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https://www.readbyqxmd.com/read/27863775/operation-for-insulinomas-in-multiple-endocrine-neoplasia-type-1-when-pancreatoduodenectomy-is-appropriate
#1
Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi
BACKGROUND: Distal pancreatectomy is the most frequent operation for insulinomas complicating multiple endocrine neoplasia type 1 insulinoma, although there are conditions for which a different operative approach might be preferable. In this article, we report the operative experience of a referral center for multiple endocrine neoplasia type 1 insulinoma. METHODS: Twelve patients underwent operations between 1992 and 2015: 8 underwent a distal pancreatic resection, and 4 underwent a pancreatoduodenectomy...
November 15, 2016: Surgery
https://www.readbyqxmd.com/read/27822633/survival-analysis-and-risk-for-progression-of-intraductal-papillary-mucinous-neoplasia-of-the-pancreas-ipmn-under-surveillance-a-single-institution-experience
#2
Marco Del Chiaro, Zeeshan Ateeb, Marcus Reuterwall Hansson, Elena Rangelova, Ralf Segersvärd, Nikolaos Kartalis, Christoph Ansorge, Matthias J Löhr, Urban Arnelo, Caroline Verbeke
PURPOSE: While surveillance of the majority of patients with IPMN is considered best practice, consensus regarding the duration of follow-up is lacking. This study assessed the survival rate and risk for progression of IPMN under surveillance. METHODS: All patients diagnosed with and surveyed for IPMN between January 2008 and December 2013 were identified and assigned to two groups: patients without indication for surgery (Group 1), and patients whose IPMN required surgery but were inoperable for general reasons (Group 2)...
November 7, 2016: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/27816353/predisposing-factors-for-pancreatic-adenocarcinoma-what-is-the-role-of-imaging
#3
M-P Vullierme, M Lagadec
Early detection of pancreatic adenocarcinoma is the goal of imaging, enabling curative surgery. The identification of high-grade dysplastic precursor lesions is even more beneficial. Two forms are now better known: pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasm (IPMN). To detect these lesions with imaging, we need to know the patterns associated with them. A screening program could then be used to pinpoint them. This program could not be applied to the entire population...
November 2, 2016: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/27811505/early-and-late-complications-after-surgery-for-men1-related-nonfunctioning-pancreatic-neuroendocrine-tumors
#4
Sjoerd Nell, Inne H M Borel Rinkes, Helena M Verkooijen, Bert A Bonsing, Casper H van Eijck, Harry van Goor, Ruben H J de Kleine, Geert Kazemier, Elisabeth J Nieveen van Dijkum, Cornelis H C Dejong, Gerlof D Valk, Menno R Vriens
OBJECTIVE: To estimate short and long-term morbidity after pancreatic surgery for multiple endocrine neoplasia type 1 (MEN1)-related nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs). BACKGROUND: Fifty percent of the MEN1 patients harbor multiple NF-pNETs. The decision to proceed to NF-pNET surgery is a balance between the risk of disease progression versus the risk of surgery-related morbidity. Currently, there are insufficient data on the surgical complications after MEN1 NF-pNET surgery...
November 1, 2016: Annals of Surgery
https://www.readbyqxmd.com/read/27803029/prkar1a-gene-knockout-in-the-pancreas-leads-to-neuroendocrine-tumorigenesis
#5
Emmanouil Saloustros, Paraskevi Salpea, Matthew Starost, Sissi Liu, Fabio R Faucz, Edra London, Eva Szarek, Woo-Jin Song, Mehboob Hussain, Constantine A Stratakis
Carney complex (CNC) is a rare disease associated with multiple neoplasias, including a predisposition to pancreatic tumors; it is caused most frequently by the inactivation of the PRKAR1A gene, a regulator of the cyclic AMP (cAMP)-dependent kinase (PKA). The method used was to create null alleles of prkar1a in mouse cells expressing pdx1 (Δ-Prkar1a). We found that these mice developed endocrine or mixed endocrine/acinar cell carcinomas with 100% penetrance by the age of 4-5 months. Malignant behavior of the tumors was seen as evidenced by stromal invasion and metastasis to locoregional lymph nodes...
January 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27799361/de-novo-mutation-in-men1-is-not-associated-with-parental-somatic-mosaicism
#6
Yael Laitman, Anat Jaffe, Hagit Schayek, Eitan Friedman
Extract: Dear Editor, Multiple Endocrine Neoplasia Type 1 (MEN1- OMIM #131100) is characterized by the co-occurrence of tumors in at least two of the following three endocrine tissues: parathyroid, endocrine pancreas and anterior pituitary [Thakker 2014]. Familial MEN1 follows an autosomal dominant mode of inheritance and germline mutations in the MEN1 gene at 11q13 can be detected in affected family members [Chandrasekharappa et al 1997]. Mutations in the CDKN1B gene (CDKN1B, KIP1, OMIM #600778) have been also reported in MEN1 families but are far less frequent [Pellegata et al 2006]...
October 31, 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27795812/mucins-in-neoplasms-of-pancreas-ampulla-of-vater-and-biliary-system
#7
REVIEW
Dimitrios Moschovis, Giorgos Bamias, Ioanna Delladetsima
Tumors of the pancreas, the ampulla of Vater, and the extrahepatic and intrahepatic bile ducts have significant histological similarities due to the common embryonic origin of the pancreatobiliary system. This obviates the need for discovery of biomarkers with diagnostic and prognostic value for these tumors. Mucins, especially MUC-1, -2, -4 and -5AC, are important candidates for developing into such reliable biomarkers. Increased expression of MUC1 occurs in pancreatic ductal adenocarcinomas and is associated with increased degrees of dysplasia in pancreatic intraepithelial neoplasia (PanIN)...
October 15, 2016: World Journal of Gastrointestinal Oncology
https://www.readbyqxmd.com/read/27788047/updates-on-precancerous-lesions-of-the-biliary-tract-biliary-precancerous-lesion
#8
Sanaz Ainechi, Hwajeong Lee
Precursor lesions of invasive adenocarcinoma of the bile duct (cholangiocarcinoma) have been increasingly recognized during the past decade because of the results of multiple studies on the carcinogenesis of cholangiocarcinoma, technologic advancements in diagnostic imaging modalities, and an increase in the volume of elective procedures. The two main precursor lesions of cholangiocarcinoma that have evolved are biliary intraepithelial neoplasia and intraductal papillary neoplasm of the bile duct. These lesions demonstrate histomorphologic similarities to pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasm of the pancreas, respectively, whereas mechanisms of carcinogenesis and risk of progressive disease appear distinct...
November 2016: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/27783689/epithelial-to-stromal-re-distribution-of-primary-cilia-during-pancreatic-carcinogenesis
#9
Simon Schimmack, Sarah Kneller, Nigora Dadabaeva, Frank Bergmann, Andrew Taylor, Thilo Hackert, Jens Werner, Oliver Strobel
BACKGROUND: The Hedgehog (HH) pathway is a mediator in pancreatic ductal adenocarcinoma (PDAC). Surprisingly, previous studies suggested that primary cilia (PC), the essential organelles for HH signal transduction, were lost in PDAC. The aim of this study was to determine the presence of PC in human normal pancreas, chronic pancreatitis, and during carcinogenesis to PDAC with focus on both epithelia and stroma. METHODS: PC were analyzed in paraffin sections from normal pancreas, chronic pancreatitis, intraductal papillary-mucinous neoplasia, and PDAC, as well as in primary human pancreatic stellate cells (PSC) and pancreatic cancer cell lines by double immunofluorescence staining for acetylated α-tubuline and γ-tubuline...
2016: PloS One
https://www.readbyqxmd.com/read/27738535/pancreatic-neuroendocrine-tumor-in-the-setting-of-dorsal-agenesis-of-the-pancreas
#10
Samih Nassif, Cecilia Ponchiardi, Teviah Sachs
Dorsal agenesis of the pancreas (DAP) is an uncommon embryological abnormality where there is absence of the distal pancreas. DAP is mostly asymptomatic, but common presenting symptoms include diabetes mellitus, abdominal pain, pancreatitis, enlarged pancreatic head, and, in a few cases, polysplenia. MRCP and ERCP are the gold standard imaging techniques to demonstrate the absence of the dorsal pancreatic duct. The literature on the association of pancreatic neoplasia and DAP is limited. We present the case of a pancreatic neuroendocrine tumor in a patient with dorsal agenesis of the pancreas, with a review of the related literature...
2016: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/27734708/-multiple-endocrine-neoplasia-i-wermers-syndrome-forms-of-clinical-manifestation-5-case-studies
#11
Karolína Drbalová, Kateřina Herdová, Petr Krejčí, Monika Nývltová, Svatopluk Solař, Lenka Vedralová, Pavel Záruba, David Netuka, Petr Bavor
Multiple Endocrine Neoplasia (MEN) is a condition in which several endocrine organs of an individual are affected by adenoma, hyperplasia and less often carcinoma, either simultaneously or at different stages of life. Two existing syndromes, MEN1 and MEN2 (2A, 2B), in literature is also mentioned MEN4, are associated also with other non-endocrine disorders. MEN1 (Wermer syndrome) affects the pituitary, parathyroid, and pancreatic area. 95 % of patients show very early manifestation of hyperparathyroidism, often before 40 years of age...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27673548/nonmucinous-biliary-epithelium-is-a-frequent-finding-and-is-often-the-predominant-epithelial-type-in-mucinous-cystic-neoplasms-of-the-pancreas-and-liver
#12
Kristen Zhelnin, Yue Xue, Brian Quigley, Michelle D Reid, Hyejeong Choi, Bahar Memis, Volkan Adsay, Alyssa M Krasinskas
Mucinous cystic neoplasms (MCNs) can occur in the pancreas and liver. Classically, these cystic lesions are lined by columnar mucinous epithelium with underlying ovarian-type stroma. It has been proposed that cysts with ovarian-type stroma and nonmucinous epithelium be considered separate entities in both the pancreas and liver. Using a series of 104 pancreatic and 32 hepatic cases, we aimed to further characterize the epithelium present in MCNs. Mucinous epithelium was defined as pancreatic intraepithelial neoplasia-like columnar cells with pale pink/clear apical mucin...
September 26, 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27670143/pancreatic-neuroendocrine-microadenomatosis-a-case-report-of-cytology-and-histology-correlation
#13
Nguyen Vo, David W Cohen, Mary E Dillhoff, Ming Jin
Pancreatic neuroendocrine neoplasia is categorized by grade, size, and functional status. Neuroendocrine microadenoma (NEMA) is defined as a neuroendocrine tumor (NET) that measures less than 0.5 cm in diameter. Multiple NEMAs are termed neuroendocrine adenomatosis (NEMAtosis). The surgical pathology and clinical aspects of pancreatic NEMAtosis have been reported, but there has been no report regarding EUS-FNA cytology of NEMAtosis. We report a case of NEMAtosis with cytologic correlation and illustrate the diagnostic challenges and potential pitfalls...
September 27, 2016: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/27639167/hes1-controls-exocrine-cell-plasticity-and-restricts-development-of-pancreatic-ductal-adenocarcinoma-in-a-mouse-model
#14
Ana Hidalgo-Sastre, Roxanne L Brodylo, Clara Lubeseder-Martellato, Bence Sipos, Katja Steiger, Marcel Lee, Guido von Figura, Barbara Grünwald, Suyang Zhong, Marija Trajkovic-Arsic, Florian Neff, Roland M Schmid, Jens T Siveke
Perturbation of the pancreatic acinar cell state can lead to acinar-to-ductal metaplasia (ADM), which is a precursor lesion to the development of pancreatic ductal adenocarcinoma (PDAC). In the pancreas, Notch signaling is active both during development and in adult cellular differentiation processes. Hes1, a key downstream target of the Notch signaling pathway, is expressed in the centroacinar compartment of the adult pancreas as well as in both preneoplastic and malignant lesions. In this study, we used a murine genetic in vivo approach to ablate Hes1 in pancreatic progenitor cells (Ptf1a(+/Cre); Hes1(fl/fl))...
September 14, 2016: American Journal of Pathology
https://www.readbyqxmd.com/read/27600643/endoscopic-imaging-in-the-management-of-gastroenteropancreatic-neuroendocrine-tumors
#15
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
https://www.readbyqxmd.com/read/27572829/concomitant-existence-of-pheochromocytoma-in-a-patient-with-multiple-endocrine-neoplasia-type-1
#16
Ryo Okada, Tatsuo Shimura, Shigeyuki Tsukida, Jin Ando, Yasuhide Kofunato, Tomoyuki Momma, Rei Yashima, Yoshihisa Koyama, Shinichi Suzuki, Seiichi Takenoshita
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal-dominant inherited disorder that is classically characterized by the presence of neoplastic lesions of the parathyroid glands, the anterior pituitary gland, and the pancreas. However, MEN1 with concomitant pheochromocytoma is extremely rare. CASE REPORT: We report a case of MEN1 concomitant with pheochromocytoma. A 44-year-old Japanese man, who had undergone total parathyroidectomy due to primary hyperparathyroidism at the age of 18, was referred to our hospital with a complaint of a large abdominal tumor...
December 2016: Surgical Case Reports
https://www.readbyqxmd.com/read/27545110/histological-changes-in-endocrine-and-exocrine-pancreatic-tissue-from-patients-exposed-to-incretin-based-therapies
#17
Sandra Ueberberg, Hendrik Jütte, Waldemar Uhl, Wolfgang Schmidt, Michael Nauck, Eduard Montanya, Andrea Tannapfel, Juris Meier
AIMS: Incretin-based therapies have been associated with an increased risk of pancreatitis. Recently, various histological abnormalities have been reported in human pancreatic tissue from brain-dead organ donors who had been exposed to incretin-based drugs. In the present study we examined pancreatic tissue collected at surgery. METHODS: Human pancreatic tissue from 7 type 2-diabetic patients treated with incretin-based drugs (type 2-I), 6 diabetic patients without incretin treatment (type 2-NI), 11 patients without diabetes (no diabetes group) and 9 brain-dead organ donors (BDOD group) was examined...
December 2016: Diabetes, Obesity & Metabolism
https://www.readbyqxmd.com/read/27539223/bcl9l-expression-in-pancreatic-neoplasia-with-a-focus-on-spn-a-possible-explanation-for-the-enigma-of-the-benign-neoplasia
#18
Cora Hallas, Julia Phillipp, Lukas Domanowsky, Bettina Kah, Katharina Tiemann
BACKGROUND: Solid pseudopapillary neoplasms of the pancreas (SPN) are rare tumors affecting mainly women. They show an activating mutation in CTNNB1, the gene for β-catenin, and consequently an overactivation of the Wnt/β-catenin pathway. This signaling pathway is implied in the pathogenesis of various aggressive tumors, including pancreatic adenocarcinomas (PDAC). Despite this, SPN are characterized by an unusually benign clinical course. Attempts to explain this lack of malignancy have led to the discovery of an aberrant expression of the transcription factor FLI1 in SPN...
2016: BMC Cancer
https://www.readbyqxmd.com/read/27532258/analysis-of-founder-mutations-in-rare-tumors-associated-with-hereditary-breast-ovarian-cancer-reveals-a-novel-association-of-brca2-mutations-with-ampulla-of-vater-carcinomas
#19
Pedro Pinto, Ana Peixoto, Catarina Santos, Patrícia Rocha, Carla Pinto, Manuela Pinheiro, Luís Leça, Ana Teresa Martins, Verónica Ferreira, Carla Bartosch, Manuel R Teixeira
BRCA1 and BRCA2 mutations are responsible for hereditary breast and ovarian cancer, but they also confer an increased risk for the development of rarer cancers associated with this syndrome, namely, cancer of the pancreas, male breast, peritoneum, and fallopian tube. The objective of this work was to quantify the contribution of the founder mutations BRCA2 c.156_157insAlu and BRCA1 c.3331_3334del for cancer etiology in unselected hospital-based cohorts of Portuguese patients diagnosed with these rarer cancers, by using a strategy that included testing of archival tumor tissue...
2016: PloS One
https://www.readbyqxmd.com/read/27523981/loss-of-trefoil-factor-2-from-pancreatic-duct-glands-promotes-formation-of-intraductal-papillary-mucinous-neoplasms-in-mice
#20
Junpei Yamaguchi, Mari Mino-Kenudson, Andrew S Liss, Sanjib Chowdhury, Timothy C Wang, Carlos Fernández-Del Castillo, Keith D Lillemoe, Andrew L Warshaw, Sarah P Thayer
BACKGROUND & AIMS: Little is known about the origin of pancreatic intraductal papillary mucinous neoplasms (IPMN). Pancreatic duct glands (PDGs) are gland-like outpouches budding off the main pancreatic ducts that function as a progenitor niche for the ductal epithelium; they express gastric mucins and have characteristics of side-branch IPMNs. We investigated whether PDGs are a precursor compartment for IPMNs and the role of Trefoil factor family 2 (TFF2)-a protein expressed by PDGs and the gastric mucosa that are involved in epithelial repair and tumor suppression...
December 2016: Gastroenterology
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