Aortico‐pulmonary paraganglioma. Report of a case with ultrastructural study and review of the literature

Publisher Website
Google Scholar
Abstract
The light microscopic and ultrastructural features of an aortico-pulmonary paraganglioma (A-PP) are presented. The tumor was characterized by organoid clustering of neoplastic chief cells to form Zellballen. Argyrophilic granules were demonstrated within chief cell cytoplasm using a modified Grimelius technique. Ultrastructurally, 3 distinct cell types were present within the tumor: endothelial cells, pericytes and neoplastic chief cells. Membrane-bound neurosecretory granules were present and measured 100-200 nm in diameter. Light and dark chief cells were less distinct than previously reported in other head and neck paragangliomas. Analysis of 36 documented A-PP cases reveals that the tumor was either incompletely excised or considered unresectable in 1/3 of the cases. The reported surgical mortality is 9%, or approximately equal to the incidence of malignant behavior. The treatment of choice is surgical resection but when this is not possible, radiation may be a useful adjunct in therapy.