Key points
- •
Differentiating primary benign vs malignant cardiac tumors can be done using multimodal imaging as well as tissue biopsy.
- •
Clinical features including rapid growth, involvement of multiple cardiac chambers or structures, and local invasion are all suggestive for a malignant cardiac tumor.
- •
Complete tumor resection may involve complex reconstruction or the potential need for cardiac explant, tumor resection, reconstruction, and then auto-transplantation.
- •
The prognosis is generally poor; however, there is a survival benefit for patients undergoing surgical resection compared to those patients who do not undergo resection.
- •
The goals of surgery may include both an R0 resection and tumor debulking for symptomatic improvement.
Less than 10% of primary cardiac tumors are malignant, and of those the majority (75%) are sarcomas . There are several important factors to consider when evaluating a malignant cardiac tumor for surgical resection. In addition, patient comorbidities, tumor involvement of multiple cardiac structures, blood supply, rapid growth with presence of necrosis, metastatic disease, and goals of resection (R0 resection vs symptomatic improvement) are important considerations which influence clinical outcome.
Multimodal imaging (CT, Cardiac MR, and Echo) is a gold standard for diagnosis, staging, and surgical planning. It is important to not only characterize the tumor with preoperative imaging but also establish an accurate diagnosis, preferably with tissue biopsy. This is critical for (a) differentiating benign from malignant histology, and (b) identifying certain malignant tumors where optimal treatments may be nonsurgical such as cardiac lymphoma . Adjuvant chemotherapy may be necessary in many of these patients. Survival is poor; however, there is a benefit of surgical resection for select patients.
Modern series on these tumors focus on the advantages of combined selected cardiac resection with systemic adjuvant therapy. In some cases, a complete resection might require cardiac auto-transplantation whereby the heart is explanted, to allow full access to the posterior structures of the heart and the posterior mediastinum. Ex vivo tumor resection is completed along with any required reconstruction, and the heart is then reimplanted into the mediastinum. One of the largest series reported on 34 patients who underwent this technique . Of those, 26 were for primary cardiac sarcomas, Overall 1-year survival for primary malignant tumors was 46%, and two-year survival was a dismal 28%. Among patients with primary malignant tumors, 19 had isolated cardiac auto-transplantation and 7 had auto-transplantation plus pneumonectomy. Operative mortality was significantly higher for patients requiring pneumonectomy. Interestingly, for primary sarcomas, survival was not impacted by the presence of microscopically positive resection .
The most common location of primary malignant cardiac tumors is either the right or left atrium. If a malignant tumor is located in the right atrium, it is most commonly an angiosarcoma. Accurate imaging is necessary to better understand tumor involvement with the tricuspid valve as well as the right ventricle ( Fig. 20.1 ) and assess potential occlusion of blood flow from the IVC or SVC, which may ultimately require palliative resections and reconstruction. Reconstruction of the right atrium and, if necessary, the inferior or superior cava with an autologous pericardium patch may prevent narrowing of these structures at the time of resection.
Overall prognosis of primary cardiac angiosarcoma is poor and only marginally improved with surgical resection . Most of the studies comparing surgery vs nonsurgical options involve small numbers of patients and are associated with a strong selection bias, since patients undergoing surgery usually were found to have more localized disease. A significant percentage of patients with angiosarcoma present with advanced disease, where complete surgical resection is not possible and incomplete resection is associated with local recurrence . In those instances, a combination of neoadjuvant chemo/radiation therapy has been demonstrated to reduce tumor size and allow for a more complete resection . In select patients, an aggressive combination of resection, chemotherapy, radiation therapy, and even cardiac transplantation for nonresectable disease has been attempted .
In the left atrium, the most common malignant tumors are pleomorphic sarcomas (malignant fibrous histiocytoma) and leiomyosarcoma ( Fig. 20.2 ). Leiomyosarcomas are extremely rare with only a limited number of cases reported in the literature. In one report, a 24-year-old patient was found to have a left atrial leiomyosarcoma involving both the interatrial septum and the right atrial free wall . The patient underwent two surgical excisions followed by adjuvant radiation and chemotherapy resulting in a nearly 7-year survival ( Fig. 20.3 ).
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
