Cyanotic congenital heart disease with malignant paraganglioma

A malignant paraganglioma of the subclavian (supra-aortic) area and organ of Zuckerkandl with metastases to the liver and pancreas was discovered at autopsy in a 22-year-old man with known transposition of the great arteries. Light microscopy showed the typical "Zellballen" pattern and Grimelius stain showed intracytoplasmic argyrophilic granules, which appeared ultrastructurally as electron dense granules. Review of the literature disclosed 59 previously reported cases of hypoxia associated with endocrine tumors. The case presented is believed to be the first example of a subclavian paraganglioma associated with hypoxemia. One previous case of a paraganglioma arising in the organ of Zuckerkandl that occurred in a hypoxic state has been reported. The possibility of cyanotic congenital heart disease with chronic long-standing hypoxia, predisposing to the development of paraganglioma with malignant transformation is presented.