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Duodenal Gangliocytic paraganglioma

Keisuke Nonaka, Yoko Matsuda, Akira Okaniwa, Atsuko Kasajima, Hironobu Sasano, Tomio Arai
BACKGROUND: Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown. CASE PRESENTATION: A nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman...
August 2, 2017: Diagnostic Pathology
Sahara J Cathcart, Aaron R Sasson, Jessica A Kozel, Jennifer M Oliveto, Quan P Ly
Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy...
June 16, 2017: World Journal of Clinical Cases
Ming Yang, Jonathan A Leighton, Douglas O Faigel, Nitin N Katariya
No abstract text is available yet for this article.
October 2017: Digestive and Liver Disease
Hiroyuki Matsubayashi, Hirotoshi Ishiwatari, Toru Matsui, Shinya Fujie, Katsuhiko Uesaka, Teiichi Sugiura, Yukiyasu Okamura, Yusuke Yamamoto, Ryo Ashida, Takaaki Ito, Keiko Sasaki, Hiroyuki Ono
A duodenal polyp was found during a health check of a 71-year-old asymptomatic man. Duodenoscopy demonstrated a pedunculated, smooth-surfaced tumor of 18 mm in size, protruding from the minor papilla. Endoscopic ultrasonography demonstrated a homogeneously low-echoic submucosal tumor. Enhanced computed tomography and magnetic resonance imaging demonstrated a well-enhanced duodenal tumor without obvious metastasis. A tumor biopsy revealed a well-differentiated neuroendocrine tumor, and laparotomic transduodenal polypectomy with regional lymph node dissection was performed...
2017: Internal Medicine
B D Palomino-Martínez, H Espino-Cortés, J Cerna-Cardona, L E Godínez-Martínez, M A Chávez-García
No abstract text is available yet for this article.
March 16, 2017: Revista de Gastroenterología de México
Dimetrios Papaconstantinou, Nikolaos Machairas, Vasileia Damaskou, Nikolaos Zavras, Christine Kontopoulou, Anastasios Machairas
INTRODUCTION: Duodenal gangliocytic paragangliomas are rare neoplasms often arising in proximity to the major duodenal papilla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastatic disease even more so. Resection of the tumor is the only definitive therapy. PRESENTATION OF CASE: A 67year old male presented to a referring hospital with symptoms of fatigue and malaise. Evaluation with CT imaging revealed a 3...
2017: International Journal of Surgery Case Reports
Yuichi Sato, Satoru Hashimoto, Ken-Ichi Mizuno, Manabu Takeuchi, Shuji Terai
Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, like all NETs. However, the incidence of GI-NETS has been increasing in recent years. Gastric NETs (G-NETs) and duodenal NETs (D-NETs) are the common types of upper GI-NETs based on tumor location. G-NETs are classified into three distinct subgroups: type I, II, and III. Type I G-NETs, which are the most common subtype (70%-80% of all G-NETs), are associated with chronic atrophic gastritis, including autoimmune gastritis and Helicobacter pylori associated atrophic gastritis...
August 14, 2016: World Journal of Gastroenterology: WJG
Zhengping Zhuang, Chunzhang Yang, Ales Ryska, Yuan Ji, Yingyong Hou, Sky D Graybill, Petra Bullova, Irina A Lubensky, Günter Klöppel, Karel Pacak
No abstract text is available yet for this article.
May 2016: Endocrine-related Cancer
Alessandro Vanoli, Stefano La Rosa, Catherine Klersy, Federica Grillo, Luca Albarello, Frediano Inzani, Roberta Maragliano, Rachele Manca, Ombretta Luinetti, Massimo Milione, Claudio Doglioni, Guido Rindi, Carlo Capella, Enrico Solcia
BACKGROUND: Several types of neuroendocrine neoplasms (NENs) have been described in the duodenal tract, from low-grade tumors (NETs) to high-grade neuroendocrine carcinomas (NECs). A comprehensive analysis of histology, hormonal profile and prognostic parameters of a sufficiently large duodenal NEN series to cover all main kinds of neoplasms is however lacking. METHODS: We collected a retrospective series of 203 duodenal wall and ampullary region NENs, from six specialized endocrine pathology centers...
2017: Neuroendocrinology
Hyung Kyu Park, Hye Seung Han
Gangliocytic paraganglioma is a rare tumor that occurs most commonly in the second portion of the duodenum. It is characterized by its triphasic cellular differentiation: epithelioid neuroendocrine cells, spindle cells with Schwann cell differentiation, and ganglion cells. Most gangliocytic paragangliomas are considered benign and are amenable to local excision. However, to our knowledge, 23 cases with lymph node metastasis have been reported, 1 case of bone metastasis, and 2 cases of liver metastases. Predictive factors that have been suggested for lymph node metastasis include size (larger than 2 cm), young age, and tumors exceeding the submucosal layer...
January 2016: Archives of Pathology & Laboratory Medicine
Bo Wang, Yinying Zou, Han Zhang, Liming Xu, Xiaojun Jiang, Ke Sun
We report two cases of Gangliocytic paraganglioma (GP), one of which was accompanied by lymph node metastasis. Histologically, the tumor was composed of three morphologically distinct cell populations: spindle cells, ganglion-like cells and epithelioid cells. The epithelioid cells were positive for cytokeratin (AE1/AE3), synaptophysin (Syn), chromogranin A (CgA), CD56 and progesterone receptor (PR). Ganglion-like cell types showed positive reactivity for Syn and CD56. In contrast, the spindle-shaped cells showed positive reactivity for S-100...
2015: International Journal of Clinical and Experimental Pathology
Iolanda Ribeiro, Carlos Fernandes, Sónia Fernandes, Luísa Proença, Joana Silva, Ana Ponte, Jaime Rodrigues, João Carvalho
No abstract text is available yet for this article.
November 2016: Gastroenterología y Hepatología
Pradnya V Manglekar, Vijayshree Bhide, Nachiket A Dubale, Amol Bapaye
No abstract text is available yet for this article.
November 2015: Gastrointestinal Endoscopy
Yoichiro Okubo, Tetsuo Nemoto, Megumi Wakayama, Naobumi Tochigi, Minoru Shinozaki, Takao Ishiwatari, Kyoko Aki, Masaru Tsuchiya, Hajime Aoyama, Kanade Katsura, Takeshi Fujii, Takashi Nishigami, Tomoyuki Yokose, Yasuo Ohkura, Kazutoshi Shibuya
BACKGROUND: Gangliocytic paraganglioma (GP) is an extremely rare benign tumor that commonly arises from the second part of the duodenum. Since GP exhibit neither prominent mitotic activity nor Ki-67 immunoreactivity, this tumor is often misdiagnosed as neuroendocrine tumor (NET) G1 (carcinoid tumor). However, patients with GP may have a better prognosis than patients with NET G1. This fact emphasizes the importance of differentiating GP from NET G1, but few studies have reported the epidemiology and histopathology of GP because of its rarity...
2015: BMC Cancer
Alina Boeriu, Daniela Dobru, Rareș Georgescu, Simona Mocan, Cristian Boeriu
Duodenal neuroendocrine tumors (NETs) are rare tumors, consisting of five different types of tumors. In many cases, they may be asymptomatic, leading to delay in diagnosis. Clinical symptoms are related to local tumor growth and mucosal ulceration. We report a 38-year old man with duodenal gangliocytic paraganglioma causing overt upper gastrointestinal bleeding and anemia. We describe specific clinical and histopathological features of the tumor, and review the diagnostic and therapeutic strategy. Gangliocytic paragangliomas are regarded as benign tumors...
March 2015: Journal of Gastrointestinal and Liver Diseases: JGLD
Tyler J Loftus, Jesse L Kresak, David H Gonzalo, George A Sarosi, Kevin E Behrns
INTRODUCTION: Gangliocytic paraganglioma is a rare tumor that is most commonly located in the duodenum. At presentation, it may be confused with a gastrointestinal stromal tumor (GIST), but distinguishing between these tumors is critical because the natural history and treatment of these two tumors differs markedly. Duodenal gangliocytic paraganglioma typically exhibits benign behavior with occasional regional lymph node metastasis and no reports of tumor associated deaths. Recurrence after resection is rare...
2015: International Journal of Surgery Case Reports
Nicholas Lau, Danielle Hari, Samuel French
We present a case of a 49-year-old woman who underwent a pancreaticoduodenectomy for symptomatic treatment of an obstructive periampullary duodenal mass initially found on CT imaging. Histologically, the tumor showed a triphasic pattern including small round cells, a spindle-cell component and ganglion cells of varying size. Furthermore, the tumor was surrounded by a proliferation of pancreatic ducts. These features led to the diagnosis of a gangliocytic paraganglioma arising in an ectopic pancreas. Sections of the tumor were then stained for SOX10, a neural crest transcription factor, and it was shown to be positive in the ganglion cells...
February 2015: Experimental and Molecular Pathology
Bin Li, Yang Li, Xiao-Ying Tian, Bo-Ning Luo, Zhi Li
Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery...
November 7, 2014: World Journal of Gastroenterology: WJG
Stephanie Downs-Canner, Wald J Van der Vliet, Stijn J J Thoolen, Brian A Boone, Amer H Zureikat, Melissa E Hogg, David L Bartlett, Mark P Callery, Tara S Kent, Herbert J Zeh, A James Moser
INTRODUCTION: Benign duodenal and periampullary tumors are uncommon lesions requiring careful attention to their complex anatomic relationships with the major and minor papillae as well as the gastric outlet during surgical intervention. While endoscopy is less morbid than open resection, many lesions are not amenable to endoscopic removal. Robotic surgery offers technical advantages above traditional laparoscopy, and we demonstrate the safety and feasibility of this approach for a variety of duodenal lesions...
February 2015: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
R Bhattacharyya, P Bhandari
INTRODUCTION: It is very difficult to establish an accurate diagnosis for upper GI submucosal tumours. Biopsy during endoscopy cannot go deep enough. EUS is unable to give a tissue diagnosis. The risks of surgical resection are higher than the benefits as the lesion may very well be benign. As a result most of these patients keep having endoscopic surveillance as 'possible' GISTs. METHODS: A retrospective cohort study of patients undergoing ESD for upper GI submucosal tumours...
June 2014: Gut
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