Arash Aghel, MD Department of Internal Medicine, Cleveland Clinic
Richard A. Krasuski, MD Department of Cardiovascular Medicine, Section of Clinical Cardiology, Cleveland Clinic
Address: Richard Krasuski, MD, Department of Cardiovascular Medicine, J2-4, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail krasusr@ccf.org
Dr. Krasuski has disclosed that he has received honoraria from Pfizer for teaching and speaking and honoraria and consulting fees from Actelion for teaching, speaking, and consultation.
A DIAGNOSIS IS MADE
Figure 5. Liver biopsy shows cellular endothelial atypia with mitotic activity (black arrow), growth along the hepatic sinusoids, and papillary tufting (white arrow), all features of low-grade angiosarcoma.Fluorodeoxyglucose (FDG) positron-emission tomography is done, and shows a hypermetabolic right-sided pericardial tumor in addition to several suspicious hepatic lesions with heterogeneously increased FDG uptake. Biopsy with ultrasonographic guidance is performed again and reveals tissue consistent with hemangioma in addition to other areas with features strongly suggestive of a low-grade angiosarcoma (Figure 5). Pathology findings are unable to differentiate primary cardiac angiosarcoma from a metastatic cardiac tumor; however, given the multiple liver lesions and the presence of a solitary cardiac mass, this is most likely a primary cardiac tumor with metastasis to the liver.
CARDIAC ANGIOSARCOMA
Cardiac angiosarcoma, the most common malignant primary cardiac tumor, has a predilection for the right atrium.13 These tumors tend to occur between the third and fifth decade of life and are three times more common in men than in women. Cardiac sarcomas proliferate rapidly and commonly extend into the pericardial space, causing pericardial effusion in up to one-fourth of patients.
Surgical resection is the treatment of choice, but due to the location and extent of involvement, complete resection is often difficult. Also, distant metastases are present at the time of diagnosis in 80% of cases, precluding a surgical cure.17 Adjuvant chemotherapy, radiotherapy, and even heart transplantation do not substantially improve the survival of these patients.18–20 Because no effective treatment is available, the prognosis is dismal, with a median survival of 6 to 12 months.
Our patient is discharged home to follow up with an oncologist and initiate chemotherapy.
Acknowledgment: We thank Lisa M. Yerian, MD, for interpreting the biopsy specimens described in this article.
