Gangliocytic paraganglioma is a rare, typically benign tumor of the gastrointestinal tract most commonly located in the second portion of the duodenum, with a few cases having involved the jejunum and pylorus [5–7]. Burke et al reported that there seems to be a slight male predominance and an average age of 54 at presentation . Other authors have denied that there is any gender preference . This lesion usually presents with abdominal pain and gastrointestinal bleeding due to mucosal ulceration. Obstructive jaundice is less common .
Histologically, our patient's tumor demonstrated the characteristic tricellular pattern of gangliocytic paragangliomas. These tumors are typically composed of an admixture of ganglion cells, spindle cells and epithelial cells [5, 10–13]. These tumors are submucosal, and rarely recur or metastasize [14–16]. In most reported cases regional lymph node involvement, the metastatic cells consist predominantly of epithelial cells . A relatively unique element of the case we present is that six of 7 regional lymph node metastases contained all three characteristic cell types, and thus, demonstrated the possibility for each of these cell types to acquire a malignant potential.
Immunohistochemically these tumors stain positive for a variety of markers as was demonstrated in this report. Such markers include those mentioned above as well as neuron-specific enolase, pancreatic polypeptide, somatostatin, myelin basic protein and neurofilament proteins [6, 12, 13, 17]. The origin of gangliocytic paragangliomas has been widely debated and includes hypotheses ranging from a hamartomatous derivation to cellular elements arising from pancreatic neuroendocrine tissue, or that of the retroperitoneal celiac sympathetic or parasympathetic plexuses [4, 12].
There is no data in the literature to guide clinicians on the use of adjuvant therapy despite the fact that approximately 5% of cases demonstrate malignant features . Since this patient had multiple positive lymph nodes and is relatively young, a trial of adjuvant radiotherapy to the operative bed was considered reasonable and was endorsed by radiation oncologists at high volume cancer centers queried via email.