Michael D. McPhee, Attending Surgeon, Cleveland Clinic Florida, Naples, FL; Carl Goins, Physician Assistant, Physicians Regional Medical Center, Naples, FL
Michael D. McPhee, MD
Cleveland Clinic Florida
Carl Goins, PA-S
Introduction: Cardiac sarcoma that metastasizes solely to the adrenal glands is rare and has a poor prognosis.
Results and discussion: This paper describes a left atrial sarcoma in a patient who developed adrenal gland metastases 16 months after her cardiac sarcoma was treated, highlighting the aggressive nature of these tumors.
Conclusion: Cardiac sarcomas and subsequent isolated adrenal metastases can be managed using resection for palliation, with the understanding that recurrent distant disease will ultimately develop.
Cardiac sarcomas are primary malignant tumors of the heart that are highly aggressive. Symptoms vary and depend on the tumor's location in the heart and whether distant metastases are present. Patients may experience chest pain, shortness of breath, fatigue, palpitations, hemoptysis, and upper facial congestion.1 Treatment is surgical resection for palliation. After our patient's left atrial sarcoma was resected, she required a simultaneous bioprosthetic mitral valve replacement for mitral valve regurgitation. The patient developed subsequent isolated bilateral adrenal gland metastases 16 months later, resulting in significant pain. Radiation therapy with palliative intent failed. Salvage resection was performed successfully in the left adrenal gland, but only surgical debulking could be accomplished in the right adrenal gland due to the effects of radiation therapy. The patient developed additional distant disease and died 19 months after initial treatment of her sarcoma. Our case highlights the highly aggressive nature of these tumors.
A 41-year-old woman presented with extreme dyspnea, orthopnea, and chest pain. An echocardiogram showed a large tumor in the left atrium. The mass was attached to the posterior wall and annulus. It extended into the atrial appendage and prolapsed into the left ventricle. The gross tumor was resected, but debridement of the tumor off the posterior wall was necessary. Mitral valve regurgitation developed, requiring the patient to undergo simultaneous mitral valve replacement with a bioprosthetic valve. Pathology revealed a high-grade III/III pleomorphic sarcoma. No adjuvant treatment was administered postoperatively.
Sixteen months after the operation, the patient developed bilateral back and flank pain. Computed tomography (CT) scans showed bilateral adrenal gland tumors measuring 10 cm at their widest dimension (Figure 1). Work-up for pheochromocytoma was negative. Percutaneous fine-needle aspiration biopsy of the left adrenal tumor revealed metastatic high-grade spindle cell sarcoma consistent with the patient's primary cardiac atrial sarcoma (Figure 2). A transthoracic echocardiogram revealed an increased area of echodensity in the left atrium. A repeat staging work-up revealed no distant disease aside from the adrenal gland tumors. The patient developed increasingly severe right-sided back and flank pain. She also became anemic (hemoglobin, 7.1 g/dL), which was partly attributed to spontaneous bleeding associated with the right adrenal tumor despite the needle biopsy being performed on the left adrenal gland. On repeat CT scanning, the right adrenal tumor was found to contain a large central hematoma (Figure 3).
Because most of the patient's back pain was on the right side, palliation of her symptoms using radiation therapy on the right adrenal gland tumor was attempted. The patient received 2,600 cGy to the right adrenal tumor, delivered in 13 fractions over 16 days. Follow-up CT scans showed no response from the tumors to radiation therapy, and the tumors in both adrenal glands continued to grow. The patient subsequently required hospitalization for fever and leukocytosis secondary to adrenal gland tumor necrosis.
We planned to proceed with palliative bilateral adrenalectomy to control her pain and prevent further infection and bleeding. The patient underwent a preoperative angiogram and embolization of the tumors to decrease the risk of bleeding during surgery. Three days after the angiogram, she underwent resection via bilateral subcostal incisions of the left adrenal gland with en bloc splenectomy and distal pancreatectomy due to tumor adherence. Cytoreduction of the right adrenal tumor was also performed. The right adrenal tumor could not be resected because it was adherent to the inferior vena cava as a result of the radiation treatments. Pathology revealed metastatic pleomorphic sarcoma (Figure 4).
The patient was discharged from the hospital on postoperative day 7, but she returned to the emergency department several weeks later because of chest pain and dyspnea. A CT scan revealed local recurrence of the cardiac atrial sarcoma, which was acutely obstructing and protruding through the bioprosthetic valve (Figure 5). Periaortic and retroperitoneal lymphadenopathy, right upper lobe lung nodules, and a mediastinal mass were also observed. The patient succumbed to her disease approximately 19 months following resection of the primary atrial sarcoma.
Cardiac atrial sarcomas are rare, aggressive malignancies. Complete resection is the only treatment and offers the best hope for palliation and survival. Complete resection offers a median survival of 24 months versus 10 months for patients who did not undergo complete resection (P = .035).2 One study that evaluated factors influencing survival found through multivariate analysis that a low level of mitotic activity and administration of any therapy were predictive of improved outcomes.3 Other reports confirmed that histologic grade correlates with survival.4 The most common cause of death is local recurrence, which is observed in 50% of cases. Because local recurrence rates are so high for atrial sarcomas, advanced techniques to enhance resection have been attempted. These measures include cardiac explantation, which involves extracorporeal resection of the tumor and cardiac reconstruction using autotransplantation.5
The resistance of cardiac sarcomas to radiotherapy and chemotherapy further complicates management of these lesions. The limited number of cases prevents drawing solid conclusions about the effectiveness of adjuvant therapies. One study found that postoperative chemotherapy did not enhance survival in patients with incomplete resection.2 Radiation therapy proved ineffective in our patient.
We found one other case in the literature of an atrial sarcoma metastatic to the adrenal glands.6 The patient was a 61-year-old Japanese woman who underwent tumor resection of a primary cardiac left atrial angiosarcoma. She developed a right adrenal gland metastasis 3 months postoperatively and died of heart failure from local recurrence approximately 6 months postoperatively.
Because it is so rare for sarcomas to metastasize to the adrenal glands, it is difficult to make recommendations for managing them. In a series of patients with metastasis to the adrenal glands, sarcomas were the primary tumors in only 2 of 41 patients (5%).7 The patients in this study represented eight disease types: non?small-cell lung cancer, renal cell carcinoma, colorectal cancer, melanoma, sarcoma, adenocarcinoma of unknown primary origin, hepatocellular carcinoma, and cholangiocarcinoma. Based on a multivariate analysis, those who underwent adrenalectomy for metastasis had improved survival rates when the disease-free interval was greater than 6 months. Our patient had a disease-free interval of 16 months, but the degree of tumor burden nullified any chance for a reasonable improvement in her life expectancy. Although laparoscopic resection has been proposed, open adrenalectomy was required in our case because of the tumors' massive sizes and their adherence to contiguous structures, including the inferior vena cava.
This case presentation of a cardiac atrial sarcoma is unusual because isolated adrenal metastases most likely developed concurrent with an occult local recurrence and before any additional distant disease was clinically evident. Because radiation is ineffective, surgical resection of the adrenal tumors was palliative and the only option. Our patient experienced rapid progression of her disease postoperatively, evidenced by changes in CT findings over a 1-month period. While the progression of her disease was alarming, it is indicative of this cancer's aggressive tumor biology and growth kinetics. As with other sarcomas, the development of novel chemotherapy or targeted therapies may alter the outcome of this malignancy for future patients.
We would like to thank Bruno Dipasquale, MD, PhD, Chief of Pathology and Laboratory Director for Physicians Regional Medical Center, Naples, FL, for his assistance with this case.
The authors have no relationship with any commercial entity that might represent a conflict of interest with the content of this article and attest that the data meet the requirements for informed consent and for the Institutional Review Boards.