心外膜内褐色細胞腫の一例と文献的考察

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A patient with intrapericardial pheochromocytoma is presented and the literature on this subject is reviewed. The patient was a 45-year-old housewife who was first referred to Keio University hospital in February 1981 for a 6-year-history of headaches, palpitations and excessive sweating. An episode of hypertensive crisis was observed during surgery for myoma uteri 3 months before her admission. On physical examination, the patient was a normal-appearing, pleasant woman. Supine blood pressure was 130/80mmHg and standing blood pressure was 124/72 mmHg. Results of routine laboratory studies (including fasting serum glucose and calcium determinations and thyroid function tests) were normal. A 24-hour-urine collection showed VMA level of 10.5 mg and noradrenaline of 809μg. Also repeated measurements of plasma noradrenaline on supine position revealed averages of 2.45 ng/ml. These high levels of catecholamines, clinical manifestations, and provocative tests including a metoclopramide test were strongly suggestive of pheochromacytoma, and further studies for determination of the localization were performed. Results of computed tomography (CT), adrenal scintigraphy using 131I-Adosterol and selective venous sampling for catecholamines were equivocal. Then she was followed in an outpatient clinic and treated with α-and β-bl oc ker In October 1985, she was readmitted to our hospital because of paroxysms which had gradually increased in frequency and severity. Extensive venous sampling in order to determine the localization of the tumor was performed after demonstration of an increased uptake area in the anterior thorax in 131I-metaiodobenzylguanidine scintigraphy. Further, an anterior mediastinal mass was identified in the CT scan of the chest with contrast material. Finally, a thoracic angiogram revealed that the tumor was highly vascular and was fed by the branch of the left internal mammary artery. In January 1986, the patient underwent middle sternotomy with cardiopulmonary bypass. A 4.5 by 3.5 by 3.0cm soft, dark brown tumor was found on the anterior surface of the heart and excised without difficulty. The tumor seemed to arise from the root of the aorta and main pulmonary artery, and extended inferiorly over the anterior surface of the right ventricle. It was also fed by branches of the right coronary artery. Histologically, the resected tumor was compatible with pheochromocytoma. The tumor content of noradrenaline was 3.34mg/g wet tissue; however, adrenaline and dopamine were not detected. The postoperative course was uneventful. Intrapericardial pheochromacytoma is extremely rare and to our knowledge only 13 cases have been reported in the literature. Of 14 patients (including the present case) involved, 4 were males and 10 females; the age range was 18-76 years. Nine of the lesions were attached to the posterior aspect of the left or right atrium. Two of the lesions were located in the interatrial septum, and the remainder were found on the left lateral surface of the heart. It is assumed that a pheochromocytoma at this site may arise from chromaffin cells which are distributed adjacent to sympathetic fibers innervating the heart. Compared to the previously reported cases, the location of the described case herein is unusual, because it was found on the anterior surface of the heart. Our case will support the concept that all paraganglion-bearing tissue is capable of giving rise to functioning paragangliomas.

心外膜内褐色細胞腫の一例と文献的考察

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