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A case of
gangliocytic paraganglioma in duodenum.Korean
J Gastroenterol. 2004 Jan;43(1):47-51
Duodenal
gangliocytic paraganglioma derived from neural crest is a peculiar
neuroendocrine tumor. It is incidentally found during radiographic
studies or due to gastrointestinal hemorrhage caused by frequent
ulceration of the overlying mucosa. Most lesions are pedunculated and
submucosal with distinctive histology consisting of endocrine cells,
ganglion cells and spindle-shaped Schwann cells. We experienced a
duodenal gangliocytic paraganglioma in a 40-year-old woman presenting
with episodes of melena. Esophagogastroduodenoscopy revealed a
submucosal ulcerated tumor in the second portion of duodenum and a
biopsy confirmed gangliocytic paraganglioma. The tumor was enucleated
through a duodenotomy. It's size was 5.5 X 3 X 1.5 cm sized and
revealed positive cellular reaction for chromogranin, synaptophysin,
neuron-specific enolase, and neurofilament by immunohistochemistry.
The majority of the reported duodenal gangliocytic paraganglioma were
of benign nature. Therefore, radical surgery or a lymph node
dissection could be avoided if that disease was confirmed. However,
thorough investigation for lymph node metastasis and postoperative
follow-up are needed. We report the first case of a duodenal
gangliocytic paraganglioma in Korea with a review of literature.
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