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Rved, surgery was performed based on the judgment that gross findings indicated that the possibility of malignancy was high. During surgery, a papillary 2.5 ?2.0 ?0.7 cm sized mass was found in the ampulla of Vater. Distant metastasis or?2010 Kwon et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Kwon et al. World Journal of Surgical Oncology 2010, 8:42 http://www.wjso.com/content/8/1/Page 2 ofwas diagnosed as a gangliocytic paraganglioma. During on-going regular follow-up visits no evidence of recurrence or metastasis was observed from December 2007 to April 2009.Figure 1 Esophagogastroduodenoscopic findings showing the periampullary submucosal tumor and surface ulcer bleeding.any of lymph node enlargement were not observed. Pylorus preserving pancreaticoduodenectomy (PPPD) was performed. The pathological result of the excised specimen showed that the tumor was limited to the mucosa and proper muscle layer and had not invaded the pancreas or common bile duct. Furthermore, no lymph node metastasis was detected. The submucosal tumor was found to have a triphasic pattern in low power fields, whereas high power fields showed that the tumor was composed of nests of endocrine cell and ganglion cells with abundant cytoplasm, and spindle cells were found to surround tumor cells (Figures 2A and 2B). Immunohistochemistry showed that tumor cells were positive for synaptophysin, neuron specific antigen, and S-100. In addition, focal positive responses were observed for chromogranin, but no cytokeratin response was observed (Figures 3-A, B, C). Based on the above features, the massFigure 2 (A) The mass lesion showed a triphasic pattern comprised of epithelioid cell nests, neurofibromatous spindle cells and ganglion cells. (?00) (B) Carcinoid-like epithelioid cell nests with surrounding spindle cells. (?00).Discussion Gangliocytic paraganglioma is a rare benign tumor of the digestive tract. Although some have reported cases of gangliocytic paraganglioma invading the proximal jejunum, about 90 are found in the second part of the duodenum, from where the tumor can invade the ampulla of Vater[3]. In the WHO classification of tumors of digestive tract (2000), gangliocytic paraganglioma was independently classified as a type of epithelial tumor. Other duodenal neuroendocrine tumors, except for nondifferentiated neuroendocrine carcinoma, were classified PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/16989806 as carcinoid tumors[4]. Males are affected slightly more commonly than females (1 to 1.8/1) and in terms of age at onset although the fifties are preferred, it has been encountered over an age range from 23 to 83 years[5]. Lenalidomide The endoscopic features of gangliocytic paraganglioma do not differ from those of other submucosal tumors. However, its preoperative pathologic diagnosis is difficult based on endoscopic biopsy alone, because of its submucosal nature, and therefore, endoscopy must be assisted by radioscopy. Gangliocytic paraganglioma is well defined by ultrasonography and is visualized as an isoechoic mass, whereas abdominal computer tomography visualizes it as mass-like soft tissue that is homogenously iso-attenuated, as is observed in muscles beside the vertebrae [6]. Pancreatic head cancer in the duodenum, duodenal cancer, duodenal sarcoma, angiom.