Gangliocytic Paraganglioma, Duodenal

Synonyms

Nonchromaffin paraganglioma of the duodenum

Definition

Gangliocytic paraganglioma is a rare and unique neuroendocrine neoplasm which is currently classified as an epithelial tumor according to the classification of tumors of the digestive tract by the World Health Organization (WHO). The tumor reveals histologic and immunohistochemical features of both neural and endocrine tissues, and histologic diagnosis requires the presence of three different cell lineages: epithelioid cells resembling paraganglioma or well-differentiated neuroendocrine tumor; spindle cells reminiscent of Schwann cells; and ganglion or ganglion-like cells.

Gangliocytic paraganglioma has been considered as a hyperplastic or hamartomatous counterpart to the neoplastic duodenal well-differentiated neuroendocrine tumor (carcinoid), both derived from the same embryonic anlage. Although it is regarded as a benign neoplasm, the benign nature of this tumor is controversial due to the several reports of lymph node...