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pancreas neoplasia

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
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
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
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
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
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
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
S Ueberberg, H Jütte, W Uhl, W E Schmidt, M A Nauck, E Montanya, A Tannapfel, J J Meier
AIMS/HYPOTHESIS: 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 that had been exposed to incretin-based drugs. In the present study we examined pancreatic tissue collected at surgery. METHODS: Human pancreatic tissue from seven type 2-diabetic patients treated with incretin-based drugs (type 2-I), six diabetic patients without incretin treatment (type 2-NI), eleven patients without diabetes (no diabetes) and nine brain-dead organ donors (BDOD) was examined...
August 22, 2016: Diabetes, Obesity & Metabolism
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
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
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 progenitor niche for the ductal epithelium; they express gastric mucins and have characteristics of side-branch IPMN. We investigated whether PDGs are a precursor compartment for IPMN 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...
August 11, 2016: Gastroenterology
Barbara Grünwald, Veronika Harant, Susanne Schaten, Monika Frühschütz, Ria Spallek, Bastian Höchst, Katharina Stutzer, Sonja Berchtold, Mert Erkan, Olga Prokopchuk, Marc Martignoni, Irene Esposito, Mathias Heikenwalder, Aayush Gupta, Jens Siveke, Paul Saftig, Percy Knolle, Dirk Wohlleber, Achim Krüger
BACKGROUND & AIMS: Pancreatic ductal adenocarcinoma (PDAC) metastasizes to liver at early stages, making this disease highly lethal. Tissue inhibitor of metalloproteinases-1 (TIMP1) creates a metastasis-susceptible environment in the liver. We investigated the role of TIMP1 and its receptor CD63 in metastasis of early-stage pancreatic tumors using mice and human cell lines and tissue samples. METHODS: We obtained liver and plasma samples from patients in Germany with chronic pancreatitis, pancreatic intra-epithelial neoplasia, or PDAC, as well as hepatic stellate cells (HSCs)...
August 6, 2016: Gastroenterology
Abid Ali Mirza, Esha Pai, Kodaganur Gopinath Srinivas, Shankarappa Amarendra, K S Gopinath
With the increasing use of 18F-Fluro-Deoxyglucose (FDG) Positron Emission Tomography (PET) the number of thyroid incidentalomas is on the rise. Focal thyroid incidentalomas identified by FDG-PET have been reported to have a high incidence of malignancy. Neuroendocrine tumours of the thyroid are rare entities. The most common neuroendocrine tumour of the thyroid is medullary carcinoma. A thyroid nodule in a patient with a known neuroendocrine tumour must be differentiated from a primary medullary carcinoma which can present as a diagnostic challenge to the clinician...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
Andreia V Pinho, Amanda Mawson, Anthony Gill, Mehreen Arshi, Max Warmerdam, Marc Giry-Laterriere, Nils Eling, Triyana Lie, Evelyne Kuster, Simone Camargo, Andrew V Biankin, Jianmin Wu, Ilse Rooman
Metabolic reprogramming is a feature of neoplasia and tumor growth. Sirtuin 1 (SIRT1) is a lysine deacetylase of multiple targets including metabolic regulators such as p53. SIRT1 regulates metaplasia in the pancreas. Nevertheless, it is unclear if SIRT1 affects the development of neoplastic lesions and whether metabolic gene expression is altered.To assess neoplastic lesion development, mice with a pancreas-specific loss of Sirt1 (Pdx1-Cre;Sirt1-lox) were bred into a KrasG12D mutant background (KC) that predisposes to the development of pancreatic intra-epithelial neoplasia (PanIN) and ductal adenocarcinoma (PDAC)...
August 2, 2016: Oncotarget
Toni Raffoul, Alexandre Balaphas, Raphael P H Meier, Catrina Hansen, Giacomo Puppa, Jean-Louis Frossard, Philippe Morel, Leo H Bühler
The detection rate of cystic lesions of the pancreas has increased following the widespread use of high-resolution imaging technologies. CT-scan, MRI and echo-endoscopy are diagnostic modalities. Pseudocyst is the most common lesion. It is benign and can be managed with endoscopic treatment. Mucinous cystic neoplasia and Intraductal Papillary Mucinous Neoplasia (IPMN) carry a risk for malignant transformation. The surgical treatment of these lesions has to be discussed by a multidisciplinary board. Serous cystic neoplasia and pseudopapillar and solid neoplasia are two rare types of lesion...
June 15, 2016: Revue Médicale Suisse
Javier A Cienfuegos, Fernando Rotellar, Joseba Salguero, Alberto Benito, José Luis Solórzano, Bruno Sangro
BACKGROUND: Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015. METHODS: A systematic review of the MedLine and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults...
August 2016: Revista Española de Enfermedades Digestivas
Guilherme Naccache Namur, Thiago Costa Ribeiro, Marcelo M Souto, Estela Regina Ramos Figueira, Telesforo Bacchella, Ricardo Jureidini
BACKGROUND: Solid pseudopapillary pancreatic neoplasia is usually a large well-circumscribed pancreatic mass, with cystic and solid areas more frequently found in young women. It is a benign pancreatic neoplasia in most cases, therefore minimally invasive surgery could be an interesting approach. AIM: Evaluate the results of minimally invasive surgery for this neoplasia. METHODS: Patients with this tumor who underwent minimally invasive pancreatectomies between 2009 and 2015 in a single institution, were analyzed regarding demographic, clinical-pathological futures, post-operative morbidity and disease-free survival...
April 2016: Arquivos Brasileiros de Cirurgia Digestiva: ABCD, Brazilian Archives of Digestive Surgery
Jorge Roberto Marcante Carlotto, Franz Robert Apodaca Torrez, Adriano Miziara Gonzalez, Marcelo Moura Linhares, Tarcisio Triviño, Benedito Herani-Filho, Alberto Goldenberg, Gaspar de Jesus Lopes-Filho, Edson José Lobo
BACKGROUND: The solid pseudopapillary neoplasm is a rare tumor of the pancreas. However, it´s etiology still maintain discussions. AIM: To analyze it´s clinical data, diagnosis and treatment. METHODS: A retrospective study of medical records of all patients treated from January 1997 until July 2015. RESULTS: Were identified 17 cases. Most patients were women (94.11%) and the average age was 32.88 years. The main complaint was abdominal mass (47...
April 2016: Arquivos Brasileiros de Cirurgia Digestiva: ABCD, Brazilian Archives of Digestive Surgery
Zhongxiuzi Gao, Li Zhang, Wenting Xie, Siqi Wang, Xiaorui Bao, Yuli Guo, Houjian Zhang, Qingzhong Hu, Yi Chen, Zeen Wang, Maoqiang Xue, Guanghui Jin
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited syndrome characterized by multiple tumors in the parathyroid glands, endocrine pancreas and anterior pituitary. Recent clinical studies have revealed a strong association between MEN1 syndrome and the risk of developing diabetes mellitus; however, the underlying mechanisms remain unknown. In this study, heterozygous Men1 knockout (Men1(+/-)) mice were used as MEN1 models to investigate MEN1-associated glucose metabolic phenotypes and mechanisms...
September 2016: Journal of Endocrinology
Jun Yu, Yoshihiko Sadakari, Koji Shindo, Masaya Suenaga, Aaron Brant, Jose Alejandro Navarro Almario, Michael Borges, Thomas Barkley, Shahriar Fesharakizadeh, Madeline Ford, Ralph H Hruban, Eun Ji Shin, Anne Marie Lennon, Marcia Irene Canto, Michael Goggins
OBJECTIVE: Secretin-stimulated pancreatic juice contains DNA shed from cells lining the pancreatic ducts. Genetic analysis of this fluid may form a test to detect pancreatic ductal neoplasia. DESIGN: We employed digital next-generation sequencing ('digital NGS') to detect low-abundance mutations in secretin-stimulated juice samples collected from the duodenum of subjects enrolled in Cancer of the Pancreas Screening studies at Johns Hopkins Hospital. For each juice sample, digital NGS necessitated 96 NGS reactions sequencing nine genes...
July 18, 2016: Gut
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