Four cases of paraganglioma originating in the supra-aortic or aortico-pulmonary bodies are described. A review of the reported examples of aortic body tumors and a study of these four cases indicate that there is a high incidence of aggressive tumor growth in the mediastinum, with resultant important morbidity or death in 16 of 35 cases. The morphologic features of aortic body tumors are identical to those of paragangliomas of other locations, and the occurrence of invasive growth and/or metastasis cannot be predicted on histologic grounds.Cancer 4 1 : 2405-24 12, 1978.
N 1950 LATTES REPORTED TWO EXAMPLES OFI paragangliomas originating in the area of the aortic arch. Since that time an additional 47 mediastinal paragangliomas have been described. Twelve of these were located in the costo-vertebral sulcus (mediastinal aortico-sympathetic paraganglioma). The remaining 35 tumors and the four described in this report originated in the area of the aortic arch and are classified as aortico-pulmonary paragangliomas as defined by Glenner and Grimley. Review of these cases revealed a high incidence of morbidity and death associated with aortic body paragangliomas, largely due to invasive growth of residual mediastinal neoplasm.
CASE REPORTS Case 1A normotensive 37-year-old woman was found to have mediastinal mass on routine chest x-ray in 1951. The lesion was contiguous with the aortic arch. In 1954 surgical removal of the lesion was attempted, but only partial excision of the mass was possible. Following surgery, radiotherapy resulted in initial reduction in the size of the residual tumor. The tumor slowly progressed, however, leading to respiratory insufficiency and death 13 years after the original chest x-ray. At autopsy extensive local infiltration of the mediastinum by tumor was noted. Metastases were not identified.
Case 2A 51-year-old man with a 1 Yi history of cough developed hemoptysis and left chest pain. He had decreased breath sounds in the anterior left upper chest and deviation of the trachea to the right. At the time of admission the chest x-ray demonstrated a rounded density in the anterior superior mediastinum and in the left hilum. Review of previous radiographs revealed that a mass had been present for three years. At thoracotomy an extremely vascular tumor was observed in the left anterolateral aspect of the mediastinum. It was adherent to the lung and had invaded the innominate vein, adventitia of aortic arch, and anterior chest wall. The patient died intraoperatively secondary to hemorrhage from the tumor. Both routine laboratory studies and skeletal survey were within normal limits. Permission for an autopsy was denied.
.Case 3In 1960 a 55-year-old man presented with the superior vena cava syndrome. H e was known to have had a widened medastinum for the previous five years but had been asymptomatic during that period. At thoracotomy in 1960 tumor involved the superior vena cava, right and left phrenic nerves, and aorta. A partial excision was performed. At a second thoracotomy in 1963, tumor occl...