Duodenal gangliocytic paraganglioma as a radiological moving defect

Background: Gangliocytic paraganglioma of the duodenum is a peculiar neuroe ndocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucose. Most lesions are pedunculated and submucosal with distinc tive histology, consisting of endocrine, ganglion and spindle-shaped Schwan n cells. Methods: A case of a duodenal gangliocytic paraganglioma is report ed in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portio n of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography, T he tumor was simply excised through a duodenotomy. Results: The size of the tumor was 4.5 x 3 x 1.5 cm. Immunohistochemical and ultrastructural featur es of the tumor classified it as a typical duodenal gangliocytic paragangli oma with positive cellular reaction for neuron-specific enolase, chromogran in, somatostatin, gastrin and S100. Conclusion: Forty-six months after surg ery, the patient is well with no evidence of recurrence. The majority of th e reported duodenal gangliocytic paragangliomas were of bening nature. Howe ver, the fact that in 4 isolated cases there was lymph node involvement ind icates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up. Copyright (C) 2000 S. Karger AG, Basel.