Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship.

A case of a duodenal paraganglioma in a patient with von Recklinghausen's disease is presented, along with a review of the common clinical features of the 26 cases of duodenal paraganglioma and related neurogenic tumors reported in the literature. There was no reported evidence of recurrence or metastasis. Local excision was the most commonly employed method of treatment. It is proposed that duodenal paraganglioma with its admixed components and variable histologic features may represent hamartomatous hyperplasia of the paraganglia and the supporting neuroid tissue in this area. This would explain the benign behavior of these tumors as compared with carcinoids. Its association, in this case, with neurofibromatosis supports the neural crest origin of duodenal paragangliomas.