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https://www.readbyqxmd.com/read/28902384/-multiple-endocrine-neoplasia
#1
Ludwig Schaaf, Friedhelm Raue
Multiple endocrine neoplasia type 1 and 2 are hereditary cancer syndromes. They are characterized by the occurrence of many benign and malignant tumor types, in MEN1 parathyroid tumors, pituitary tumors, and pancreas tumors, in MEN2 medullary thyroid carcinoma, pheochromocytoma, and parathyroid tumors. The autosomal dominant inherited tumor syndromes are caused by mutations in the MEN1 gene, a tumor suppressor gene, and mutations in the RET gene, an activated oncogene, in MEN2. The clinical expression of the different tumors can vary within and between families, with a good genotype-phenotype correlation in MEN2...
September 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28870964/incidence-and-oncological-implications-of-previously-undetected-tumor-multicentricity-following-pancreaticoduodenectomy-for-pancreatic-adenocarcinoma-in-patients-undergoing-salvage-pancreatectomy
#2
Andreas Andreou, Fritz Klein, Rosa B Schmuck, Daniela Lee, Marianne Sinn, Timm Denecke, Johann Pratschke, Marcus Bahra
BACKGROUND: The risk for multicentricity of pancreatic adenocarcinoma remains unclear and the question whether pancreaticoduodenectomy represents sufficient oncological treatment for patients with ductal adenocarcinoma of the head of the pancreas needs further investigation. PATIENTS AND METHODS: Clinicopathological data of patients who underwent pancreaticoduodenectomy for pancreatic adenocarcinoma between 2005 and 2015 were assessed and the incidence of tumor multicentricity among patients who required salvage pancreatectomy within 90 postoperative days was evaluated...
September 2017: Anticancer Research
https://www.readbyqxmd.com/read/28852316/serous-pancreatic-neoplasia-data-and-review
#3
Christoph F Dietrich, Yi Dong, Christian Jenssen, Valentina Ciaravino, Michael Hocke, Wen-Ping Wang, Eike Burmester, Kathleen Moeller, Nathan Ss Atkinson, Paola Capelli, Mirko D'Onofrio
AIM: To describe the imaging features of serous neoplasms of the pancreas using ultrasound, endoscopic ultrasound, computed tomography and magnetic resonance imaging. METHODS: This multicenter international collaboration enhances a literature review to date, reporting features of 287 histologically confirmed cases of serous pancreatic cystic neoplasms (SPNs). RESULTS: Female predominance is seen with most SPNs presenting asymptomatically in the 5(th) through 7(th) decade...
August 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28840303/pancreatic-cancer-screening
#4
Chris M Lindquist, Frank H Miller, Nancy A Hammond, Paul Nikolaidis
PURPOSE: This article reviews the current evidence surrounding pancreatic cancer screening. The current targets of screening include identification of early pancreatic cancer, as well as the two most important precursor lesions; intraductal papillary mucinous neoplasm and high grade pancreatic intraepithelial neoplasia. Given the relatively low incidence of pancreatic adenocarcinoma in the general population, patients with elevated risk based on family history or an underlying genetic syndrome are felt to be the most appropriate patients to undergo screening...
August 24, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28831506/-cystic-pancreatic-tumors-diagnostics-and-new-biomarkers
#5
REVIEW
A W Berger, T Seufferlein, A Kleger
Mortality due to pancreatic ductal adenocarcinoma (PDAC) will increase in the near future. The only curative treatment for PDAC is radical resection; however, even small carcinomas exhibit micrometastases leading to early relapse. Accordingly, detection of premalignant precursor lesions is important. In essence, PDAC develops from three precursor lesions: pancreatic intraepithelial lesions (PanIN), intraductal papillary-mucinous neoplasia (IPMN) and mucinous-cystic neoplasia (MCN). Together with serous cystic neoplasia (SCN) and solid pseudopapillary neoplasia (SPN), these cystic lesions constitute the most common cystic neoplasms in the pancreas...
August 22, 2017: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/28793277/o-2-18-f-fluoroethyl-l-tyrosine-18-f-fet-uptake-in-insulinoma-first-results-from-a-xenograft-mouse-model-and-from-human
#6
Alessio Imperiale, Frédéric Boisson, Guillaume Kreutter, Bernard Goichot, Izzie Jacques Namer, Philippe Bachellier, Patrice Laquerriere, Laurence Kessler, Patrice Marchand, David Brasse
INTRODUCTION: Herein we have evaluated the uptake of O-(2-(18)F-fluoroethyl)-l-tyrosine ((18)F-FET) in insulinoma in comparison with those of 6-(18)F-fluoro-3,4-dihydroxy-l-phenylalanine ((18)F-FDOPA) providing first data from both murine xenograft model and one patient with proved endogenous hyperinsulinemic hypoglycemia. METHODS: Dynamic (18)F-FET and carbidopa-assisted (18)F-FDOPA PET were performed on tumor-bearing nude mice after subcutaneous injection of RIN-m5F murine beta cells and on a 30-year-old man with type-1 multiple endocrine neoplasia and hyperinsulinemic hypoglycemia defined by a positive fasting test...
July 12, 2017: Nuclear Medicine and Biology
https://www.readbyqxmd.com/read/28767565/small-intestine-follicular-dendritic-cell-sarcoma-with-liver-metastasis-a-case-report
#7
Yun-Chen Chang, Ivy Yenwen Chau, Yi-Chen Yeh, Gar-Yang Chau
RATIONALE: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasia composed of spindle or oval cells with follicular dendritic cell differentiation, usually occurring in lymphoid tissue. In this report, we present a case of FDCS of the small intestine with liver metastasis. PATIENT CONCERNS: A 19-year-old female presented with recent onset of left upper abdominal pain. Abdominal computed tomography scan showed a large tumor mass in the liver lateral segment with compression to the pancreas upper part, and a smaller mass in the terminal ileum, respectively...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28752115/acinar-to-ductal-metaplasia-induced-by-transforming-growth-factor-beta-facilitates-kras-g12d-driven-pancreatic-tumorigenesis
#8
Nicolas Chuvin, David F Vincent, Roxane M Pommier, Lindsay B Alcaraz, Johann Gout, Cassandre Caligaris, Karam Yacoub, Victoire Cardot, Elodie Roger, Bastien Kaniewski, Sylvie Martel, Celia Cintas, Sophie Goddard-Léon, Amélie Colombe, Julie Valantin, Nicolas Gadot, Emilie Servoz, Jennifer Morton, Isabelle Goddard, Anne Couvelard, Vinciane Rebours, Julie Guillermet, Owen J Sansom, Isabelle Treilleux, Ulrich Valcourt, Stéphanie Sentis, Pierre Dubus, Laurent Bartholin
BACKGROUND & AIMS: Transforming growth factor beta (TGFβ) acts either as a tumor suppressor or as an oncogene, depending on the cellular context and time of activation. TGFβ activates the canonical SMAD pathway through its interaction with the serine/threonine kinase type I and II heterotetrameric receptors. Previous studies investigating TGFβ-mediated signaling in the pancreas relied either on loss-of-function approaches or on ligand overexpression, and its effects on acinar cells have so far remained elusive...
September 2017: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28736585/late-onset-asymptomatic-pancreatic-neuroendocrine-tumor-a-case-report-on-the-phenotypic-expansion-for-men1
#9
Charu Kaiwar, Sarah K Macklin, Jennifer M Gass, Jessica Jackson, Eric W Klee, Stephanie L Hines, John A Stauffer, Paldeep S Atwal
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary cancer syndrome associated with several endocrine as well as non-endocrine tumors and is caused by mutations in the MEN1 gene. Primary hyperparathyroidism affects the majority of MEN1 individuals by age 50 years. Additionally, MEN1 mutations trigger familial isolated hyperparathyroidism. We describe a seemingly unaffected 76-year-old female who presented to our Genetics Clinic with a family history of primary hyperparathyroidism and the identification of a pathogenic MEN1 variant...
2017: Hereditary Cancer in Clinical Practice
https://www.readbyqxmd.com/read/28736522/gliotoxin-targets-nuclear-notch2-in-human-solid-tumor-derived-cell-lines-in-vitro-and-inhibits-melanoma-growth-in-xenograft-mouse-model
#10
Rainer Hubmann, Wolfgang Sieghart, Susanne Schnabl, Mohammad Araghi, Martin Hilgarth, Marlies Reiter, Dita Demirtas, Peter Valent, Christoph Zielinski, Ulrich Jäger, Medhat Shehata
Deregulation of NOTCH2 signaling is implicated in a wide variety of human neoplasias. The current concept of targeting NOTCH is based on using gamma secretase inhibitors (GSI) to regulate the release of the active NOTCH intracellular domain. However, the clinical outcome of GSI remains unsatisfactory. Therefore we analyzed human solid tumor derived cell lines for their nuclear NOTCH activity and evaluated the therapeutic potential of the NOTCH2 transactivation inhibitor gliotoxin in comparison to the representative GSI DAPT...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28733926/perioperative-management-of-endocrine-insufficiency-after-total-pancreatectomy-for-neoplasia
#11
REVIEW
Ajay V Maker, Raashid Sheikh, Vinita Bhagia
PURPOSE: Indications for total pancreatectomy (TP) have increased, including for diffuse main duct intrapapillary mucinous neoplasms of the pancreas and malignancy; therefore, the need persists for surgeons to develop appropriate endocrine post-operative management strategies. The brittle diabetes after TP differs from type 1/2 diabetes in that patients have absolute deficiency of insulin and functional glucagon. This makes glucose management challenging, complicates recovery, and predisposes to hospital readmissions...
July 21, 2017: Langenbeck's Archives of Surgery
https://www.readbyqxmd.com/read/28679961/islet-inflammation-and-ductal-proliferation-may-be-linked-to-increased-pancreatitis-risk-in-type-2-diabetes
#12
Belinda Schludi, Abu Saleh Md Moin, Chiara Montemurro, Tatyana Gurlo, Aleksey V Matveyenko, David Kirakossian, David W Dawson, Sarah M Dry, Peter C Butler, Alexandra E Butler
Pancreatitis is more frequent in type 2 diabetes mellitus (T2DM), although the underlying cause is unknown. We tested the hypothesis that ongoing β cell stress and apoptosis in T2DM induces ductal tree proliferation, particularly the pancreatic duct gland (PDG) compartment, and thus potentially obstructs exocrine outflow, a well-established cause of pancreatitis. PDG replication was increased 2-fold in human pancreas from individuals with T2DM, and was associated with increased pancreatic intraepithelial neoplasia (PanIN), lesions associated with pancreatic inflammation and with the potential to obstruct pancreatic outflow...
July 6, 2017: JCI Insight
https://www.readbyqxmd.com/read/28665730/cutaneous-leiomyosarcoma-with-multiple-visceral-metastases-in-a-domestic-pigeon
#13
Tomasz Stenzel, Michał Gesek, Katarzyna Paździor-Czapula, Iwona Otrocka-Domagała, Andrzej Koncicki
A clinical examination of a 4-yr-old fantail pigeon revealed a large skin tumor in the area of the left wing. The tumor had solid consistency and a single cavity filled with fluid. During necropsy, multiple tumors of varied size and diameter were found in the liver, spleen, pancreas, and kidneys. A histopathological examination of the skin tumor revealed extensive multifocal neoplasia with a tendency to infiltrate, composed of polygonal, round, oval, and elongated cells forming a solid system with cellular bands and obliteration of intercellular boundaries...
June 2017: Avian Diseases
https://www.readbyqxmd.com/read/28663159/synchronous-solid-pseudopapillary-tumor-and-insulinoma-in-an-adolescent-men1-patient-presenting-with-diagnostic-dilemmas
#14
Ahmet Uçar, Banu Özgüven, Muharrem Battal, Felda Alpaslan, Evrim Özmen, Aylin Yetim, Yasin Yılmaz
Multiple endocrine neoplasia (MEN1) is a rare autosomal dominant disorder characterized by primary hyperparathyroidism, enteropancreatic neuroendocrine tumors, and anterior pituitary adenomas. A 16-year-old male presented to the emergency outpatient clinic with tonic convulsions. Physical examination in the postconvulsive period was unremarkable and revealed a muscular, postpubertal adolescent. Biochemical tests at admission were consistent with hyperinsulinemic hypoglycemia and remarkable for elevated levels of liver transaminases and creatine kinase...
June 30, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28650600/clear-cell-neuroendocrine-tumor-of-the-pancreas-in-von-hippel-lindau-disease-a-case-report-and-literature-review
#15
Chang Gok Woo, Seo-Youn Choi, Jeong Ja Kwak, Susie Chin, Hee Kyung Kim
Clear cell neuroendocrine tumor (NET) of the pancreas is found in von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia type I (MEN I), and sporadic form. Clear cell NETs are often misdiagnosed as metastatic renal cell carcinoma. A 47-year-old woman with VHL was found to have a mass in the pancreatic tail and two masses in the right kidney with two cysts. A distal pancreatectomy and right radical nephrectomy were performed. The pancreatic lesion was a well-circumscribed, golden-yellow solid mass, which was lobulated by septal fibrosis...
May 1, 2017: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28639725/targeting-nad-p-h-quinone-oxidoreductase-nqo1-in-pancreatic-cancer
#16
Anne M Lewis, Matthew Ough, Juan Du, Ming-Sound Tsao, Larry W Oberley, Joseph J Cullen
Quinone oxidoreductase (NQO1) functions as an important part of cellular antioxidant defense by detoxifying quinones, thus preventing the formation of reactive oxygen species. The aims of our study were to determine if NQO1 is elevated in pancreatic cancer specimens and pancreatic cancer cell lines and if so, would compounds previously demonstrated to redox cycle with NQO1 be effective in killing pancreatic cancer cells. Immunohistochemistry of resected pancreatic specimens demonstrated an increased immunoreactivity for NQO1 in pancreatic cancer and pancreatic intraepithelial neoplasia (PanIN) specimens versus normal human pancreas...
July 2017: Molecular Carcinogenesis
https://www.readbyqxmd.com/read/28639695/glycogen-synthase-kinase-3%C3%AE-ablation-limits-pancreatitis-induced-acinar-to-ductal-metaplasia
#17
Li Ding, Geou-Yarh Liou, Daniel M Schmitt, Peter Storz, Jin-San Zhang, Daniel D Billadeau
Acinar-to-ductal metaplasia (ADM) is a reversible epithelial transdifferentiation process that occurs in the pancreas in response to acute inflammation. ADM can rapidly progress towards pre-malignant pancreatic intraepithelial neoplasia (PanIN) lesions in the presence of mutant KRas and ultimately pancreatic adenocarcinoma (PDAC). In the present work, we elucidate the role and related mechanism of glycogen synthase kinase-3beta (GSK-3β) in ADM development using in vitro 3D cultures and genetically engineered mouse models...
September 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28617892/intestinal-heterotopic-pancreas-involved-by-simultaneous-panin-2-lesion-and-endocrine-microadenoma-a-unique-case
#18
Remus Cornea, Sorina Taban, Cristian Suciu, Codruta Lazureanu, Alis Dema
We hereby present the case of a 58-year-old male who underwent a total gastrectomy for gastric neoplasm. During the surgery, a tumor mass in the jejunum was identified, considered as metastasis, and resected. The histopathological examination of the jejunal lesion showed ectopic pancreas. In this area, two pathological distinct lesions were identified, one histologically compatible with pancreatic intraepithelial neoplasia (PanIN) type 2 lesion and the other with morphologic criteria for endocrine microadenoma...
June 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28589571/laparoscopic-total-pancreatectomy-for-multiple-endocrine-neoplasia-type-1-syndrome-associated-multifocal-non-functioning-pancreatic-neuroendocrine-tumor-a-case-report
#19
Palanisamy Senthinathan, Samrat Vijaykumar Jankar, Sandeep C Sabnis, Vivek Kaje, Sivakumar Srivatsan Gurumurthy, Natesan Anand Vijai, Vaiyapurigounder Palanisamy Nalankilli, Palanivelu Praveen Raj, Ramakrishanan Parthasarathi, Subbiah Rajapandian, Chinnusamy Palanivelu
Pancreatic neuroendocrine tumors are rare, accounting for less than 3% of all pancreatic tumors. Although laparoscopic pancreas-preserving surgery for managing sporadic pancreatic neuroendocrine tumors has been described in the literature, laparoscopic total pancreatectomy has rarely been reported. We present a 30-year-old man who was incidentally diagnosed with multiple endocrine neoplasia type 1 syndrome with parathyroid hyperplasia and a non-functioning pancreatic neuroendocrine tumor. He underwent laparoscopic total pancreatectomy with splenectomy...
June 6, 2017: Asian Journal of Endoscopic Surgery
https://www.readbyqxmd.com/read/28579586/a-case-of-pancreatic-intraepithelial-neoplasia-3-with-autoimmune-pancreatitis
#20
Yasuharu Kakizaki, Naohiko Makino, Yoshiaki Ando, Akiko Matsuda, Tetsuya Ishizawa, Yoshihiko Saito, Aya Suto, Mitsunori Yamakawa, Wataru Kimura, Yoshiyuki Ueno
We report a case of pancreatic intraepithelial neoplasia-3 (PanIN-3) with autoimmune pancreatitis (AIP). The patient, a 75-year-old man, had been diagnosed to have AIP with stenosis of the main pancreatic duct. After six years, computed tomography demonstrated dilatation of the main pancreatic duct in the mid-pancreas. Although we could not confirm the presence of any pancreatic tumor on the basis of imaging modalities alone, cytological examination of the pancreatic juice obtained by endoscopic retrograde pancreatography revealed atypical cells...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
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