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Pancreatic neuroendocrin neoplasm

Kathrin Fielitz, Kristina Althoff, Katleen De Preter, Julie Nonnekens, Jasmin Ohli, Sandra Elges, Wolfgang Hartmann, Günter Klöppel, Thomas Knösel, Marc Schulte, Ludger Klein-Hitpass, Daniela Beisser, Henning Reis, Annette Eyking, Elke Cario, Johannes H Schulte, Alexander Schramm, Ulrich Schüller
Amplification or overexpression of MYCN is involved in development and maintenance of multiple malignancies. A subset of these tumors originates from neural precursors, including the most aggressive forms of the childhood tumors, neuroblastoma and medulloblastoma. In order to model the spectrum of MYCN-driven neoplasms in mice, we transgenically overexpressed MYCN under the control of the human GFAP-promoter that, among other targets, drives expression in neural progenitor cells. However, LSL-MYCN;hGFAP-Cre double transgenic mice did neither develop neural crest tumors nor tumors of the central nervous system, but presented with neuroendocrine tumors of the pancreas and, less frequently, the pituitary gland...
October 19, 2016: Oncotarget
Nitya Raj, Emily Valentino, Marinela Capanu, Laura H Tang, Olca Basturk, Brian R Untch, Peter J Allen, David S Klimstra, Diane Reidy-Lagunes
OBJECTIVES: Emerging data suggest that not all grade 3 (G3) pancreatic neuroendocrine neoplasms (panNENs) behave the same; tumor differentiation may predict outcome. METHODS: Patients with G3 panNENs treated at our institution between 1999 and 2014 were identified. Demographics, response to therapy, and overall survival were determined. RESULTS: Forty-five patients were identified, 16 with G3 well differentiated pancreatic neuroendocrine tumors (WD-panNETs) and 29 with poorly differentiated neuroendocrine carcinomas (PDNEC)...
October 18, 2016: Pancreas
Yugang Cheng, Hanxiang Zhan, Lei Wang, Jianwei Xu, Guangyong Zhang, Zongli Zhang, Sanyuan Hu
The incidence rate of pancreatic neuroendocrine neoplasms (pNENs) has increased rapidly in recent years. However, the clinicopathological characteristics of pNENs are poorly understood. Medical records of patients who underwent surgery and were confirmed as pNENs by pathological examination from January 2003 to February 2015 in Qilu Hospital were reviewed retrospectively. A total of 100 patients, 36 males and 64 females, were included with a mean operation age of 46.26 + 13.41 years. Among the 100 cases, 76 had insulinomas and 24 had non-functional pNENs...
October 17, 2016: Frontiers of Medicine
Simon Schimmack, Ben Lawrence, Barton Kenney, Hubertus Schmitz-Winnenthal, Irvin M Modlin, Mark Kidd
BACKGROUND: Small intestinal neuroendocrine neoplasm (SI-NEN) proliferation is quantified by Ki67 measurements which capture G1-G2M phases of the cell cycle. G0 and early G1 phases, typical of slow-growing cells, can be detected by minichromosome maintenance protein (MCM) expression. We hypothesized that these replication licensing markers may provide clinically relevant information to augment Ki67 in low-grade neuroendocrine neoplasia. METHODS: Immunohistochemical staining (IHC), Western blot analysis, quantitative polymerase chain reaction, and copy number variations of MCM2, MCM3, and Ki67 were undertaken in SI-NENs (n = 22)...
October 2016: Translational Oncology
Hazem M Zakaria, John A Stauffer, Massimo Raimondo, Timothy A Woodward, Michael B Wallace, Horacio J Asbun
AIM: To identify the current indications and outcomes of total pancreatectomy at a high-volume center. METHODS: A single institutional retrospective study of patients undergoing total pancreatectomy from 1995 to 2014 was performed. RESULTS: One hundred and three patients underwent total pancreatectomy for indications including: Pancreatic ductal adenocarcinoma (n = 42, 40.8%), intraductal papillary mucinous neoplasms (n = 40, 38.8%), chronic pancreatitis (n = 8, 7...
September 27, 2016: World Journal of Gastrointestinal Surgery
S Partelli, R Cirocchi, S Crippa, L Cardinali, V Fendrich, D K Bartsch, M Falconi
BACKGROUND: The incidence of asymptomatic, sporadic, small non-functioning pancreatic neuroendocrine neoplasms (NF-PNENs) has increased in recent decades. Conservative treatment has been advocated for these tumours. The aim of this study was systematically to evaluate the literature on active surveillance and to compare this with surgical management for asymptomatic sporadic small NF-PNENs. METHODS: PubMed, Embase and the Cochrane Library were searched systematically for studies that compared the active surveillance of asymptomatic, sporadic, small NF-PNENs with surgical management...
October 5, 2016: British Journal of Surgery
Selma J Lekkerkerker, Marc G Besselink, Olivier R Busch, Joanne Verheij, Marc R Engelbrecht, Erik A Rauws, Paul Fockens, Jeanin E van Hooft
BACKGROUND AND AIMS: Currently, 3 guidelines are available for the management of pancreatic cysts. These guidelines vary in their indication for resection of high-risk cysts. We retrospectively compared the final pathological outcome of surgically removed pancreatic cysts with the indications for resection according to 3 different guidelines. METHODS: Patients who underwent pancreatic resection were extracted from our prospective pancreatic cyst database (2006-present)...
September 29, 2016: Gastrointestinal Endoscopy
G C Ginesu, M Barmina, P Paliogiannis, M Trombetta, M L Cossu, C F Feo, F Addis, A Porcu
BACKGROUND: Paragangliomas are rare neoplasms that originate from the neural crest. They are malignant in approximately 10% of cases, with a 50% survival rate at 5 years from diagnosis. In most cases, manifestations of malignancy (such as metastasis) are lacking, and paragangliomas are considered benign lesions. Pancreatic paragangliomas are extremely rare, with only 31 cases described in the scientific literature to date. CASE SUMMARY: Here we describe a case of a 55-year-old Caucasian male patient referred to our institution in September 2013 for lumbar pain lasting five months...
September 21, 2016: International Journal of Surgery Case Reports
Lene E Johannessen, Ioannis Panagopoulos, Sven-Petter Haugvik, Ivar Prydz Gladhaug, Sverre Heim, Francesca Micci
Fusion transcripts arising from the combination of exons residing on neighboring genes on the same chromosome may give rise to chimeric or novel proteins. Such read-through transcripts have been detected in different cancers where they may be of pathogenetic interest. In this study, we describe for the first time the expression of a read-through transcript in insulinomas, a functioning neuroendocrine pancreatic neoplasm. The read-through transcript INS-IGF2, composed of exons from the two genes proinsulin precursor (INS) and insulin‑like growth factor 2 (IGF2), both mapping to chromosomal subband 11p15...
September 23, 2016: Oncology Reports
Joo Young Kim, Jacqueline A Brosnan-Cashman, Soyeon An, Sung Joo Kim, Ki Byung Song, Min-Sun Kim, Mi-Ju Kim, Dae Wook Hwang, Alan K Meeker, Eunsil Yu, Song Cheol Kim, Ralph H Hruban, Christopher M Heaphy, Seung-Mo Hong
PURPOSE: Alternative lengthening of telomeres (ALT), a telomerase-independent telomere maintenance mechanism, is strongly associated with ATRX and DAXX alterations and occurs frequently in pancreatic neuroendocrine tumors (PanNETs). EXPERIMENTAL DESIGN: In a Korean cohort of 269 surgically resected primary PanNETs and 19 sporadic microadenomas, ALT status and nuclear ATRX and DAXX protein expression were assessed and compared with clinicopathologic factors. RESULTS: In PanNETs, ALT or loss of ATRX/DAXX nuclear expression was observed in 20...
September 23, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Stergios Boussios, Ioannis Zerdes, Ourania Batsi, Vasilios P Papakostas, Esmeralda Seraj, George Pentheroudakis, George K Glantzounis
INTRODUCTION: Pancreatic metastases are uncommon and only found in a minority of patients with widespread metastatic disease at autopsy. The most common primary cancer site resulting in pancreatic metastases is the kidney, followed by colorectal cancer, melanoma, breast cancer, lung carcinoma and sarcoma. PRESENTATION OF CASE: Herein, we report a 63-year-old male patient who presented -3.5 years after radical nephrectomy performed for renal cell carcinoma (RCC)-with a well-defined lobular, round mass at the body of the pancreas demonstrated by abdominal Magnetic Resonance Imaging (MRI)...
2016: International Journal of Surgery Case Reports
Shigeru Horiguchi, Hironari Kato, Hidenori Shiraha, Koichiro Tsutsumi, Naoki Yamamoto, Kazuyuki Matsumoto, Takeshi Tomoda, Daisuke Uchida, Yutaka Akimoto, Syou Mizukawa, Takehiro Tanaka, Koichi Ichimura, Akinobu Takaki, Takahito Yagi, Hiroyuki Okada
BACKGROUND AND AIM: Pathological grading is important in defining the therapeutic strategy in pancreatic neuroendocrine neoplasm (PNEN), but is difficult for unresectable cases. Endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) is useful in the diagnosis of PNEN, but its usefulness for pathological grading is not well established. No studies have examined the diagnostic ability of dynamic computed tomography (CT) for pathological grading of PNEN. We investigated the usefulness of EUS-FNA and dynamic CT in the diagnosis and pathological grading of PNEN...
September 16, 2016: Journal of Gastroenterology and Hepatology
David Altree-Tacha, Jillian Tyrrell, Faqian Li
Context .- High-grade neuroendocrine carcinomas and carcinoids can arise in different sites such as lung, gastrointestinal tract, prostate, and skin. Classic neuroendocrine markers such as CD56, synaptophysin, and chromogranin cannot distinguish carcinoids from high-grade neuroendocrine carcinomas. Recently, mouse monoclonal mASH1 has been shown to help discriminate carcinoids from high-grade neuroendocrine carcinomas in various neoplastic sites. To date, there have been no comprehensive immunohistochemistry studies with mASH1 on nonneuroendocrine neoplasms...
September 15, 2016: Archives of Pathology & Laboratory Medicine
Jennifer J Findeis-Hosey, Kelly Q McMahon, Sarah K Findeis
Von Hippel-Lindau disease is an autosomal dominant syndrome which occurs secondary to germline mutations in the VHL tumor suppressor gene, located on chromosome 3. Clinically von Hippel-Lindau disease is characterized by an increased risk of developing simple visceral cysts, most commonly in the pancreas and kidneys, in addition to an increased risk of developing neoplasms, often with clear cell features, in a multitude of organ systems. The most common neoplasms are cerebellar and retinal hemangioblastomas, adrenal pheochromocytomas, clear cell renal cell carcinomas, pancreatic neuroendocrine tumors, pancreatic serous cystadenomas, and endolymphatic sac tumors...
June 2016: Journal of Pediatric Genetics
Sofia Xenaki, Konstantinos Lasithiotakis, Alexandros Andreou, Sofia Aggelaki, Maria Tzardi, Anna Daskalaki, George Chalkiadakis, Emmanuel Chrysos
Introduction. Neuroendocrine carcinoma (NEC) of pancreas is a rare tumor with aggressive progression and poor prognosis. Its coexistence with adenocarcinoma poses significant clinical problems and has not been addressed in the literature. Methods. We describe a case of a 51-year-old male who underwent pancreatoduodenectomy due to pancreatic head tumor 1.5 × 1 × 1.4 cm. Histological examination of the specimen revealed a mixed neoplasm: (1) a well differentiated adenocarcinoma, neoplastic blasts of which are extended focally to the submucosa without invading the muscular layer, and (2) a low differentiated NEC consisting of solid clusters and pagetoid formations...
2016: Case Reports in Surgery
Rinaldo Pellicano, Sharmila Fagoonee, Fiorella Altruda, Mauro Bruno, Giorgio M Saracco, Claudio De Angelis
Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a heterogeneous group of tumors deriving from the gastrointestinal (GI) neuroendocrine system. Since these neoplasms are usually very small, located deeply within the retroperitoneum or into an extramucosal site of the GI tract and, lastly, because they may be multi-sited, radiological imaging modalities, in combination with endoscopy, are the diagnostic workhorses in patients with GEP-NETs. Endoscopic approach is useful for detection, bioptic diagnosis and curative resection of small GEP-NETs of stomach, duodenum, jejuno-ileum, and colon-rectum...
December 2016: Minerva Endocrinologica
John R Bergquist, Christopher R Shubert, Daniel S Ubl, Cornelius A Thiels, Michael L Kendrick, Mark J Truty, Elizabeth B Habermann
BACKGROUND: Expected mortality after elective pancreaticoduodenectomy (PD) in contemporary series is less than 5% in elderly patients; however, to our knowledge, mortality rate has not been correlated with indication for PD. We hypothesized that perioperative risk following PD would correlate with diagnostic indication in older patients. METHODS: The American College of Surgeons NSQIP database was reviewed to identify patients (<80 and ≥80 years) who underwent PD from January 1, 2005, through December 31, 2012...
September 1, 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Vivian Weiss, Julie Dueber, Jesse P Wright, Justin Cates, Frank Revetta, Alexander A Parikh, Nipun B Merchant, Chanjuan Shi
AIM: To investigate the role of the Wnt/β-catenin pathway in pancreatic neuroendocrine neoplasms (PanNENs). METHODS: Tissue microarrays containing 88 PanNENs were immunohistochemically labeled with antibodies to β-catenin, E-cadherin, adenomatous polyposis coli (APC), chromogranin and synaptophysin. One case had only metastatic tumors resected, whereas others (n = 87) received pancreatectomy with or without partial hepatectomy. Pathology slides, demographic, clinicopathologic, and follow up data were reviewed...
August 15, 2016: World Journal of Gastrointestinal Oncology
Yatong Li, Menghua Dai, Xiaoyan Chang, Wendi Hu, Jie Chen, Junchao Guo, Wenming Wu, Taiping Zhang, Quan Liao, Ziwen Liu, Ya Hu, Yupei Zhao
INTRODUCTION: The aim of this study was to report a new case of mixed serous neuroendocrine neoplasm (MSNN) and review the literature concerning this type of lesion, which was added to the World Health Organization classification of pancreatic tumors in 2010. RESULTS: A 73-year-old woman presented with a pancreatic mass. The lesion was an intriguing combination of serous cystic neoplasm (SCN) and pancreatic neuroendocrine tumor (PanNET), in which the PanNET component grew into the wall of the serous oligocystic adenoma...
August 2016: Medicine (Baltimore)
Tetsuhide Ito, Susumu Hijioka, Toshihiko Masui, Atsuko Kasajima, Yuji Nakamoto, Noritoshi Kobayashi, Izumi Komoto, Masayuki Hijioka, Lingaku Lee, Hisato Igarashi, Robert Thomas Jensen, Masayuki Imamura
Several new developments have occurred in the field of pancreatic neuroendocrine neoplasm (PNEN) recently in Japan. First, the utility of chromogranin A (CgA), useful for the diagnosis and monitoring of the treatment response of neuroendocrine neoplasm (NEN), has been demonstrated in Japan. For PNEN diagnosis and treatment, grading and correct histological diagnosis according to the WHO 2010 classification is important. Regarding the histological diagnosis, the advent of endoscopic ultrasonography-guided fine-needle aspiration (EUS-FNA) has enabled correct pathological diagnosis and suitable treatment for the affected tissue...
August 18, 2016: Journal of Gastroenterology
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