Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association: ultrastructural study and review.
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.
D000328:Adult; D000368:Aged; D002345:Carotid Body Tumor / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D008566:Membranes / Q000648:ultrastructure; D008875:Middle Aged; D009333:Neck; D009378:Neoplasms, Multiple Primary / Q000473:pathology; D010236:Paraganglioma, Extra-Adrenal / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D014630:Vagus Nerve