Cardiac pheochromocytomas

Abstract

Cardiac pheochromocytomas are rare. Thirty cases have been reported in the literature. We report the cases of 2 more patients in whom the diagnosis was established using coronary angiography and who underwent surgical resection using cardiopulmonary bypass. We also review the literature on the subject.

Intrapericardial paraganglioma

“…In a review of the literature, we found 19 cases reported to the roof of the left atrium, 7 in the interatrial septum and 6 in the anterior surface of the heart [4]. The combined use of I-MIBG scintigraphy and thoracic CT scan allowed the diagnosis of thoracic pheochromocytoma in the majority of cases reported [2,4,8]_ The use of I-MIBG permits the location of the tumor and whole body search for metastases during a single procedure [4,5]. Twodimensional echocardiography is very helpful when the tumor is intracardiac [4], but fails in determining tissue discrimination and in the evaluation of the pericardium and other extracardiac structures.…”

“…The combined use of I-MIBG scintigraphy and thoracic CT scan allowed the diagnosis of thoracic pheochromocytoma in the majority of cases reported [2,4,8]_ The use of I-MIBG permits the location of the tumor and whole body search for metastases during a single procedure [4,5]. Twodimensional echocardiography is very helpful when the tumor is intracardiac [4], but fails in determining tissue discrimination and in the evaluation of the pericardium and other extracardiac structures. However, it establishes with certainty whether the tumor involves the heart and the necessity of other diagnostic procedures.…”

“…It is important to emphasize that these tumors are not well encapsulated. Safer and adequate ablation usually requires full thickness resection of the myocardium contiguous to the tumor because of strong adherence or even invasion [ 1,4]. Coronary grafting or cardiac chamber reconstruction may be necessary [6].…”

A rare cause of hypertension in children: intrathoracic pheochromocytoma

“…The majority occurs in the posterior mediastinum and there are fewer than 50 reported cases of these tumors within the pericardium in association with heart in the world literature [2]. Extraadrenal pheochromocytomas are more frequent in pediatric than in adult population (30 vs. 10%) [3].…”

Cardiac Malignant Pheochromocytoma with Bone Metastases.

“…Only two cases, however, have been reported to be definitely malignant with bone metastasis (4,5). While the outcome of the surgical intervention for the benign cardiac tumor has been quite satisfactory despite the difficult anatomical localization (6), the prognosis of cases with malignant pheochromocytoma is poor (7). Recently, we treated a patient with cardiac pheochromocytoma with bone metastasis of the iliac bone, in which treatments with an antihypertensive agent, a-methylparatyrosine (a-MPT), and intense chemotherapywith a combination of cyclophosphamide, vincristine, and dacarbazine (8) were partially effective in improving the symptomsand the metastatic lesion of the iliac bone in addition to reducing catecholamine levels.…”