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pancreas neoplasia

Giulio Innamorati, Thomas M Wilkie, Havish S Kantheti, Maria Teresa Valenti, Luca Dalle Carbonare, Luca Giacomello, Marco Parenti, Davide Melisi, Claudio Bassi
BACKGROUND: Mutations activating the α subunit of heterotrimeric Gs protein are associated with a number of highly specific pathological molecular phenotypes. One of the best characterized is the McCune Albright syndrome. The disease presents with an increased incidence of neoplasias in specific tissues. MAIN BODY: A similar repertoire of neoplasms can develop whether mutations occur spontaneously in somatic tissues during fetal development or after birth. Glands are the most "permissive" tissues, recently found to include the entire gastrointestinal tract...
March 15, 2018: BMC Cancer
Austin T Kerns, Kelsey A Brakel, Christopher Premanandan, Ashlie Saffire, Sarah A Moore
Case summary: A 9-year-old spayed female domestic shorthair cat with clinical signs suggestive of chronic recurrent otitis media and recent seizures was presented with multifocal nervous system disease, including bilateral central and/or peripheral vestibular, cerebellar and forebrain deficits. Prior to presentation, there was inadequate improvement after 6 weeks of treatment for bilateral middle ear effusion from which a highly susceptible Staphylococcus species was cultured. This was followed by the development of seizures...
January 2018: JFMS Open Reports
Vaishali I Parekh, Sita D Modali, James Welch, William Simonds, Lee Scott Weinstein, Electron Kebebew, Sunita K Agarwal
Extract: Pancreatic neuroendocrine tumors (PNETs/pNETs/p-NETs/PanNETs) are rare endocrine neoplasms that can be either functioning tumors that secrete hormones characteristic of their endocrine cell of origin, or nonfunctioning tumors. The most common functioning PNETs are the insulin-secreting b-cell tumors (insulinomas) that are mainly sporadic, but may also occur in 10% of patients with the hereditary tumor syndrome multiple endocrine neoplasia type 1 (MEN1) (OMIM ID: 131100). Patients with the MEN1 syndrome carry a heterozygous germline inactivating mutation in the MEN1 tumor suppressor gene and specific somatic loss of the normal MEN1 allele, leading to endocrine tumors mainly of the parathyroids, pituitary and pancreas (PNETs)...
February 21, 2018: Endocrine-related Cancer
R Feldmann, S Wahl, A Steiner
Glucagonoma is an extremely rare tumor of the pancreatic alpha-cells. Its estimated annual incidence is 1 case per 20 million individuals. Necrolytic migratory erythema (NME) is the hallmark clinical sign. We report a patient with normoglycemic glucagonoma and NME. A 44-year-old male patient presented with a 4-year history of unexplained exanthema and unintentional weight loss. Skin examination revealed a mildly pruritic rash on the trunc, the extremities and the face (fig 1). One found erythematous polycyclic migratory lesions with scaling and crusting margins and central resolution...
February 8, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Stefania Innaurato, Gemma V Brierley, Valeria Grasso, Arianna Massimi, Rossella Gaudino, Sara Sileno, Sergio Bernardini, Robert Semple, Fabrizio Barbetti
AIM: Hypoglycemia in childhood is very rare and can be caused by genetic mutations or insulin-secreting neoplasms. Postprandial hypoglycemia has previously been associated with insulin receptor (INSR) gene mutations. We aimed to identify the cause of postprandial hypoglycemia in a 10-year-old boy. SUBJECTS: We studied the symptomatic proband and his apparently asymptomatic mother and elder brother. All of them were lean. METHODS: Metabolic screening of the proband included a 5-hour oral glucose tolerance test (OGTT), angio-magnetic resonance imaging, and18 F-dihydroxyphenylalanine positron emission tomography/computed tomography imaging of the pancreas...
February 7, 2018: Pediatric Diabetes
Michael J Bartel, Ruchir Puri, Bhaumik Brahmbhatt, Wei-Chung Chen, Daniel Kim, Carlos Roberto Simons-Linares, John A Stauffer, Mauricia A Buchanan, Steven P Bowers, Timothy A Woodward, Michael B Wallace, Massimo Raimondo, Horacio J Asbun
BACKGROUND: Sporadic nonampullary duodenal neoplasms (SNADN) can have malignant potential for which endoscopic and surgical resections are offered. We report combined gastroenterologic and surgical experience for treatment of SNADN, including endoscopic mucosal resection (EMR) and pancreas-preserving partial duodenectomy (PPPD). METHODS: We retrospectively reviewed 121 consecutive patients, who underwent 30 PPPDs and 91 EMRs for mucosal and submucosal SNADN. Decision to undergo EMR or surgical resection was based on expert endoscopist and surgeon discretion including multidisciplinary tumor board review...
February 1, 2018: Surgical Endoscopy
Justyna Zińczuk, Konrad Zaręba, Katarzyna Guzińska-Ustymowicz, Bogusław Kędra, Andrzej Kemona, Anna Pryczynicz
BACKGROUND: Deregulation of cell cycle takes place during the development of many cancers as well as pancreatic ductal adenocarcinoma (PDA), which develops from precursor lesions, most frequently including pancreatic intraepithelial neoplasia (PanIN). AIMS: The aim of this study was to evaluate and compare the expression of p16, p21, and p53 proteins taking part in the regulation of the cell cycle in normal pancreatic ducts and pancreatic intraepithelial neoplasia at its various advancing stages...
February 1, 2018: Irish Journal of Medical Science
Volker Fendrich, Frederike Jendryschek, Saskia Beeck, Max Albers, Matthias Lauth, Farzad Esni, Kristin Heeger, Janina Dengler, Emily P Slater, Julia P N Holler, Aninja Baier, Detlef K Bartsch, Jens Waldmann
Pancreatic cancer (PDAC) is one of the most dismal of human malignancies. Inhibiting or delaying the progression of precursor lesions of PDAC, pancreatic intraepthial neoplasia (PanINs), to invasive cancer, would be a major step. In the present study, we used a transgenic murine model of pancreatic cancer to evaluate the impact of a conditional knockout of the transcription factor Snail1, a major factor in epithelial-to-mesenchymal transition, on acinar-to-ductal formation and on PanIN progression. By interbreeding conditional LsL-Snail floxf/wt ; LsL-Kras G12D and Pdx1-Cre strains, we obtained LsL-Kras G12D ;Pdx1-Cre(KP) mice, Snail1 heterozygous knockout LsL-Kras G12D ; LsL-Snail flox/- ;Pdx1-Cre(KPShet) mice or Snail1 homozygous knockout LsL-Kras G12D ;LsL-Snail flox/flox ;Pdx1-Cre(KPS) mice...
January 25, 2018: Oncogene
Jeffrey A Norton, Deshka S Foster, Leslie H Blumgart, George A Poultsides, Brendan C Visser, Douglas L Fraker, H Richard Alexander, Robert T Jensen
Importance: Zollinger-Ellison syndrome (ZES) is a life-threatening disease caused by a malignant tumor that secretes gastrin (gastrinoma). Gastrinomas typically occur in the pancreas or the duodenum. Objective: To describe the incidence and prognosis of very unusual gastrinomas originating in the hepatobiliary tract. Design, Setting, and Participants: This study included 223 consecutive patients at the National Institutes of Health and Stanford University Hospital who were enrolled in a prospective protocol to treat ZES using proton pump inhibitors to control acid hypersecretion and surgical resection to ameliorate the tumoral process...
January 24, 2018: JAMA Surgery
Kayla A Quirin, Jason J Kwon, Arafat Alioufi, Tricia Factora, Constance J Temm, Max Jacobsen, George E Sandusky, Kim Shontz, Louis G Chicoine, K Reed Clark, Joshua T Mendell, Murray Korc, Janaiah Kota
Recombinant adeno-associated virus (rAAV)-mediated gene delivery shows promise to transduce the pancreas, but safety/efficacy in a neoplastic context is not well established. To identify an ideal AAV serotype, route, and vector dose and assess safety, we have investigated the use of three AAV serotypes (6, 8, and 9) expressing GFP in a self-complementary (sc) AAV vector under an EF1α promoter (scAAV.GFP) following systemic or retrograde pancreatic intraductal delivery. Systemic delivery of scAAV9.GFP transduced the pancreas with high efficiency, but gene expression did not exceed >45% with the highest dose, 5 × 1012 viral genomes (vg)...
March 16, 2018: Molecular Therapy. Methods & Clinical Development
Azita Monazzam, Joey Lau, Irina Velikyan, Su-Chen Li, Masoud Razmara, Ulrika Rosenström, Olof Eriksson, Britt Skogseid
Multiple endocrine neoplasia type 1 (MEN1) is an endocrine tumor syndrome caused by heterozygous mutations in the MEN1 tumor suppressor gene. The MEN1 pancreas of the adolescent gene carrier frequently contain diffusely spread pre-neoplasias and microadenomas, progressing to macroscopic and potentially malignant pancreatic neuroendocrine tumors (P-NET), which represents the major death cause in MEN1. The unveiling of the molecular mechanism of P-NET which is not currently understood fully to allow the optimization of diagnostics and treatment...
January 15, 2018: Scientific Reports
Thais Ligiero Braga, Ralph Santos-Oliveira
Generally, pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma) is classified as a rare tumor, and may occur sporadically or be associated in families or with multiple endocrine neoplasia type 1 (NEM 1). It grows slowly, reaching large dimensions at the time of diagnosis and the symptomatology is fundamentally due to the mass effect, causing either non-specific abdominal pain or symptoms suggestive of obstruction of the pancreatic or biliary duct. Therefore, when detected, they are usually malignant, with metastases mainly in the liver...
January 11, 2018: Diseases (Basel)
T Araki, L M Sholl, H Hatabu, M Nishino
AIM: To investigate the clinical and image features of thymic neuroendocrine tumours (NETs), and characterise the radiological patterns of recurrence and metastasis on serial imaging studies. MATERIALS AND METHODS: The study included 14 patients (11 males) with a histopathological diagnosis of thymic NETs (one typical carcinoid, eight atypical carcinoid, and five large cell neuroendocrine carcinoma). Preoperative images were assessed for features of primary tumours...
January 5, 2018: Clinical Radiology
Masaya Suenaga, Jun Yu, Koji Shindo, Koji Tamura, Jose Alejandro N Almario, Christopher M Zaykoski, P Dane Witmer, Shah Fesharakizadeh, Michael Borges, Anne Marie Lennon, Eun J Shin, Marcia I Canto, Michael G Goggins
Purpose: The measurement of mutations in pancreatic juice samples collected from the duodenum during endoscopic ultrasound (EUS) may improve the diagnostic evaluation of patients undergoing pancreatic surveillance. Our aim was to evaluate the accuracy of using pancreatic juice mutation concentrations to predict the presence and histologic grade of neoplasia in the pancreas.Experimental Design: Digital next-generation sequencing (NGS) of pancreatic juice DNA using a targeted 12-gene panel was performed on 67 patients undergoing pancreatic evaluation during EUS including patients with pancreatic ductal adenocarcinoma, patients who subsequently underwent pancreatic resection for precursor lesions, patients undergoing surveillance for their familial/inherited susceptibility to pancreatic cancer, and normal pancreas disease controls...
January 4, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Ahmad Abu Turab Naqvi, Gulam Mustafa Hasan, Md Imtaiyaz Hassan
Pancreatic cancer (PC) is the seventh most common cause of cancer-related deaths worldwide that kills more than 300,000 people every year. Prognosis of PC is very poor with a five-year survival rate about 5%. The most common and highly observed type of PC is pancreatic ductal adenocarcinoma (PDAC). It is preceded by the progression of precursor lesions such as Pancreatic Intraepithelial Neoplasia (PanIN), Intraductal Papillary Neoplasm (IPMN) and Mucinous Cystic Neoplasm (MCN). PanIN is the most common among these premalignant lesions...
March 2018: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
Ramen Sakhi, Ameer Hamza, Muhammad Siddique Khurram, Warda Ibrar, Paul Mazzara
Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UC-OGC) is a rare and poorly described pancreatic malignancy. It is comprised of mononuclear, pleomorphic, and undifferentiated cells as well as osteoclast-like giant cells (OGC's). It constitutes less than 1% of pancreatic non-endocrine neoplasia and is twice as likely to occur in females as in males. Its histopathologic properties remain poorly understood. It is suspected that UC-OGC is of epithelial origin that can then transition to mesenchymal elements...
October 2017: Autopsy & Case Reports
Henricus J M Handgraaf, Leonora S F Boogerd, Shirin Shahbazi Feshtali, Arantza Fariña Sarasqueta, Marieke Snel, Rutger-Jan Swijnenburg, Alexander L Vahrmeijer, Bert A Bonsing, J Sven D Mieog
Multiple endocrine neoplasia type 1 syndrome can feature pancreatic neuroendocrine lesions that have the potential to degenerate into malignancies (pancreatic neuroendocrine tumors [PNETs]). Resection is required in selected cases and aims to cure patients and to prevent metastasis. Preoperative imaging is important to assess the number, size, and location of PNETs. However, sensitivity of preoperative imaging modalities to detect small lesions can be rather disappointing. This makes intraoperative reassessment of the pancreas crucial...
January 2018: Pancreas
P K Wright, D A Shelton, M R Holbrook, S A Thiryayi, N Narine, D Slater, D N Rana
OBJECTIVE: To compare endoscopic ultrasound (EUS)-FNAC diagnosis of pancreatic lesions with patient outcome based upon the Papanicolaou Society of Cytopathology pancreaticobiliary terminology classification scheme diagnostic categories: Panc 1 (non-diagnostic); Panc 2 (negative for malignancy/neoplasia); Panc 3 (atypical); Panc 4B (neoplastic, benign); Panc 4O (neoplastic, other); Panc 5 (suspicious of malignancy); and Panc 6 (positive/malignant). METHODS: All EUS-FNA pancreas specimens taken at Manchester Royal Infirmary in 2015 were prospectively classified according to the above scheme at the time of cytology reporting and data recorded prospectively...
February 2018: Cytopathology: Official Journal of the British Society for Clinical Cytology
Meredith E Pittman, Rema Rao, Ralph H Hruban
CONTEXT: - Invasive pancreatic ductal adenocarcinoma has a greater than 90% mortality rate at 5 years. Understanding noninvasive, curable precursor lesions gives us the best hope for reducing mortality from pancreatic ductal adenocarcinoma. The 3 pancreatic precursor lesions that have been well studied include intraductal papillary mucinous neoplasm, mucinous cystic neoplasm, and pancreatic intraepithelial neoplasia. OBJECTIVE: - To give an update on the latest clinical, molecular, and pathologic advances in intraductal papillary mucinous neoplasm, mucinous cystic neoplasm, and pancreatic intraepithelial neoplasia for the general surgical pathologist...
December 2017: Archives of Pathology & Laboratory Medicine
Michael Lause, Alisha Kamboj, Esteban Fernandez Faith
The skin serves as a window for clinicians to understand, diagnose, and monitor endocrine disease. Dermatologic manifestations of endocrinopathies contribute significantly to an individual's health and quality of life. In this review, we outline various disorders of the hypothalamic-pituitary axis, thyroid gland, pancreas, adrenal gland, and androgen axis as well as hereditary endocrine syndromes. In acromegaly, glycosaminoglycan deposition contributes to a thickening of skin and soft tissue, which manifests as coarsening and enlargement of facial and acral structures...
October 2017: Translational pediatrics
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