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    2235 results match your criteria Wermer Syndrome MEN Type 1

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    Complicated Case Presentation: Management of Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1.
    Pancreas 2017 Mar;46(3):416-426
    From the *Department of Internal Medicine, †Division of Hematology/Oncology, Department of Medicine, ‡Division of Endocrinology and Metabolism, Department of Medicine, and §Section of Surgical Oncology/Hepatopancreaticobiliary Surgery, Division of General Surgery, Department of Surgery, University of California, San Francisco, San Francisco, CA.
    Multiple endocrine neoplasia type 1 (MEN1) is an inherited predisposition to tumors of the parathyroid glands, anterior pituitary, and pancreatic islet cells. In this review, we discuss the clinical case of a 45-year-old woman with MEN1 that was presented at the 2015 North American Neuroendocrine Tumor Society Symposium. In our review of this patient's complicated clinical course and subsequent operative management, we highlight controversies in the diagnosis and management of pancreatic neuroendocrine tumors in MEN1. Read More

    Genetics of multiple endocrine neoplasia type 1 syndrome: what's new and what's old.
    F1000Res 2017 24;6. Epub 2017 Jan 24.
    EndOsMet Unit, Villa Donatello, Piazzale Donatello 2, Florence 50100, Italy; Hercolani Clinical Center, Via D'Azeglio 46, Bologna 40136, Italy.
    Despite its identification in 1997, the functions of the MEN1 gene-the main gene underlying multiple endocrine neoplasia type 1 syndrome-are not yet fully understood. In addition, unlike the RET-MEN2 causative gene-no hot-spot mutational areas or genotype-phenotype correlations have been identified. More than 1,300 MEN1 gene mutations have been reported and are mostly "private" (family specific). Read More

    Limited Value of Ga-68-DOTATOC-PET-CT in Routine Screening of Patients with Multiple Endocrine Neoplasia Type 1.
    World J Surg 2017 Jan 30. Epub 2017 Jan 30.
    Department of Visceral, Thoracic and Vascular Surgery, University Hospital of Giessen and Marburg, Philipps University of Marburg, Marburg, Germany.
    Background: Routine screening is recommended for patients with multiple endocrine neoplasia type 1 (MEN1) to enable early detection and treatment of associated neuroendocrine neoplasms (NEN). Gallium(68)-DOTATOC-Positron emission tomography combined with computed tomography (Ga-68-DOTATOC-PET-CT) is a very sensitive and specific imaging technique for the detection of sporadic neuroendocrine tumors. The present study evaluated the value of Ga-68-DOTATOC-PET-CT in routine screening of patients with MEN1. Read More

    Type 2 gastric neuroendocrine tumor: report of one case.
    Transl Gastroenterol Hepatol 2016 30;1:88. Epub 2016 Nov 30.
    Department of Integrative Oncology, China-Japan Friendship Hospital, Beijing 100029, China.
    In this article we reported a female patient with type 2 gastric neuroendocrine tumor (NET). The patient developed upper abdominal pain, acid reflux, heartburn, nausea, and vomiting without obvious cause 16 years ago. Later, a tumor was found in her stomach. Read More

    Metastatic multiple endocrine neoplasia type 1: report of one case.
    Transl Gastroenterol Hepatol 2016 23;1:73. Epub 2016 Sep 23.
    Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
    A 46-year-old Chinese woman was admitted to our hospital because of presence of space-occupying lesions in the liver for 2 months in April, 2015. She had a family history of multiple endocrine neoplasia type 1 (MEN1) and physical examination is unremarkable. Previously, she has performed surgery for primary pituitary tumor in 2002 and radiosurgery for its recurrence. Read More

    [Multiple Endocrine Neoplasia Type 1(MEN1)Presenting with Hypoglycemic Attacks - A Case Report].
    Gan To Kagaku Ryoho 2016 Nov;43(12):2196-2198
    Dept. of Surgery, Kobe Ekisaikai Hospital.
    A woman in her 60s visited our hospital because of frequent hypoglycemia and episodes of unconsciousness over the last 6 years. A 4 cm tumor was detected on the pancreatic tail using abdominal computed tomography and ultrasonography. An insulinoma was strongly suspected from the results of the fasting test and glucagon load test, and a distal pancreatectomy with splenectomy was performed. Read More

    Multiple endocrine neoplasia syndrome type 1: institution, management, and data analysis of a nationwide multicenter patient database.
    Endocrine 2017 Jan 28. Epub 2017 Jan 28.
    Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
    Objective: The aim of this study was to integrate European epidemiological data on patients with multiple endocrine neoplasia type 1 by creating an Italian registry of this syndrome, including clinical and genetic characteristics and therapeutic management.

    Methods: Clinical, familial and genetic data of patients with multiple endocrine neoplasia type 1, diagnosed, treated, and followed-up for a mean time of 11.3 years, in 14 Italian referral endocrinological centers, were collected, over a 3-year course (2011-2013), to build a national electronic database. Read More

    Loss of menin in osteoblast lineage affects osteocyte-osteoclast crosstalk causing osteoporosis.
    Cell Death Differ 2017 Jan 20. Epub 2017 Jan 20.
    Institute of Comparative Molecular Endocrinology, University of Ulm, Ulm D-89081, Germany.
    During osteoporosis bone formation by osteoblasts is reduced and/or bone resorption by osteoclasts is enhanced. Currently, only a few factors have been identified in the regulation of bone integrity by osteoblast-derived osteocytes. In this study, we show that specific disruption of menin, encoded by multiple endocrine neoplasia type 1 (Men1), in osteoblasts and osteocytes caused osteoporosis despite the preservation of osteoblast differentiation and the bone formation rate. Read More

    Lung adenocarcinoma and adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1.
    Respir Med Case Rep 2017 18;20:77-81. Epub 2016 Dec 18.
    Department of Internal Medicine, Ojiya General Hospital, Niigata, Japan; Center of Diabetes, Endocrinology and Metabolism, Joetsu General Hospital, Niigata, Japan.
    Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder caused by heterozygous germline mutations in the tumor suppressor gene MEN1, which encodes a nuclear protein, menin. MEN1 is characterized by the combined occurrence of tumors involving the pituitary gland, pancreatic islets, and parathyroid glands. Additionally, patients with MEN1 often exhibit adrenal tumors. Read More

    Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process.
    Am J Med 2016 Dec 21. Epub 2016 Dec 21.
    Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Mayo Clinic, Rochester, Minn. Electronic address:
    Background: Zollinger-Ellison syndrome is a rare cause of tumoral hypergastrinemia; 1 of 5 patients with this syndrome also has multiple endocrine neoplasia type 1. The diagnosis of this disease is complicated by the widespread use of proton pump inhibitors that can elevate serum gastrin levels, the cornerstone for biochemical diagnosis. Abrupt discontinuation of proton pump inhibitors could lead to adverse outcomes. Read More

    Upregulation of RPA2 promotes NF-κB activation in breast cancer by relieving the antagonistic function of menin on NF-κB-regulated transcription.
    Carcinogenesis 2016 Dec 22. Epub 2016 Dec 22.
    Department of Nutrition, College of Medicine and Nursing, Hung Kuang University, Taichung 43302, Taiwan
    RPA2, a subunit of the heterotrimeric replication protein A (RPA) complex, is overexpressed in various cancers. In this study, we showed a significant RPA2 upregulation in breast cancer tissues and cell lines. Ectopic expression of RPA2 in MCF7 and MDA-MB-231 cells promoted cell proliferation, adhesion, migration and invasion, and induced epithelial-mesenchymal transition (EMT) of MCF7 cells. Read More

    Growth rate of small pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: results from an endoscopic ultrasound based cohort study.
    Endoscopy 2017 Jan 14;49(1):27-34. Epub 2016 Dec 14.
    Department of Gastroenterology and Hepatology, University Medical Center Utrecht, Utrecht, The Netherlands.
    Background and aims In multiple endocrine neoplasia type 1 (MEN1), endoscopic ultrasound (EUS) is used for identification and follow-up of pancreatic neuroendocrine tumors (PNETs). The role of EUS in surveillance of small ( < 20 mm) PNETs is unclear, mostly because the natural course of these lesions is largely unknown. We aimed to determine annual growth and incidence rate of small PNETs in patients with MEN1 using EUS-based surveillance. Read More

    Childhood neuroendocrine tumours: a descriptive study revealing clues for genetic predisposition.
    Br J Cancer 2017 Jan 13;116(2):163-168. Epub 2016 Dec 13.
    Department of Human Genetics, Radboud University Medical Center and Radboud Institute for Molecular Life Sciences, Geert Grooteplein Zuid 10, Nijmegen 6525GA, The Netherlands.
    Background: Neuroendocrine tumours (NETs) are rare in children and limited data are available. We aimed to specify tumour and patient characteristics and to investigate the role of genetic predisposition in the aetiology of paediatric NETs.

    Methods: Using the Dutch Pathology Registry PALGA, we collected patient- and tumour data of paediatric NETs in the Netherlands between 1991 and 2013 (N=483). Read More

    [Intrathyroidal location of parathyroid glands. Atypical presentation of multiple endocrine neoplasia type 1 syndrome].
    Cir Cir 2016 Dec 9. Epub 2016 Dec 9.
    Cirugía General y del Aparato Digestivo A, Hospital Universitario 12 de Octubre, Madrid, España.
    Background: The most common manifestation of MEN 1 syndrome is primary hyperparathyroidism (PHPT) with parathyroid multiglandular affectation. The intrathyroidal situation represents 3-4% of all glands, and it is the second most frequent location in the cervical ectopias.

    Clinical Case: 11 year old patient, with a family history of MEN1 syndrome and carrier of this same mutation. Read More

    Genetic Syndromes Associated with Central Nervous System Tumors.
    Radiographics 2017 Jan-Feb;37(1):258-280. Epub 2016 Dec 2.
    From the Department of Diagnostic Radiology, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, Iowa City, IA 52242.
    Several genetic tumor syndromes have associated central nervous system (CNS) neoplasms. The spectrum of syndromes that have intracranial tumor manifestations includes ataxia telangiectasia, Cowden syndrome, familial adenomatous polyposis, hereditary non-polyposis-related colorectal cancer, Li-Fraumeni syndrome, Gorlin syndrome, neurofibromatosis types 1 and 2, multiple endocrine neoplasia type 1, tuberous sclerosis complex, von Hippel-Lindau disease, and Turcot syndrome. Many of these disorders are inherited in an autosomal dominant fashion, and identification of the associated genetic defects has led to improved understanding of the molecular pathways involved in tumorigenesis, helping pave the way to the emergence of molecularly targeted therapeutics. Read More

    Multiple endocrine neoplasia type 1 with anterior mediastinal parathyroid adenoma: successful localization using Tc-99m sestamibi SPECT/CT.
    Ann Surg Treat Res 2016 Dec 25;91(6):323-326. Epub 2016 Nov 25.
    Department of Surgery, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
    The most common manifestation of multiple endocrine neoplasia type 1 (MEN1) is hyperparathyroidism. Treatment of hyperparathyroidism in MEN patients is surgical removal of the parathyroid glands, however ectopic parathyroid gland is challenging for treatment. A 51-year-old female, the eldest of 3 MEN1 sisters, had hyperparathyroidism with ectopic parathyroid adenoma in the mediastinal para-aortic region, which was detected by technetium-99m (Tc-99m) sestamibi scintigraphy and single-photon emission computed tomography/computed tomography (SPECT/CT). Read More

    [Study of Medullary Thyroid Carcinoma from a proband].
    Arch Argent Pediatr 2016 Dec;114(6):e421-e424
    Hospital Universitario Miguel Servet, Servicio de Endocrinología Pediátrica, Zaragoza, España.
    Thyroid cancer is an uncommon type of cancer, accounting less than 1% of all cancers in adults, and 0.5-3% of all cancers in children. There are four different types: papillary carcinoma (80-90% of cases), follicular (5-10%), medullary (5%) and anaplastic cell (2-3%). Read More

    Operation for insulinomas in multiple endocrine neoplasia type 1: When pancreatoduodenectomy is appropriate.
    Surgery 2017 Mar 15;161(3):727-734. Epub 2016 Nov 15.
    Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.
    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. Read More

    Clinical and Genetic Analysis of Multiple Endocrine Neoplasia Type 1-Related Primary Hyperparathyroidism in Chinese.
    PLoS One 2016 15;11(11):e0166634. Epub 2016 Nov 15.
    Department of Endocrinology, Key Laboratory of Endocrinology, National Health and Family Planning Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, People's Republic of China.
    Objective: Multiple endocrine neoplasia type 1-related primary hyperparathyroidism (MHPT) differs in many aspects from sporadic PHPT (SHPT). The aims of this study were to summarize the clinical features and genetic background of Chinese MHPT patients and compare the severity of the disease with those of SHPT.

    Design And Methods: A total of 40 MHPT (27 sporadic, 7 families) and 169 SHPT cases of Chinese descent were retrospectively analyzed. Read More

    Operative intervention for primary hyperparathyroidism offers greater bone recovery in patients with sporadic disease than in those with multiple endocrine neoplasia type 1-related hyperparathyroidism.
    Surgery 2017 Jan 11;161(1):107-115. Epub 2016 Nov 11.
    Department of Surgical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, TX. Electronic address:
    Background: We investigated whether the outcome of bone disease of primary hyperparathyroidism differs in multiple endocrine neoplasia type 1-associated disease and sporadic hyperparathyroidism at 1-year postoperatively.

    Methods: Multiple endocrine neoplasia type 1/hyperparathyroidism and sporadic hyperparathyroidism patients who underwent parathyroidectomy from 1990 to 2013 and dual-energy x-ray absorptiometry at baseline and 1-year postoperatively were included. Preoperative and postoperative dual-energy x-ray absorptiometry measurements (bone mineral density and Z-score at the lumbar spine, total hip, and femoral neck) were analyzed. Read More

    MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients.
    BMC Med 2016 Nov 15;14(1):182. Epub 2016 Nov 15.
    Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.
    Background: Multiple Endocrine Neoplasia type 1 (MEN1) is diagnosed when two out of the three primary MEN1-associated endocrine tumors occur in a patient. Up to 10-30 % of those patients have no mutation in the MEN1 gene. It is unclear if the phenotype and course of the disease of mutation-negative patients is comparable with mutation-positive patients and if these patients have true MEN1. Read More

    Early and Late Complications After Surgery For MEN1-related Nonfunctioning Pancreatic Neuroendocrine Tumors.
    Ann Surg 2016 Nov 1. Epub 2016 Nov 1.
    *Department of Endocrine Surgical Oncology and Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands †Department of Surgical Oncology and Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, the Netherlands ‡Imaging Division, University Medical Center Utrecht, Utrecht, the Netherlands §Department of Surgery, Leiden University Medical Center, Leiden, the Netherlands ¶Department of Surgery, Erasmus Medical Center, Rotterdam, the Netherlands ||Department of Surgery, Radboud University Medical Center, Nijmegen, the Netherlands **Department of Hepato-Pancreatico-Biliary and Liver Transplantation, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands ††Department of Surgery, VU University Medical Center, Amsterdam, the Netherlands ‡‡Department of Surgery, Academic Medical Center, Amsterdam, the Netherlands §§Department of Surgery, Maastricht University Medical Center, Maastricht, the Netherlands ¶¶Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands ||||Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, the Netherlands.
    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. Read More

    [The expression of p27(Kip1) and β-catenin in multiple endocrine neoplasia type 1-related parathyroid tumors].
    Zhonghua Nei Ke Za Zhi 2016 Nov;55(11):859-862
    Department of Endocrinology, Key Laboratory of Endocrinology, National Health and Family Planning Commission, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
    Objective: To explore tissue expression of cyclin-dependent kinase inhibitor p27(Kip1) and β-catenin in multiple endocrine neoplasia type1 (MEN1)-related parathyroid tumors (MHPT). Methods: Immunohistochemistry was performed to analyze the expression of p27(Kip1) and β-catenin in parathyroid glands from 31 subjects with MHPT collected at Peking Union Medical College Hospital from 2002 to 2013. Five normal parathyroid glands were used as control. Read More

    Fluorine-18 fluorocholine PET-CT localizes hyperparathyroidism in patients with inconclusive conventional imaging: a multicenter study from the Netherlands.
    Nucl Med Commun 2016 Dec;37(12):1246-1252
    Departments of aSurgery bInternal Medicine cNuclear Medicine, University Medical Center Utrecht dDepartment of Surgery, Diakonessenhuis Hospital Utrecht, Utrecht eDepartment of Nuclear Medicine, Meander Medical Centre, Amersfoort, The Netherlands.
    Background: Several reports have shown good performance of fluorine-18 fluorocholine (F-FCH) PET-computed tomography (CT) for parathyroid localization, although overall evidence remains scarce. We collected data from three institutions in the Netherlands and investigated the performance of F-FCH PET-CT as a second-line imaging modality.

    Materials And Methods: We performed a retrospective review of all patients at least 18 years who underwent F-FCH PET-CT for biochemically proven hyperparathyroidism (HPT) and inconclusive ultrasound and sestamibi scintigraphy. Read More

    Long-term outcomes in patients with multiple endocrine neoplasia type 1 and pancreaticoduodenal neuroendocrine tumours.
    Clin Endocrinol (Oxf) 2017 Feb 17;86(2):199-206. Epub 2016 Nov 17.
    Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.
    Background: In patients with multiple endocrine neoplasia type 1 (MEN-1), pancreaticoduodenal (PD) neuroendocrine tumours (NETs) are associated with early mortality, yet the best treatment strategy remains uncertain.

    Aim: To assess patient important outcomes (mortality and metastasis) of PD-NETs and predictors of outcomes in patients with MEN-1.

    Methods: Retrospective cohort of patients with MEN-1 who attended the Mayo Clinic, Rochester, MN from 1997 to 2014. Read More

    Multiple endocrine neoplasia phenocopy revealed as a co-occurring neuroendocrine tumor and familial hypocalciuric hypercalcemia type 3.
    Clin Case Rep 2016 Oct 18;4(10):922-927. Epub 2016 Aug 18.
    Department of Endocrinology and Internal Medicine Aarhus University Hospital Aarhus Denmark.
    Familial hypocalciuric hypercalcemia type 3 should be considered as differential diagnosis in patients with suspected primary hyperparathyroidism and/or suspected multiple neoplasia syndrome, as correct diagnosis will spare the patients for going through multiple futile parathyroidectomies and for the worry of being diagnosed with a cancer susceptibility syndrome. Read More

    Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies.
    Expert Opin Pharmacother 2016 Nov 23;17(16):2191-2205. Epub 2016 Sep 23.
    b Digestive Diseases Branch , NIDDK, NIH , Bethesda , MD , USA.
    Introduction: Neuroendocrine tumors(NETs), once thought rare, are increasing in frequency in most countries and receiving increasing-attention. NETs present two-treatment problems. A proportion is aggressive and a proportion has a functional, hormone-excess-state(F-NET), each of which must be treated. Read More

    GCM2-Activating Mutations in Familial Isolated Hyperparathyroidism.
    Am J Hum Genet 2016 Nov 13;99(5):1034-1044. Epub 2016 Oct 13.
    The National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD 20892, USA. Electronic address:
    Primary hyperparathyroidism (PHPT) is a common endocrine disease characterized by parathyroid hormone excess and hypercalcemia and caused by hypersecreting parathyroid glands. Familial PHPT occurs in an isolated nonsyndromal form, termed familial isolated hyperparathyroidism (FIHP), or as part of a syndrome, such as multiple endocrine neoplasia type 1 or hyperparathyroidism-jaw tumor syndrome. The specific genetic or other cause(s) of FIHP are unknown. Read More

    Novel Genetic Causes of Pituitary Adenomas.
    Clin Cancer Res 2016 Oct;22(20):5030-5042
    Centre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom.
    Recently, a number of novel genetic alterations have been identified that predispose individuals to pituitary adenomas. Clinically relevant pituitary adenomas are relatively common, present in 0.1% of the general population. Read More

    Efficacy and Long-Term Safety of Everolimus in Pancreatic Neuroendocrine Tumor Associated with Multiple Endocrine Neoplasia Type I: Case Report.
    Oncol Res Treat 2016 12;39(10):643-645. Epub 2016 Sep 12.
    Department of Clinical Oncology, Instituto de Ensino e Pesquisa São Lucas, São Paulo, Brazil.
    Background: Approximately 10% of pancreatic neuroendocrine tumors (NET) are associated with familial syndromes, with the most common type being multiple endocrine neoplasia type 1 (MEN-1). However, the available evidence on how to treat NET comes from studies in sporadic NET.

    Case Report: Here we report the case of a 51-year-old male patient with a metastatic MEN-1-associated pancreatic NET and hypercalcemia related to primary hyperparathyroidism and tumor-secreted parathyroid-related protein. Read More

    Genetics of gigantism and acromegaly.
    Growth Horm IGF Res 2016 Oct - Dec;30-31:37-41. Epub 2016 Aug 10.
    Section on Endocrinology & Genetics (SEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, MD 20892, USA. Electronic address:
    Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Read More

    Endoscopic imaging in the management of gastroenteropancreatic neuroendocrine tumors.
    Minerva Endocrinol 2016 Dec;41(4):490-8
    Department of Gastroenterology and Hepatology, Molinette Hospital, Turin, Italy -
    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. Read More

    Concomitant existence of pheochromocytoma in a patient with multiple endocrine neoplasia type 1.
    Surg Case Rep 2016 Dec 30;2(1):84. Epub 2016 Aug 30.
    Department of Organ Regulatory Surgery, Fukushima Medical University, Fukushima, Japan.
    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. Read More

    Management of gastric and duodenal neuroendocrine tumors.
    World J Gastroenterol 2016 Aug;22(30):6817-28
    Yuichi Sato, Satoru Hashimoto, Ken-ichi Mizuno, Manabu Takeuchi, Shuji Terai, Department of Gastroenterology, Niigata University Graduate School of Medical and Dental Sciences, Niigata 951-8121, Japan.
    Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, like all NETs. However, the incidence of GI-NETS has been increasing in recent years. Gastric NETs (G-NETs) and duodenal NETs (D-NETs) are the common types of upper GI-NETs based on tumor location. Read More

    [Neuroendocrine neoplasms of the mediastinum].
    Pathologe 2016 Sep;37(5):434-40
    Institut für Pathologie, Medizinische Universität Graz, Auenbruggerplatz 25, 8036, Graz, Österreich.
    Primary neuroendocrine tumors (NET) in the mediastinum are very rare and among them thymic NETs are the most common. They represent 5 % of all thymic and mediastinal tumors. The WHO classification from 2015 subdivides thymic NETs into three groups; low grade (typical carcinoid), intermediate grade (atypical carcinoid) and high grade (large cell neuroendocrine carcinoma and small cell carcinoma). Read More

    Comparative characteristics of primary hyperparathyroidism in pediatric and young adult patients.
    Surgery 2016 Oct 6;160(4):1008-16. Epub 2016 Aug 6.
    Department of Surgery, Division of Endocrine Surgery, University of Pittsburgh, Pittsburgh, PA. Electronic address:
    Background: Primary hyperparathyroidism is rare in pediatric patients. Our study aim was to compare primary hyperparathyroidism in pediatric (<19 years) and young adult (19-29 years) patients.

    Methods: A prospectively collected database from a single, high-volume institution was queried for all patients age <30 years who had initial parathyroidectomy for primary hyperparathyroidism yielding 126/4,546 (2. Read More

    Reoperative Surgery in Patients with Multiple Endocrine Neoplasia Type 1 Associated Primary Hyperparathyroidism.
    Ann Surg Oncol 2016 Dec 27;23(Suppl 5):701-707. Epub 2016 Jul 27.
    Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
    Background: Persistent/recurrent primary hyperparathyroidism (pHPT) occurs frequently in multiple endocrine neoplasia type 1 (MEN1). We assessed the usefulness of intraoperative PTH (IOPTH) and preoperative localizing studies based on the outcome of patients with MEN1-associated pHPT undergoing reoperative surgery.

    Methods: A retrospective analysis identified MEN1 patients with persistent/recurrent pHPT. Read More

    Male Men1 heterozygous mice exhibit fasting hyperglycemia in the early stage of MEN1.
    J Endocrinol 2016 Sep 18;230(3):347-55. Epub 2016 Jul 18.
    Department of Basic Medical SciencesMedical College, Xiamen University, Xiamen, China
    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. Read More

    A novel in-frame deletion in MEN1 (p.Ala416del) causes familial multiple endocrine neoplasia type 1 with an aggressive phenotype and unexpected inheritance pattern.
    Mol Med Rep 2016 Sep 1;14(3):2061-6. Epub 2016 Jul 1.
    Department of Endocrinology, Jagiellonian University Medical College, 31‑501 Krakow, Poland.
    The present study describes a family with multiple endocrine neoplasia type 1 (MEN1) caused by a previously undescribed in-frame deletion c.1246_1248delGCC (Ala416del) in the MEN1 gene. Evidence for the pathogenic character of this mutation, which triggers an aggressive clinical outcome, is demonstrated. Read More

    Pancreatic neuroendocrine tumors: contemporary diagnosis and management.
    Hosp Pract (1995) 2016 Aug 18;44(3):109-19. Epub 2016 Jul 18.
    a Department of Internal Medicine, Division of Gastroenterology and Hepatology , University of Utah School of Medicine, Huntsman Cancer Center , Salt Lake City , Utah , USA.
    Pancreatic neuroendocrine tumors (PNETs) are neoplasms that arise from the hormone producing cells of the islets of Langerhans, also known as pancreatic islet cells. PNETs are considered a subgroup of neuroendocrine tumors, and have unique biology, natural history and clinical management. These tumors are classified as 'functional' or 'non-functional' depending on whether they release peptide hormones that produce specific hormone- related symptoms, usually in established patterns based on tumor subtype. Read More

    Unilateral Clearance for Primary Hyperparathyroidism in Selected Patients with Multiple Endocrine Neoplasia Type 1.
    World J Surg 2016 Dec;40(12):2964-2969
    Department of Surgery, University of California, Mt Zion, 1600 Divisadero Street, San Francisco, CA, 94115, USA.
    Background: Primary hyperparathyroidism is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1). Guidelines advocate subtotal parathyroidectomy (STP) or total parathyroidectomy with autotransplantation due to high prevalence of multiglandular disease; however, both are associated with a significant risk of permanent hypoparathyroidism. More accurate imaging and use of intraoperative PTH levels may allow a less extensive initial parathyroidectomy (unilateral clearance, removing both parathyroids with cervical thymectomy) in selected MEN1 patients with primary hyperparathyroidism. Read More

    Novel multiple endocrine neoplasia type 1 variations in patients with sporadic primary hyperparathyroidism.
    Indian J Endocrinol Metab 2016 Jul-Aug;20(4):432-6
    Laboratory of Cyto-Molecular Genetics, Department of Anatomy, AIIMS, New Delhi, India.
    Background And Objectives: Primary hyperparathyroidism (PHPT) can occur either as a sporadic case or in association with syndromes such as multiple endocrine neoplasia. Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal-dominant disease resulting from mutations in MEN1 gene encoding a 621 amino acid long tumor suppressor protein "menin." We report here the results of MEN1 screening in 31 patients diagnosed with sporadic PHPT. Read More

    The Selective Arterial Calcium Injection Test is a Valid Diagnostic Method for Invisible Gastrinoma with Duodenal Ulcer Stenosis: A Case Report.
    Hiroshima J Med Sci 2016 Mar;65(1):13-7
    The localization and diagnosis of microgastrinomas in a patient with multiple endocrine neoplasia type 1 is difficult preoperatively. The selective arterial calcium injection (SACI) test is a valid diagnostic method for the preoperative diagnosis of these invisible microgastrinomas. We report a rare case of multiple invisible duodenal microgastrinomas with severe duodenal stenosis diagnosed preoperatively by using the SACI test. Read More

    Clinical Features, Treatments, and Outcomes of Patients with Thymic Carcinoids and Multiple Endocrine Neoplasia Type 1 Syndrome at MD Anderson Cancer Center.
    Horm Cancer 2016 Aug 16;7(4):279-87. Epub 2016 Jun 16.
    Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1400 Pressler Street, FCT17.6014, Unit 1484, Houston, TX, 77030-4009, USA.
    Thymic carcinoids are rare neuroendocrine tumors that occur in 1-5 % of patients with multiple endocrine neoplasia type 1 (MEN1) and are a major cause of morbidity and mortality. The few published reports associate these tumors with male sex and smoking. Our objective was to describe cases of these tumors treated at our institution. Read More

    Zollinger-Ellison Syndrome Associated with von Recklinghausen Disease: Case Report and Literature Review.
    Am J Case Rep 2016 Jun 13;17:398-405. Epub 2016 Jun 13.
    Department of Gastroenterology, Aleppo University, Aleppo, Syrian Arab Republic.
    Background: Pancreatic endocrine tumors (PETs) are rare and can occur as part of neurofibromatosis type 1 (NF1). Gastrinomas are functional PETs that are rarely associated with NF1. Only two cases of their occurrence have been reported in the literature. Read More

    Robot-assisted spleen preserving pancreatic surgery in MEN1 patients.
    J Surg Oncol 2016 Sep 6;114(4):456-61. Epub 2016 Jun 6.
    Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.
    Background: Multiple Endocrine Neoplasia type 1 (MEN1) patients often undergo multiple pancreatic operations at a young age.

    Objective: To describe robot-assisted and laparoscopic spleen-preserving pancreatic surgery in MEN1 patients, and to compare both techniques.

    Methods: Robot-assisted pancreatectomies of the DutchMEN1 study group and the Université de Lorraine, Nancy, France were compared to a historical cohort of laparoscopic treated MEN1 patients. Read More

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