Gangliocytic paraganglioma of the papilla of Vater with regional lymph node metastasis.

We report a case of duodenal gangliocytic paraganglioma (GP) in a 47-yr-old man. The GP arose in the second portion of the duodenum and was shown by histological examination to consist of epithelioid cells, spindle cells, and ganglion-like cells. Although most of the reported cases of GP have been regarded as benign, in the present case we found that a lymph node in the peripancreatic region contained a metastatic tumor. This is believed to be the third documented case of GP showing regional lymph node metastasis. The metastatic tumor consisted of only epithelioid cells, without spindle cells or ganglion-like cells, supporting the hypothesis that only epithelioid cells have malignant potential.