Composite pheochromocytoma-ganglioneuroma: a case with two distinct components radiographically

Composite pheochromocytoma is an extremely rare tumor that comprises a pheochromocytoma and an embryologically related neurogenic tumor, such as ganglioneuroma, ganglioneuroblastoma, neuroblastoma, or peripheral nerve sheath tumor. A 46-year-old male with hypertension, elevated plasma catecholamine levels, and suspected pheochromocytoma presented to the National Defense Medical College Hospital. CT and MRI showed two adjacent masses in the left adrenal gland; one was a 6 cm cephalic lesion and the other was a 1.5 cm caudal lesion. Only the 1.5 cm caudal mass showed uptake on ¹²³I-metaiodobenzylguanisine single photon emission CT/CT. Pheochromocytoma was suspected and a left adrenalectomy was performed. Pathology confirmed that the 6 cm mass was a ganglioneuroma and the 1.5 cm mass a pheochromocytoma, with cellular intermingling at their border. The two masses were diagnosed as a composite pheochromocytoma-ganglioneuroma. This is the first report in which the two components of a composite pheochromocytoma can be clearly distinguished in the pre-operative images. If a patient with clinically suspected pheochromocytoma has different components from a typical pheochromocytoma, composite pheochromocytoma should be considered.