Bilateral pheochromocytoma-ganglioneuroma of the adrenal in type 1 neurofibromatosis.

Abstract

A 61-year-old woman with café-au-lait pigmentation and severe cutaneous neurofibromatosis type I was noted to have persistent hypertension after coronary artery bypass grafts. Clinical investigation revealed bilateral adrenal medullary tumors. The patient did not have a duodenal lesion or gastrointestinal symptoms. Histologic examination showed both tumors to be composed of typical pheochromocytoma with large areas of ganglioneuroma (compound or composite pheochromocytomas). The neuromatous foci contained areas of cystic degeneration and thick-walled vessels. The ganglion cells and neuromatous areas were negative for chromogranin, glial fibrillary acidic protein, synaptophysin and vasoactive intestinal peptide. The typical pheochromocytomatous areas were strongly immunopositive for chromogranin and synaptophysin. Bilateral classic pheochromocytomas are rare in type 1 neurofibromatosis, and we believe that bilateral composite pheochromocytomas are an extension of this association.