after diagnosis.



Conclusions

All types of sarcomas may be observed in the heart with a predominance of undifferentiated sarcomas. Histological grading, unlike histological type, seems to correlate with survival which remains extremely poor.

Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases.

Two cases of angiosarcomas of the heart are described. In one the tumour, which arose from the right atrium, was demonstrated during life by angiography. In the other, diagnosed only at necropsy, the tumour arose from the right ventricle. Both cases illustrate many of the typical features of this rare tumour and the difficulties of antemortem diagnosis.

https://heart.bmj.com/content/heartjnl/41/4/498.full.pdf

A Mitko

Papers

Angiosarcoma of the heart.