We describe a rare case of adrenocortical carcinoma that extended to the inferior vena cava and the right atrium. Tumors that affect the right atrium include primary neoplasms and secondary tumors such as hypernephroma, hepatoma, testicular sarcoma and melanoma .
Adrenal carcinoma may also extend to the renal veins and the inferior vena cava, usually accompanied by a thrombus. Right atrial involvement is very rare [4–15].
Most of the described cases of adrenal carcinomas extending to the right atrium including the present case were right-sided [4, 6, 7, 9–15]. This is explained by the direct course of the right adrenal vein to the inferior vena cava.
Other forms of cardiac involvement include infiltration of the inter-atrial septum, and a malignant pericardial effusion [8, 17].
The main clinical manifestations of vena caval and right atrial extension of the tumor include peripheral edema, ascites and hepatomegaly as in our case. A patient with bouts of dyspnea and cyanosis due to a patent foramen ovale and a right atrial tumor was described . Sudden death, presumably caused by right ventricular inflow obstruction was also reported .
Due to the tendency of the tumor to disseminate, a precise pre-operative assessment is essential. Evaluation should involve various additional modalities such as CT, [4, 6–8] venography and MRI [7, 19]. Echocardiography is a very useful tool in the assessment of cardiac tumors [5, 7, 8, 10, 13, 16, 17, 19–22]. In the present case transthoracic echo identified the cardiac involvement, leading to change in the operative approach by adding cardiac surgeons to the operation.
Echocardiography can define the various patterns of cardiac extension with excellent anatomic correlations [20, 21]. Transesophageal echo can provide high quality images of the inferior vena cava and right atrial involvement, and direct the surgeons in the removal of the neoplasm from both sites [8, 22]. In cases in which a good quality transthoracic study is positive for cardiac involvement, transesophageal study can be performed only intraoperativlely, to monitor and direct surgery in the operative arena. A patent foramen ovale should be looked for before putting the patient on by-pass.
We suggest that adrenal carcinoma, although very rare, should be included in the differential diagnosis of right atrial tumors. Once an adrenal tumor is detected, the work-up should include echocardiography, which is of a paramount importance in the evaluation of cardiac involvement, operative planning and follow-up.