A patient with nonfunctional paraganglioma situated adjacent to the pancreas is presented. The clinical picture, the laboratory studies, as well as the gross appearance of the tumor and a frozen section at operation were not diagnostic. Internal drainage of the cystic tumor, assumed to be a pseudocyst of the pancreas, was done. Excision was performed when the diagnosis was histologically and ultrastructurally established. The microscopic and the electron microscopic features of this rare neoplasm are discussed. Excision of the tumor is recommended as the treatment of choice because of the malignant potential that does not correlate well with the histological appearance.
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