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Nicholas Nissen, MD, has been appointed as director of Hepatobiliary and Pancreatic Surgery at Cedars-Sinai.

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Treatment of cardiac tumors

Cardiac echocardiography

Because primary cardiac tumors are rare, many hospitals refer their cases to Cedars-Sinai where the most advanced treatment is routinely available. Tumors of the heart typically present in one of four ways: 1) they are found incidentally; 2) after embolization or stroke; 3) with obstruction of blood flow; or 4) with vague constitutional symptoms.The diagnostic approach relies upon echocardiography (shown above), MRI, and CT to define the presence of a tumor and its anatomic relationship to normal structures.

Most benign lesions are treated with complete resection via a full sternotomy. However, here at Cedars-Sinai, a minimally invasive robotic approach is used under the leadership of Alfredo Trento, MD, FACS, director of the Division of Cardiothoracic Surgery and the Estelle, Abe and Marjorie Sanders Endowed Chair in Cardiac Surgery. This system enables complex minimally invasive surgery and microsurgical procedures which conventional surgical techniques cannot achieve.The robot consists of four mechanical arms. The surgeon can precisely control the surgical instruments and endoscope with his hands. A video display of the high-definition endoscopic image allows the surgeon to see inside the patient's body in far greater detail.

A malignant lesion, however, remains treatable only by a full sternotomy. In general, sarcomas proliferate rapidly, and cause death through widespread infiltration of the myocardium, obstruction of blood flow through the heart, and/or distant metastases. The poor results with surgical resection have led to occasional attempts to treat patients with cardiac transplantation, a method which introduces its own set of issues.

However, an alternative treatment, cardiac autotransplantation, can take the place of complicated transplants. In these cases, the heart is excised, the tumor is resected ex vivo, and the heart is reconstructed before being reimplanted. The advantage of this procedure is the increased ease with which major resection and reconstruction can be performed, while at the same time avoiding the need for antirejection treatment. The disadvantage, however, is that removing the heart from the body cavity is time consuming and requires more surgery. For this reason, yet another method, invivo repair, also has been developed. Although technically more challenging it is faster and less invasive

Such was the case confronting a Cedars-Sinai cardiothoracic surgeon on a recent weekend. Danny Ramzy, MD,PhD, FRCSC, received a call for a 53-year-old female, who had presented to another hospital with hemoptysis and varying heart failure symptoms, which included a two week siege of shortness of breath.

Cardiac histologyThe heart failure work up revealed a large mass occluding 80 percent of the left atrium. Obstruction to the mitral valve had resulted in severe mitral stenosis. While the etiology of the mass was initially unknown, the emergent issue was its two mobile components, raising the potentially fatal prospect of embolization. Because of the emergency circumstances, the team was unable to await the histology results (at right).

Cardiac mass Upon entering the atrium, the left atrium was resected,along with the intra atrial septum, a portion of the right atrium and the mass, itself (at left). Reconstruction was then performed using bovine pericardium, bypassing the need for transplantation. The mitral valve repair also was completed without the need for replacing the valve. The patient did well in recovery, with discharge following postoperatively on day five.

In spite of the overall success of the operation, unfortunately her final pathology demonstrated the mass to be a high-grade pleomorphic sarcoma.

In general, complete resection is the treatment of choice for sarcomas. Yet most patients develop recurrent disease and die of their malignancy even if their tumor can be completely resected. The median survival is typically six to 12 months, although long-term survival has been reported with complete resection. Patients with low-grade sarcomas also may have a better prognosis. Adjuvant chemotherapy has been used in an effort to improve on the poor results with resection alone. However, most of the published experience consists of anecdotal case reports, and no randomized trials have been conducted. Radiation has been used infrequently, and primarily as a treatment of metastases.

In spite of the challenging prognosis, the emergency surgery performed by the Cedars-Sinai on-call surgeon has given this patient the only chance she can possibly have.

Submitted by the Division of Cardiothoracic Surgery in the Department of Surgery.