A case of a vagal body tumor with a solitary cervical node metastasis is described. There was a remarkable familial association: a biopsy-proven paraganglioma occurred in two members of the patient's family and there was a strong clinical suspicion of a paraganglioma in two others. In a review of the literature, eight patients with metastasizing vagal body tumors and eight patients in whom the vagal body tumor was a component of multicentric presentation of paragangliomas were found. Only one other patient in whom there was a familial occurrence involving a vagal body tumor has been reported. Ultrastructural study of the cervical node metastasis revealed the presence of light and dark chief cells containing scanty, membrane-bound, densecore, neurosecretory-type granules. Atypical granules were noted in a few of the dark cells. Nerve fibers, synaptic vesicles, and sustentacular cells, such as occur in normal paraganglionic tissue, were not observed in this tumor.Cancer 38 : 2367-237 7 , 19 76.
INCE THE FIRST DESCRIPTION OF A TUMOR ARIS-S ing from paraganglionic tissue related to the vagus nerve by Stout41 in 1935, 56 more cases have appeared in the literature to date. This paper documents the light microscopic and ultrastructural features of a vagal body tumor (VBT) with a cervical node metastasis. It is only the second electron microscopic study of a V B T and the first of a metastasis from such a tumor. There was evidence of the occurrence of a paraganglioma in a sister, father, paternal aunt, cousin, and possibly in a grandfather. This is only the second recorded instance of familial occurrence of paragangliomas involving a VBT.A review of the literature up to the present time has revealed eight other VBTs with metastases, From the I k p a r t m e n t